Abstract: Ehlers-Danlos syndrome (EDS) is a heterogenous group of diseases that can be potentially life threatening in the vascular form. The diagnosis is mainly based on a clinical score including many items that demonstrate skin, mucosa, joint, and vessel involvement. The score is particularly helpful in hypermobile and classical forms but can be normal in the vascular type. The absence of the lingual and inferior labial frenula was reported to be a useful diagnostic tool in a series of 12 patients with classical and hypermobile EDS. However, two further reports have contested the value of this sign. To determine the diagnostic value of the absence of inferior labial or lingual frenula in EDS. Patients with EDS were prospectively recruited from November 2006 to April 2007 in a French tertiary center. Each patient was examined to check for the presence or absence of the oral frenula and matched with two controls seen from February to May 2007. Forty-three patients (ratio female/male of 2.07 : 1, mean age 31 years, range 4-63 years), 4 with classical EDS, 19 with hypermobile EDS, and 20 with vascular-type EDS, were included and matched with 86 controls. The sensitivity of the absence of the inferior labial frenulum was 42% (95% CI 27, 58) and for the lingual frenulum was 53.5% (95% CI 38, 69). The specificity was 99% (95% CI 94, 100) and 98% (95% CI 92, 100), respectively. In the vascular group, 13 of 20 patients were affected (sensitivity = 65% [95% CI 41, 85]; specificity = 97% [95% CI 87, 100]; odds ratio = 72 [95% CI 8, 645]). Inter-observer agreement was excellent (kappa value 0.91). The absence of the inferior labial frenulum or lingual frenulum is a specific sign of EDS that can be easily checked by a physician. It can be helpful in the early diagnosis of the disease, including the vascular type, in affected families. It can also be helpful to distinguish between joint hypermobility syndrome and the hypermobile type of EDS in patients with joint hypermobility.
Abstract: Summary Nonmelanoma skin cancers (NMSC) are the most common malignant tumors following solid organ transplantation. Risk factors for NMSC mainly include immunosuppression, age, sun exposure and patient phototype. Recent findings have suggested that autosomal dominant polycystic kidney disease (ADPKD) may increase the risk of developing NMSC. We performed a monocenter retrospective study including all kidney recipients between 1985 and 2006 (n = 1019). We studied the incidence of NMSC, solid cancers and post-transplantation lymphoproliferative disease (PTLD), and analyzed the following parameters: age, gender, phototype, time on dialysis, graft rank, immunosuppressive regimen, history of cancer and kidney disease (ADPKD versus others). Median follow-up was 5.5 years (range: 0.02-20.6; 79 838 patient-years). The cumulated incidence of NMSC 10 years after transplantation was 12.7% (9.3% for solid cancers and 3.5% for PTLD). Autosomal dominant polycystic kidney disease and age were risk factors for NMSC (HR 2.63; P < 0.0001 and HR 2.21; P < 0.001, respectively) using univariate analysis. The association between ADPKD and NMSC remained significant after adjustments for age, gender and phototype using multivariate analysis (HR 1.71; P = 0.0145) and for immunosuppressive regimens (P < 0.0001). Autosomal dominant polycystic kidney disease was not a risk factor for the occurrence of solid cancers after transplantation (HR 0.96; P = 0.89). Our findings suggest that ADPKD is an independent risk factor for developing NMSC after kidney transplantation.
Abstract: Low-frequency ultrasound (US) applied to skin (sonophoresis) has been investigated to enhance the transdermal transport of various drugs. Histamine is usually used in allergy investigations. We aimed to investigate, in a randomized study, the transdermal penetration of histamine with sonophoresis. Ten subjects were included. Their right forearm was divided into three zones, which were randomly assigned a treatment: no US, US(1) (I(1)=2.72 W/cm(2)), US(2) (I(2)=3.50 W/cm(2)). The primary outcome was area of induced papule, which revealed histamine penetration. Secondary outcomes were echographic measurement of papule (skin thickness) and pruritus. Measurements were taken immediately after US application and after 30 min, 2 h and 24 h. Arm zones without US application showed no papules induced by histamine; 9/10 subjects receiving US showed papules. Their mean size increased with increased intensity of US but not significantly. The skin thickness increased with US. Pruritus occurred in 7/10 cases after US and histamine. The adverse events were skin erythema, pain and tinnitus. Though this study included a few number of patients, it confirms that sonophoresis enhances skin penetration of histamine. This technology could be used at therapeutic levels: histamine could be used with sonophoresis as a positive control in allergy testing instead of prick tests, which involve skin disruption with a lancet.
Abstract: Lymphedema in children is mostly primary, due to lymphatic hypoplasia. Secondary lymphedema is caused by lymphatic injury or obstruction. We report the case of a child that developed a lymphedema of the left upper and lower extremities, with a simultaneous onset of ipsilateral hemicorporal morphea. We concluded that lymphatic obstruction was due to sclerosis from morphea. This is a unique, rarely reported mechanism of lymphedema. Lymphoscintigraphy revealed attenuated lymphatic flow in the left upper and lower limbs. Systemic corticosteroids were associated with slow improvement in the sclerotic patches. We simultaneously noticed an improvement in the lymphedema of limbs. Repeat lymphoscintigraphy revealed dramatically improved lymphatic function. This case suggests that at least in some cases lymphedema may be caused by morphea.
Abstract: INTRODUCTION: Cutaneous polyarteritis nodosa (CPAN) is an entity which needs to be acknowledged, since it can have a spontaneously adverse outcome. We report two cases of CPAN associated with Crohn's disease. CASE REPORTS: The first patient was suffering from Crohn's disease for 9 years when she was referred for a necrotic toe. A diagnosis of necrotizing angeitis was confirmed by histological examination of a skin biopsy. Despite systemic corticosteroids, the lesions became more severe, requiring immunosuppressive treatment. The second patient was a female patient referred with forefoot ischemia. Cutaneous histology confirmed the diagnosis of necrotizing angeitis that responded favourably to corticosteroid treatment. The patient had been diagnosed with Crohn's disease 2 months previously. CONCLUSION: CPAN differed from systemic PAN by the absence of visceral involvement. Its association with Crohn's disease, although uncommon, must be recognized as it affects treatment and monitoring.
Abstract: Histologic measurement of the thickness of melanoma is a major prognostic factor and governs the size of the surgical excision (1cm for melanomas less than 1 mm thick, 2 cm for melanomas thicker than 2 mm and 3 cm beyond 4 mm). To determine whether high-resolution ultrasound can be used to predict surgical margins and, thus, to operate on patients in a single procedure avoiding further re-excision, we performed a systematic review of studies published from January 1987 to June 2007 and a prospective study. The systematic review selected 14 studies comparing histologic and ultrasound measurements and showing correlation coefficients generally greater than 0.9. Data available from 7 of the 14 studies (total 869 patients) showed predictive values of adequate margins in at least 72% of lesions using preoperative measurement of ultrasound thickness. The prospective study included 31 patients referred with a primary melanoma from March 2005 to March 2007. Ultrasound measurement of thickness was possible except for thin melanomas (<0.4 mm) in areas with marked photoaging, in the plantar zone, and in the case of very thick melanomas exceeding the explored depth (7.6 mm). The average thickness was 1.96 mm measured by ultrasound (SD: 2.15) and 1.95 mm by histology (SD: 2.62) and the Bland and Altman graph showed moderate agreement between ultrasound and histology. Limits of agreement were estimated at -1.4 and +1.8, corresponding to relative limits of agreement of -40 to +80%. Ultrasound predicted appropriate margins (1, 2 or 3 cm wide according to sonometric thickness) in 26 of the 31 subjects (84%, 95% CI 66-95). Preoperative high-resolution ultrasound is a noninvasive examination that can help in choosing appropriate surgical margins and should reduce the need of partial or excisional biopsy before surgery, and the need for further re-excision.
Abstract: Summary Background Blastic plasmacytoid dendritic cell neoplasm (BPDCN) represents the malignant counterpart derived from plasmacytoid dendritic cells. This rare entity is usually revealed and diagnosed on cutaneous lesions associated or not with a leukaemic component. The prognosis associated with BPDCN is very poor. Objectives To perform a retrospective review of BPDCN cases registered in the French Study Group on Cutaneous Lymphoma database from June 1995 to May 2008. Methods Forty-seven patients were included. Demographic data, initial staging, therapeutic management and outcome were recorded. Results The mean survival was 16.7 months (95% confidence interval 12.6-20.8). Only eight (17%) and one (2%) patients reached respectively 2 and 5 years of survival. Initial spreading of the disease did not represent, in this cohort, a reliable prognosis factor. The outcome was overall influenced by treatment provided. While radiation therapy, monochemotherapy or even polychemotherapy regimens did not significantly affect the course of the disease, the survival of bone marrow transplanted patients was significantly higher. Conclusions Despite the fact that BPDCN is often initially limited to the skin, only an aggressive initial therapy may improve the patients' prognosis. Local treatments, such radiation therapy, are definitively useless. Regardless of the initial extension of the disease, in our experience only bone marrow transplantation significantly improved the outcome.
Abstract: BACKGROUND/PURPOSE: The diagnosis of the Ehlers-Danlos syndrome (EDS) is primarily clinical. Clinical signs result from modifications of the rheological properties of the skin: thickness, extensibility and hydration. Our main objective was to demonstrate what skin biometry can contribute to the diagnosis and evaluation of the different types of EDS. METHODS: Forty-one patients clinically diagnosed with EDS were paired by age and sex to 41 healthy subjects with no known dermatologic disease, in particular connective tissue diseases. We measured skin thickness, extensibility, hydration and sebum secretion by skin ultrasonography, use of a Cutometer, a Corneometer and a Sebumeter. RESULTS: The skin is thinner in all three disease types (classical, hypermobility and vascular). It is more extensible only in the classical type, and more hydrated and fatty in the hypermobility type. CONCLUSION: Non-invasive measurement of skin thickness and elasticity are quantitative parameters that can be used to better diagnose EDS. Moreover, the sebaceous rate can help to diagnose the hypermobility type whereas the immediate retraction distinguishes the classical form from vascular and hypermobile types.
Abstract: No tool is available to diagnose drug-induced pemphigus (DIP) and to predict its outcome after the withdrawal of the culprit drugs. This retrospective pemphigus case series study compared cutaneous/mucosal immunostaining of a monoclonal antibody directed toward desmogleins 1 and 3 (32-2B) in 37 patients with DIP and 56 patients with idiopathic pemphigus.There was a significant difference between the groups in terms of pruritus, superficial form, mucosal involvement, and circulating antibodies. 32-2B staining disclosed a patchy pattern in 47 (84%) of idiopathic pemphigus cases and in 11 (30%) of DIP cases (P<.0001). A normal pattern, used as a diagnostic test for DIP, had 70.3% sensitivity (95% confidence interval [CI], 53.0-84.1), 83.9% specificity (95% CI, 71.7-92.4), a 32.7% positive predictive value, and a 97.9% negative predictive value. Of 17 patients with DIP with a normal pattern of 32-2B, 14 recovered, whereas only 2 of 9 patients with DIP with a patchy pattern recovered (P<.005).32-2B immunolabeling is useful for diagnosing DIP and is an indicator of a good prognosis.
Abstract: BACKGROUND: While haemangioma is common, an unusual appearance or course should alert the clinician's concern. Congenital haemangioma, particularly rapidly involuting congenital haemangioma (RICH), may carry a risk of misdiagnosis as congenital malignant tumours such as infantile fibrosarcoma (also known as congenital infantile fibrosarcoma). In this case, histological diagnosis may prove inconclusive, as in the case reported herein. PATIENTS AND METHODS: At birth, a newborn baby presented angiomatous lesions on the sole of the left foot that was initially considered as congenital haemangioma. Histopathological examination suggested highly remodelled immature infantile haemangioma. After surgery, the tumour increased in size within eight weeks. Reanalysis of the histology slides resulted in a diagnosis of infantile fibrosarcoma. This diagnosis was confirmed by the presence of a specific translocation seen in infantile fibrosarcoma (ETV6/NTRK3). CONCLUSION: There is a risk of erroneous diagnosis in newborn infants between angiomatous tumour in RICH and malignant congenital tumours (particularly infantile fibrosarcoma). Clinicians should be attentive for this type of lesion and take all necessary diagnostic measures.
Abstract: BACKGROUND: By definition, stomatodynia or burning-mouth syndrome involves oral pain with no causes being found on history taking or examination. An allergic origin is often suspected by doctors and patients alike. In this study, we attempted to assess the value of epicutaneous tests in demonstrating allergic causes for patients presenting stomatodynia. PATIENTS AND METHODS: This was a single-centre retrospective study of patients undergoing epicutaneous tests between 1996 and 2003 to screen for allergic causes of mouth pain not accounted for by any abnormalities seen during examination performed at consultations for mouth disease. RESULTS: Forty patients were included (11 male, 29 female; mean age: 58 years), and 39 were excluded. Sixteen patients presented at least one positive test, with a total of 35 positive tests in all. In decreasing order of frequency, the causes were metals, mercury derivatives (nickel salts: n=5; chrome salts: n=3; palladium salts: n=2; phenylmercuric acetate: n=2; thiomersal: n=2; cobalt salts: n=1; gold salts: n=1; mercury: n=1) and resins (acrylates: n=4). The relevance of these test results was considered probable in three cases and possible in five cases, associated with the existence of metals or resins in patients' mouths. The Peru balm test was positive in four cases but was not relevant. Tests for personal products were negative in all cases, with the exception of one case of resin from a prosthesis and one case of tixocortol pivalate. COMMENTS: Type I stomatodynia (daily occurrence with gradually increase in discomfort throughout the day) and type II stomatodynia (permanent) are not normally attributable to allergies. However, for type III stomatodynia (non-permanent, with acute episodes followed by remission), an allergy survey guided by questioning may be undertaken to determine the cause, primarily prostheses or diet. The relevance of positive test results must be interpreted with caution in view of the incidence of positive epicutaneous tests for metals and Peru balm among the general population studied.
Abstract: BACKGROUND: Rosacea fulminans is a rare and severe form of rosacea, with acute onset in women between 20 and 40 years. Although the aetiology remains unknown, pregnancy has been reported to be a triggering factor. We report a case of rosacea fulminans occurring at the onset of pregnancy and discuss a possible triggering role of endocrine factors, as well as therapeutic options. CASE REPORT: A 32-year-old woman with no previous history of dermatological disease consulted for rosacea fulminans appearing within the first three weeks of her first pregnancy, which required hormonal stimulation with recombinant FSH (follitropin alpha, Gonal F) and an LHRH inhibitor (cetrorelix, Cetrotide). She did not use topical corticosteroids or any other medication and had no other abnormalities at clinical examination. The skin disease lasted throughout pregnancy despite different treatments. After delivery, moderate improvement was observed within two weeks. Treatment with isotretinoin 0.5 mg/kg/day was started three months after delivery and led to the disappearance of the papular and pustular lesions within three weeks, with persistence of the erythema for six months. DISCUSSION: The patient had never previously presented from acne or rosacea and the question of the responsibility of hormonal changes induced by stimulating ovulation or pregnancy can be raised. However, since her rosacea lasted throughout pregnancy, a causal role of the drugs used for ovarian stimulation appears unlikely. We suggest that pregnancy was the major triggering factor in this case. The recurrence of the skin disease on a subsequent pregnancy would constitute a strong argument to support this hypothesis and rule out simple co-incidence.
Abstract: BACKGROUND: Eosinophilic fasciitis (Shulman syndrome) is defined by the association of sclerodermatous skin changes involving underlying fascia and hypereosinophilia. While the aetiology is unknown, some observations suggest an infectious origin. We report the association of eosinophilic fasciitis with an infection involving Borrelia burgdorferi. PATIENTS AND METHODS: A 54 year-old man consulted for a hardened oedema and stiffness of the calves associated with an oedema of the left hand evolving for 4 months. Routine blood tests showed hypereosinophilia at 1.01 G/l and moderate inflammatory syndrome. Diagnosis of eosinophilic fasciitis was confirmed by MRI and muscle biopsy. Since the patient had reported previous tick bites some months before onset, he was tested for Lyme disease. An ELISA test revealed IgG directed against Borrelia burgdorferi and this was confirmed by Western blot analysis. DISCUSSION: The association of eosinophilic fasciitis with Lyme disease raises the question of a real link or a fortuitous association between the two conditions. Similar cases have been described in the literature with or without isolation of the spirochete from skin or fascia lesions. The incidence of eosinophilic fasciitis remains low compared to the prevalence of the infection in endemic areas. We suggest that in some patients, perhaps genetically predisposed, infection with B. burgdorferi may be at the origin of fasciitis.
Abstract: BACKGROUND: Ehlers-Danlos syndrome comprises a rare and heterogeneous group of diseases affecting the skin, joints and vessels. Diagnosis is based on clinical score since molecular testing is not routinely available. High sensitivity and specificity concerning the absence of inferior labial and lingual frenula has been reported in Ehlers-Danlos syndrome, but the diagnostic value of this finding has been contested. PATIENTS AND METHODS: We here report the cases of 2 female patients, one with a vascular form of Ehlers-Danlos syndrome complicated by spontaneous dissection of the carotid and vertebral arteries, and the other with classic type; in both women, inferior labial and lingual frenula were absent. DISCUSSION: We discuss the discrepancies between the 3 published studies dealing with abnormalities of the inferior labial and lingual frenula in Ehlers-Danlos syndrome. We suggest routine oral examination in patients with suspected Ehlers-Danlos syndrome. The diagnostic value of absence of oral frenula in Ehlers-Danlos syndrome requires confirmation by a prospective study.
Abstract: BACKGROUND: Dermo-hypodermal bacterial infections (erysipelas, cellulitis and necrotizing fasciitis) are frequent and may be life-threatening. PATIENTS AND METHODS: A retrospective study of a period of 4 years and 6 months (1 June 1999 to 31 December 2003) was carried out at the Donka University Hospital centre (Conakry-Guinea) in order to analyze the epidemiological, clinical and therapeutic characteristics of bacterial dermohypodermitis in a hospital environment. RESULTS: Two hundred and forty-four patients (188 women and 56 men) were hospitalized for bacterial dermohypodermitis. Mean age was 38 years. The site of dermohypodermitis comprised the entire lower limbs in 4 cases (2%), legs and feet in 200 cases (82%), thighs in 12 cases (4%), buttocks in 4 cases (2%) and upper limbs in 24 cases (10%). A previous history of dermohypodermitis, chronic alcoholism, use of non-steroidal anti inflammatory drugs, obesity and lymphoedema was identified. Necrotizing bacterial dermohypodermitis and necrotizing fasciitis were the main complications and were seen in 31 patients. These conditions were generally associated with use of non-steroidal anti inflammatory drugs (90% vs. 25%) (OR=27, CI 95=8-94), delayed initiation of suitable treatment and use of traditional medicine. CONCLUSION: Our study shows female predominance of bacterial dermohypodermitis. This is explained by cutaneous atrophy in women resulting from use of depigmenting drugs that facilitate skin breaks, thus allowing ingress of bacteria. NSAID intake, while frequent in our series, was far higher in the fasciitis group, suggesting a potentially aggravating role of these drugs.
Abstract: Patients treated with immunosuppressive agents are prone to developing lymphoproliferative disorders, in particular Epstein-Barr virus-associated lymphoproliferative disease. This complication was reported first in post-transplanted patients treated with cyclosporine, and, more recently, in patients receiving long-term methotrexate therapy for inflammatory disease. We describe the case of a 70-year-old female patient with multifocal cutaneous lymphoproliferative disease occurring in the course of long-term, weekly methotrexate therapy for rheumatoid arthritis. Immunohistochemical study revealed the presence of latent membrane protein within neoplastic cells. Cutaneous lesions initially continued to increase in number and size in the first 2 months and finally disappeared completely within 5 months after discontinuation of methotrexate. The patient is now in complete remission with 12-months' follow-up. Despite initial progression after cessation of immunosuppressive therapy, Epstein-Barr virus-induced lymphoproliferative disease may disappear completely within months, thus avoiding pointless chemotherapy.
Abstract: OBJECTIVES: To describe clinicopathologic features and to identify prognostic factors in a large series of primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL LT), as defined in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. DESIGN: Retrospective multicenter study from the French Study Group on Cutaneous Lymphomas. SETTING: Nineteen departments of dermatology in 10 regions of France. PATIENTS: Sixty patients with a PCLBCL LT included in the registry of the French Study Group on Cutaneous Lymphomas. MAIN OUTCOME MEASURES: Age, sex, outcome, therapy, B symptoms, cutaneous extent, number of lesions, location (leg vs nonleg), serum lactate dehydrogenase level, and MUM-1 and Bcl-2 expression were recorded. Disease-specific survival was used as the main end point. Prognostic factors were identified using a Cox proportional hazards model. RESULTS: Primary cutaneous diffuse large B-cell lymphoma, leg type is characterized by a predilection for the leg (72%), a high proportion of Bcl-2 expression (85%), an advanced age at onset (mean age, 76 years), and frequent relapses and extracutaneous dissemination. The overall 5-year disease-specific survival rate was 41%. Location on the leg and multiple skin lesions were predictive of death in multivariate analysis. Although no variable related to therapy was significantly associated with survival, patients recently treated with combinations of anthracycline-containing chemotherapies and rituximab had a more favorable short-term outcome. CONCLUSIONS: Primary cutaneous diffuse large B-cell lymphoma, leg type is a distinct entity with a poor prognosis, particularly in patients with multiple tumors on the legs. Despite the advanced age of many patients, the prognosis could be improved with combinations of anthracycline-containing chemotherapies and rituximab.
Abstract: INTRODUCTION: Lipoid proteinosis is a rare autosomal recessive disease that has recently been shown to result from mutations in the ECM1 gene. Some cases are associated with a more severe mucocutaneous phenotype. OBSERVATION: We report the case of a 38-year-old woman who had clinical and histological skin features typical of Urbach-Wiethe disease. Physical examination revealed neurological abnormalities including dizziness, ataxia, slight psychomotor retardation and amnesic impairment. The patient reported sudden left brachiofacial paralysis one month earlier that regressed spontaneously after one week. CT scan and MRI were normal. Cerebral scintigraphy displayed bilateral hypoperfusion of the frontal areas, of the anterior and internal right temporal lobe (which includes amygdala), and of the left thalamic core. DISCUSSION: We considered these abnormalities as neurological features of lipoid proteinosis in the absence of evidence of any other cause. Our observation as well as other recent reports suggests the need for neurological and neuropsychological investigations in patients with Urbach-Wiethe disease.
Abstract: Although less precise than histological examination, high-resolution skin imaging is a noninvasive technique that provides complementary information to the clinical examination: the lesion's US structure as well as its lateral and depth extension. Ultrasonography helps in diagnosis and initial management by measuring melanoma thickness (so as to remove this lesion with safety margins) before destructive treatment (cryosurgery or radiotherapy), by showing tumor limits, or by detecting subclinical recurrence during the follow-up. In this paper, ultrasound images will be correlated with clinical and histological data in various clinical situations such as tumors, edema, cutaneous infiltration, sclerous tissues, hyperlaxity, and cutaneous aging.
Abstract: BACKGROUND: Hepatitis C virus (HCV) frequently causes leucocytoclastic vasculitis as a result of type II or III cryoglobulinemia. HCV-associated vasculitis without cryoglobulinemia is less common. PATIENTS AND METHODS: A 33-year-old woman consulted for infiltrative necrotic purpura of the lower limbs, responsible for leg ulcers measuring less than 1 cm. Histopathological examination revealed vasculitis affecting the hypodermic arterioles and caused by periarteritis nodosa. No extracutaneous involvement was observed. The patient had presented asymptomatic untreated HVC infection (genotype 3) for two years. Antiviral treatment resulted in elimination of the patient's viremia and no relapse of skin lesions was observed two years after the end of treatment. COMMENTS: This patient presented vasculitis due to cutaneous nodular periarteritis associated with HVC without cryoglobulinemia. Hepatic impairment was mild and did not require any antiviral treatment. No further skin involvement was seen after treatment with colchicine and because the patient's viral genotype was favorable, we decided to initiate antiviral therapy. This therapeutic approach should be considered by dermatologists, but it is nevertheless important to assess the risk of interferon-induced aggravation of vasculitis.
Abstract: BACKGROUND: Vulvar pain is a common presenting complaint in dermatology consultations arising from numerous possible causes. Where such pain is mechanical, resulting from stretching of the vulva, particularly during sexual intercourse, the patient should be checked for anatomical features that could account for the pain, localised anatomical adhesions or sclerosis of the fourchette, and amenable to treatment by vulvoperineoplasty. PATIENTS AND METHODS: A retrospective study of all patients undergoing vulvoperineoplasty for vulvar pain in the fourchette was carried out between 1.1.1993 and 1.1.2003. RESULTS: Fifteen successive patients were included in the study. The mean rate of sexual intercourse doubled between the pre-operative period and the post-operative period, rising from 5 (0 to 30) to 9.8 (3 to 30). Mean pain intensity during intercourse on a scale of 1 to 10 fell from 7.6 (5 to 9) before surgery to 1.6 (0 to 5) after surgery. None of the patients experienced any sequelae. All patients except one (who was no longer sexually active), expressed willingness to undergo vulvoperineoplasty again if necessary. DISCUSSION: Vulvoperineoplasty is a simple procedure and proved useful for female patients presenting vulvar pain and adhesions or sclerosis of the fourchette.
Abstract: INTRODUCTION: Neutrophilic eccrine hidradenitis (NEH) is a rare disease belonging to the group of neutrophilic dermatoses. It has a characteristic histopathologic pattern, with necrosis of the eccrine glands and a local neutrophilic infiltrate. It occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia or, less frequently, another malignancy. Its occurrence in healthy patients is rare. CASE REPORT: We report the case of a 56-year-old woman with no remarkable medical history who developed an erythematous papular facial eruption. Skin biopsy showed typical features of neutrophilic eccrine hidradenitis including neutrophilic infiltrate. No cause was found. The patient was successfully treated with colchicine. DISCUSSION: This case is particular because NEH was not associated with malignant hematologic disease, solid cancer, chemotherapy, fever or any other disease, after a follow-up of 22 months. Such idiopathic NEH has been reported only rarely in adults. The second particularity is the length of the eruption, which required colchicine treatment. Clinical improvement occurred within 1 month. Because hematologic malignancies can in some cases be preceded by neutrophilic dermatitis, clinical follow-up is recommended in adults.
Abstract: BACKGROUND: Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. CASE REPORT: A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.
Abstract: Cutaneous side effects from heparin administration are rare and usually located at injection sites. We report 3 cases of intraepidermal hemorrhagic blisters occurring distant from sites of subcutaneous injections of heparin. A causative link is suggested by a temporal relationship between heparin introduction and onset of disease as well as exclusion of other causes, but the mechanism remains unknown.
Abstract: Rituximab (MabThera, Roche) is a chimeric monoclonal antibody directed against the CD20 antigen. Its efficacy and safety were first demonstrated in the treatment of systemic B-cell lymphomas. We report the use of intralesional injections of rituximab into some but not all cutaneous lesions in a patient with multiple primary cutaneous follicular centre B-cell lymphoma. This treatment resulted in tumour regression, even of the lesions that had not been injected. We therefore hypothesize that there is systemic diffusion of rituximab from injected sites despite the low doses injected locally, or the induction of a specific antitumour immune response acting systemically.
Abstract: INTRODUCTION: Paraneoplastic pemphigus is associated with Castleman's disease. We report a case of paraneoplastic pemphigus at the stage of the sarcomatous transformation of Castleman's disease, present for many years but without concomitant paraneoplastic pemphigus. The pemphigus was manifested by the most unusual, exclusive, involvement of the mucosa of the mouth and lung. OBSERVATION: A 32 year-old man suffering from extensive ulceration of the oral mucosa was hospitalized in December 2000 for alteration in his general status of health and acute respiratory failure. The search for intercellular anti-substance antibodies on rat spleen was positive, corresponding to anti-envoplakin IgG and leading to the diagnosis of paraneoplastic pemphigus. The thoracic x-ray and scan revealed a hilum tumor, the histological examination of which confirmed the diagnosis of Castleman's disease concomitant to sarcomatous transformation. Following surgical treatment, the respiratory failure worsened. The patient improved with systemic corticosteroids at the dose of 2 mg/kg/d and chemotherapy was initiated. The patient died suddenly within the context of acute respiratory failure, three months after surgery. DISCUSSION: This is a case of paraneoplastic pemphigus of unusual clinical and biological expression: exclusively mucosal involvement with obliterating bronchiolitis, explained by the isolated presence of antibodies recognizing envoplakin, without anti-desmoglein. The transformation of the Castleman tumor into a sarcoma may have unmasked intra-cellular antigens (plakins), initiating the specific immune reaction.
Abstract: INTRODUCTION: Castleman's disease is a lympho-proliferative disease of unknown cause. This rare disease, usually localized, is benign and develops in the young adult. The predominant localization is mediastinal. OBSERVATION: A 15 month-old infant was referred for a left latero-cervical mass that had developed since the age of 3 months. Other than this, the clinical examination was normal. Sonography, x-ray and tomodensitometry confirmed the unique nature of the lesion and the absence of loco-regional invasion. Histology following surgical exeresis found a nodule with multiple pseudo-follicular structures. These mitosis-rich structures were arranged concentrically around a vessel and were bordered by small lymphocytes forming a crown. This histological aspect corresponded to Castleman's disease in its vascular hyaline form. Local recurrence was reported twice, 2 and 8 years later and was treated each time with surgical exeresis. Histological examination was always similar to the first. DISCUSSION: Castleman's disease has rarely been reported in infants. The cervical location, at whatever age, is far rarer than the mediastinal form. Supplementary examinations are not always of interest and basically provide information on the isolated or multicentric nature. Surgical exeresis was justified because of the diagnostic doubts and the risk of compression. Diagnosis is based on anatomopathology. Relapses have rarely been described in the literature and raise the question of the potential malignant transformation after several relapses.
Abstract: INTRODUCTION: Pellagra is an exceptional disorder in France. The classical description of pellagra associates a photoinduced rash with neurological impairment and intestinal dysfunction. Without adapted treatment, the progression is fatal. CASE REPORT: A 62 year-old women developed a photoinduced rash, composed of circular and erythematous elements with pustular edges. She also had panniculitis, peripheral neuropathy, depressive mood and diarrhea. Her medical past was marked by epilepsy treated with sodium valproate and hydantoin. Biological exams revealed lowered plasma levels of vitamins PP, B1, B6 and zinc, secondary to intestinal impairment induced by bacteria proliferating in the small intestine. The rash resolved with vitamin PP and zinc supplementation. The bacterial colonisation was improved by long-term, sequential antibiotics. DISCUSSION: We report a rare clinical form of pellagroid rash. The rash was induced by chronic malabsorption resulting from excessive bacterial proliferation in the diverticules of the small intestine. The antiepileptic treatment could have facilitated vitamin PP et zinc deficiency. Panniculitis was related to the bacterial proliferation. We discuss in this paper the relationship between some vitamin deficiencies, their clinical manifestations and the direct role of intestinal bacterial proliferation in the cutaneous manifestations.
Abstract: Blastic natural killer (NK) cell lymphoma (also termed CD4+CD56+ hematodermic neoplasm) is a recently described entity, with the first case reported in 1994. It was suggested initially that the disease originates from NK cells. Since 1994, single cases and a few small series have been published. In this review, data from the literature and a series of 30 cases from the French and Dutch study groups on cutaneous lymphomas are discussed. The major clinical, histopathologic, and phenotypic aspects of the disease and diagnostic criteria and data suggesting a plasmacytoid dendritic cell origin for the tumor cells are provided.
Abstract: BACKGROUND: There is still lack of consensus regarding the most effective follow-up for stage I and II melanoma patients although some consensus conferences have provided guidelines stating that clinical examination should be the standard. OBJECTIVES: Our aim was to study the value of adding ultrasound lymph node examination (7.5 MHz) to the routine clinical examination recommended by French guidelines in melanoma follow-up. METHODS: A cohort of melanoma patients was enrolled between 1 July 1995 and 1 July 2000 in a follow-up protocol including clinical examination performed four times a year for thick melanomas (Breslow index > or = 1.5 mm) and twice a year for thin melanomas (Breslow index < 1.5 mm) according to French guidelines, and ultrasound lymph node examination performed every 6 months for thick melanomas and every year for thin melanomas. Follow-up was continued up to 1 July 2003. When clinical or ultrasound examination indicated signs of node recurrence, surgical biopsy of the involved node was performed. When ultrasound examination was only suspicious, another ultrasound examination was performed within the following 3 months. The results of both clinical and ultrasound examinations were compared with histopathology examination when node biopsy was performed. RESULTS: Ultrasound follow-up was performed for 373 patients (213 females and 160 males). Mean age at diagnosis of melanoma was 59 years (range 14-90, SD 15). In total, 1909 ultrasound examinations combined with clinical examination were analysed. Node biopsy was performed in 65 patients and demonstrated melanoma metastases in 54. Sensitivity of clinical examination and ultrasound examination was 71.4%[95% confidence interval (CI) 55.4-84.3] and 92.9 (95% CI 80.5-98.5), respectively, P = 0.02. Specificity of clinical examination and ultrasound examination was 99.6% (95% CI 99.2-99.8) and 97.8% (95% CI 97.0-98.4), respectively. Despite this apparent superiority of ultrasound examination over palpation, only 7.2% of the patients really benefited from ultrasound examination (earlier lymph node metastasis detection or avoidance of unnecessary surgery), while 5.9% had some deleterious effect from ultrasound examination (unnecessary stress caused by repetition of ultrasound examination for benign lymph nodes, useless removal of benign lymph node). CONCLUSIONS: This study confirms the greater sensitivity of ultrasound examination to clinical examination in the diagnosis of node metastases from cutaneous melanoma. However, the place of ultrasound in routine follow-up is at least questionable as only a very small proportion of patients (1.3%) really benefited from adding ultrasound examination to clinical examination.
Abstract: Agranular CD4/CD56 hematodermic neoplasm (CD4/CD56 HN), also termed blastic natural killer cell lymphoma, is characterized by a peculiar immunophenotype and high skin tropism. The lineage of origin is not known, and a plasmacytoid dendritic cell derivation has been proposed. CD4/CD56 HN generally is diagnosed by using tumor skin biopsy, with the most important differential diagnosis being myelomonocytic leukemia cutis. We evaluated the expression of 2 plasmacytoid dendritic cell antigens, T-cell leukemia 1 (TCL1) and cutaneous lymphocyte-associated antigen (CLA), in 29 cases of CD4/CD56 HN and 18 cases of myelomonocytic leukemia cutis. TCL1 and CLA were expressed in 26 (90%) of 29 CD4/CD56 HN cases vs TCL1 expression in 3 (17%) and CLA expression in 14 (78%) of 18 leukemia cutis cases. Furthermore, CLA antiserum displays a peculiar small-dot staining pattern in CD4/CD56 HN. These results suggest that TCL1 and CLA are good markers for CD4/CD56 HN tumor cells and add support for a plasmacytoid dendritic cell origin. The high skin tropism of CD4/CD56 HN might be related to the skin-homing property of CLA.
Abstract: BACKGROUND: Limb lymphedema results from incompetence of the lymphatic system, and treatment of both primary and secondary forms involves manual lymphatic drainage and support. The effectiveness of treatment varies from patient to patient and can be unpredictable.OBJECTIVE: To investigate clinical and paraclinical criteria able to predict responses to combined physical treatment (lymphatic drainage and multilayered support) during hospitalization.PATIENTS AND METHODS: A retrospective study was performed in 45 patients admitted for one week's intensive treatment of limb lymphedema in the Lymphology Unit of the Department of Dermatology of the University Hospital of Tours. Lymphoscintigraphy was performed for all patients on admission. Loss of volume in affected limbs was studied to evaluate the effectiveness of treatment.RESULTS: Two criteria were predictive of resistance to combined treatment, i.e. venous insufficiency and continuing lymph node evidence of scintigraphic activity four hours after lymphoscintigraphy. The two factors were significantly related.CONCLUSION: Failure of combined physical treatment for lymphedema appears to be related to venous insufficiency. It is therefore essential to investigate and treat venous insufficiency, particularly in patients with continuing evidence of scintigraphic activity in lymph nodes.
Abstract: INTRODUCTION: The quantity of topical treatments for leg ulcers has increased over the last 15 years.OBJECTIVE: To determine the prescriptions for topical treatments and the problems in the management of leg ulcers using a questionnaire sent to general practitioners.MATERIAL AND METHODS: A questionnaire in 3 parts was sent to 95 general practitioners in the area working in 36 different districts with an explanatory letter. The first part was composed of 29 closed questions regarding prescription of topical treatment for leg ulcers. The second part, also closed, included 3 pictures showing a budding, a necrotic and a fibrinous ulcer. The physicians had to choose which treatment they would have prescribed and the frequency of dressings changes. The third part was composed of two open questions regarding the problems encountered. The survey started on May 1st and was closed on June 30th 2002. No reminders were sent.RESULTS: We received 52 replies, 49 of which were exploitable. Forty-five percent (43/95) did not reply. Occlusive dressings were widely prescribed (mainly hydrocolloid 38/49 and hydrocellular 28/49). Vaseline impregnated gauze were less prescribed (14/49). Dressings with balsam of Peru were prescribed often by 10 and occasionally by 27 general practitioners. Alginate or charcoal dressings were not always used appropriately. Antiseptics were prescribed by 10 physicians. Mechanical debridement of fibrinous or necrotic wounds was rarely used even for necrotic ulcers. Topical anesthetics were prescribed in 21 out of 33 cases of mechanical debridement. Some physicians referred care (n=4), others complained about lack of compliance (n=15), cost (n=14), local intolerance (n=10) and the excessively wide variety of dressings (n=10). We drew-up a synopsis with guidelines for treatment, which was sent to all the general practitioners who had participated in the survey.CONCLUSION: Forty-three of the 95 physicians approached did not reply. This poor response rate, which is common in this type of survey, may include physicians who are not at ease in the management of ulcers and this may bias the results. The physicians who replied to the survey knew the subject well but complained of the costs and variety of dressings. They were all eager to receive further information and guidelines.
Abstract: BACKGROUND: Sensitization to topical treatments used in leg ulcers is common. Questioning and patch testing are used to identify causative drugs or dressings. OBJECTIVES: To study the current frequency of sensitization in our centre, to analyse previously published studies, and to compare sensitization in recent years with studies published before 1990. METHODS: A retrospective study was undertaken of all patients admitted with leg ulcers in 2001 and 2002. Of the files of 235 patients with leg ulcers reviewed, we identified 106 patients (group A) who had been patch tested in our institution between 2001 and 2002 with the European standard series and an additional series. The frequency of sensitization was compared with an earlier unpublished retrospective study in our centre of 36 leg ulcer patients tested in 1988 (group B), with a group of unselected contact dermatitis patients tested between 1 January 1997 and 31 December 2000 in our centre (group C), and with results published in the literature. We performed a computerized database search of MEDLINE and compared results obtained in recent years with those obtained before 1990 to obtain evidence of changing trends. RESULTS: Seventy-five per cent of the 106 patients had at least one positive reaction, and 57% had two or more positive reactions. Balsam of Peru was positive in 40% of cases, followed by lanolin (21%), fragrance mix (18%), trichlocarban (13%), colophony (11%), Cetavlon (cetrimide cream) (9%) and neomycin (9%). Thirty-five of the 36 group B patients had at least one positive reaction. Lanolin was the most frequent (31%), followed by balsam of Peru (22%), Cetavlon (19%), colophony (14%), terebenthene (14%), quinoline mix (11%) and benzocaine (8%). Two hundred and eighty-six of 526 patients of group C were positive in at least one test (54.4%). Three allergens gave a sensitization rate >10%: balsam of Peru (12.5%), fragrance mix (15.2%) and nickel sulphate (21.1%). Review of the literature and calculation of sensitization rates in a pool of 3043 patients extracted from 24 series, plus our own, showed persistence of high sensitization rates and a significant (8.5%) increase in sensitization between the two periods compared. There was a decrease in sensitivity to lanolin, constant sensitization to aminoglycosides, a slight increase in sensitivity to thiuram mix and glucocorticoids, and a marked increase in sensitivity to balsam of Peru in France. Newer topical treatments and dressings showed very low rates of sensitivity with the exception of hydrogels. CONCLUSIONS: Despite warnings, sensitization to topical treatments for leg ulcers is still frequent and, moreover, continues to increase, some variations reflecting local nursing practices and variations in topical treatment available. Although a decrease in sensitization rate with lanolin has been observed throughout the world, no decrease in sensitization rate has been demonstrated with aminoglycosides, and sensitization to glucocorticoids, thiuram mix and new products (hydrogels) is now increasingly being reported.
Abstract: INTRODUCTION: Systemic corticosteroid therapy is the usual treatment for hemangioma with vascular risk. Tolerance to such treatment is usually good. OBSERVATION: A two Month-old girl presented with a hemangioma of the lower right eyelid that had developed since she was 15 days old. Treatment with betamethasone at the dose of 0.3 mg/kg/day was prescribed because of the risk of alteration in sight. The treatment was highly effective. The discovery of a systolic cardiac murmur motivated a cardiac sonography that showed signs of an obstructive hypertrophic myocardiopathy. The progressive reduction of the corticosteroids led to the regression of this disease. DISCUSSION: The onset of a cardiomyopathy during systemic corticosteroid therapy has only been reported exceptionally in the literature. There is little known of the risks. When in doubt, clinical and sonographic monitoring is probably necessary. In the case of the appearance of a cardiac murmur, the sonography is crucial.
Abstract: Bcl-2 protein expression has been associated with poor prognosis in patients with noncutaneous diffuse large B-cell lymphomas. In primary cutaneous large B-cell lymphomas, the location on the leg, the round-cell morphology defined as the predominance of centroblasts and immunoblasts over large centrocytes, and multiple skin lesions were identified as adverse prognostic factors. The prognostic value of bcl-2 protein expression has not been studied in large series of patients. We evaluated 80 primary cutaneous large B-cell lymphomas collected by the French Study Group on Cutaneous Lymphomas. The prognostic value of age, sex, number of lesions, cutaneous extent, location, serum lactate dehydrogenase (LDH) level, B symptoms, morphology, and bcl-2 protein expression was studied. The overall 5-year specific survival rate was 65%. In univariate analysis, advanced age, multiple skin lesions (n = 48), location on the leg (n = 25), round-cell morphology (n = 32), and bcl-2 expression (n = 39) were significantly related to death from lymphoma. In multivariate analysis, bcl-2 expression (P =.0003), multiple skin lesions (P =.004), and age remained independent prognostic factors. The 5-year specific survival rates in bcl-2-positive and bcl-2-negative patients were 41% and 89%, respectively (P <.0001). A new prognostic classification of primary cutaneous B-cell lymphoma should be based primarily on bcl-2 protein expression rather than the location of skin lesions.
Abstract: INTRODUCTION: Diagnosis of atopic dermatitis currently relies on diagnostic criteria scales developed by Hanifin and Rafka in 1980 and by the "United Kingdom Working party" in 1994. Some authors have proposed: "AEDS" [sM1] and "Atopiform Dermatitis", which has led to the distinction between different sub-populations and the exclusion of certain diseases from the diagnosis of atopic dermatitis. The aim of our study was to collect the criteria retained in the scientific medical literature during the year 2002 for the definition of atopic dermatitis and to try to understand not only the interest but also the questions that the various definitions lead to. METHOD: A PubMed research was launched with the key word "atopic dermatitis" from January to September of 2002. All the scientific articles either in French or in English were studied. RESULTS: Hanifin and Rafka's criteria were selected in 44 p. 100 of the scientific articles, and the "United Kingdom Working Party" criteria in 12 p. 100. Personal definitions were used in 21 p. 100 of the articles; these were based on the level of total and specific IgE or on personal clinical criteria. For twenty-three p. 100 of the authors, the definition of atopic dermatitis was not specified. DISCUSSION: There was not just one definition of atopic dermatitis. This may affect the interpretation of diagnostic or therapeutic papers concerning the disease, because there has been no proof that these definitions cover the same population of patients.
Abstract: Neurofibromatosis 1 (NF1) is a genetically transmitted disease occurring approximately once in 3000 live births and resulting from mutations of the NF1 gene that encodes a protein named neurofibromin, a negative regulator of the ras-dependent pathway. An excess of neoplasia especially tumours of neuroectodermal origin is classically observed. The occurrence of malignant melanoma in patients with NF1 has already been described in scattered clinical reports but little is known as to the characteristics of melanoma arising in NF1 patients. A multicentric retrospective study was conducted on a panel of French referring centres for a period of 13 years to identify patients with both melanoma and NF1. Patients with mucosal or ocular melanoma were excluded. The diagnosis of malignant melanoma was based on specific histology whereas NF1 was identified according to the criteria proposed by the NIH Consensus Conference. All patient fulfilling criteria for both melanoma and NF1 were investigated using a common procedure recording clinical and histological data along with prognostic factors for the two diseases. Eleven patients were identified with both diseases. The clinical pattern of NF1 was quite similar to the classical form of the disease, but some unusual features were present as regards to the melanoma: a sex-ratio of 10 women for one man and an average age lower than expected (median age=33 years) for melanoma occurrence. Among prognostic factors, median thickness was high compared to large series of melanoma in the literature (3.20 versus 1.5 mm). Another neoplasia occurred in three patients. An increase in melanoma incidence in patients with NF1 remains hypothetical but our small series of malignant melanoma arising in NF1 patients displays a large female preponderance, a higher thickness than expected and a frequent association with a second neoplasia. The peculiar female proneness for cancer whatever its localization and the risk of multiple neoplasias have already been reported in NF1 patients and could be true for malignant melanoma as well.
Abstract: BACKGROUND: Agranular CD4+ CD56+ hematodermic neoplasm (blastic NK-cell lymphoma) has been recently described. The skin is often the first organ involved. OBSERVATIONS: Two old men of respectively 70 and 77 years consulted for infiltrated cutaneous lesions. Preliminary histological examination of cutaneous biopsy taken in both patients showed a malignant proliferation suggesting a cutaneous lymphoma, and the patients were referred. Histological examination of new biopsies showed a very similar proliferation in the 2 cases of monotonous medium-sized mononuclear cells without expression of the common antigens CD3 and CD20 and the expression of CD4, CD56, and CD123. No rearrangement of the T-cell receptor gene or the immunoglobulin heavy chain gene were evidenced. No extracutaneous involvement was initially detected in the first patient. Thrombocytopenia associated with the abnormal presence of 15 p. 100 of circulating CD4+ CD56+ cells was initially found in the second patient. The first patient was treated with chemotherapy, with complete remission. A cutaneous relapse promptly occurred, followed by bone and cerebral localizations. The patient died one year after the diagnosis of the disease, in spite of intensification of the treatment. Treatment is still ongoing in the second patient. COMMENTS: The histological presentation of these two patients was very similar with an unusual phenotype of tumor cells expressing CD4, CD56, CD123, but not expressing CD3 and CD20. Some cases have been published under the "term of blastic NK lymphoma" which is the actual term for the disease in the WHO classification. However, the tumor cells derive from the dendritic plasmacytoid cells, also called type 2 dendritic cells, and perhaps from a common precursor to lymphocyte T and dendritic plasmacytoid cells. In spite of complete cutaneous response in the 2 cases presented, as in other reports, extra-cutaneous involvement occurs quickly. Overall survival is usually poor since nearly all the patients died in less than 3 years. This justifies attempting aggressive protocols, with bone marrow allograft in the younger patients.
Abstract: Thin (<or=0.76 mm) level II cutaneous superficial spreading melanomas (SSMs) are known to be of excellent prognosis and very few recur, metastasize, or are lethal. Although many prognostic features at this stage have been studied, none appears to be statistically significant. The concept of tumor growth phase is correlated with Clark's level except for level II. SSM level II show either an invasive vertical growth phase or an invasive radial growth phase. The aim of our study (retrospective, multicenter, and case-control type) was to investigate the prognostic impact of vertical growth phase in thin level II cutaneous SSM. We identified 12 patients of poor outcome with complete initial excision. Each case was matched with three controls for gender, age, location, tumor thickness, and follow-up period since diagnosis. Independent pathologists studied all cases and controls. Univariate analyses were performed with a conditional logistic regression method. A kappa test was used to assess reproducibility between pathologists. Our study is the first and largest that shows that vertical growth phase is the only statistically significant prognostic factor for thin level II cutaneous SSM. We propose that growth phase evaluation (a minimum of eight serial sections being mandatory not to underdiagnose vertical growth phase) should be added to the recommendations for melanoma histologic report, at least for level II SSM.
Abstract: OBJECTIVES: The objectives of this work were a) to assess the feasibility of lymphoscintigraphy in very young children, and b) to assess its usefulness in understanding the underlying pathophysiology. PATIENTS AND METHODS: Forty-one children (age range 3 months-16 years) with primary lymphedema of the lower and/or upper limbs underwent lymphosinctigraphy using 99mTC-labeled colloid (Nanocis or Nanocol) injected subcutaneous at the foot and/or hand level. Recording time, which depended on the child's age, included at least static images during the thirty minutes following injection and four hours later. RESULTS: Abnormal activity of the lymph collectors, nodes, and interstitial space were analyzed according to age (> or < 2 years). Abnormal findings were classed as simple hypoplasia (n=18) or other type of lymph disease: diffuse lymphatic dysplasia (n=6), mixed vascular dysmorphism (n=5), amniotic bride disease (n=1), and hypoplasia revealed by occurrence of a minor factor (n=11). These observations demonstrate the role of lymphoscintigraphy in the diagnosis and prognostic evaluation of lymphedema in children. CONCLUSION: Lymphoscintigraphy is a useful technique for very young children with lymphedema providing further information contributing to overall exploration.
Abstract: BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.
Abstract: INTRODUCTION: Cutaneous lesions are rarely inaugural in Churg and Strauss syndrome. We report the case of a patient in whom recurrent papular lesions of the penis revealed a Churg-Strauss granulomatous angeitis.OBSERVATION: A 54 year-old man consulted for recurrent non-infiltrated and painless erythematous papules of the penis over the past 4 years. His past history revealed severe asthma, which had appeared at the same time, nasal polyposis and chronic sinusitis. Other than the genital organs, the clinical examination was normal. Histological exploration of a cutaneous biopsy of the penis showed a necrotic granulomatous vasculitis. The usual biological examinations were normal. Research for proteinuria, hematuria and measurement of polynuclear anticytoplasm antibodies were negative. Pulmonary X-ray revealed a bilateral interstitial syndrome with micronodules. The broncho-alveolar wash revealed moderate granulocyte alveolitis with numerous eosinophilic polynuclears. The electromyogram showed a reduction in the sensitive potential of the nerves explored in the 4 limbs. Churg-Strauss granulomatous angeitis was diagnosed. Treatment with 0.8 mg/kg/day of prednisone was initiated. The cutaneous lesions disappeared rapidly. Since three months later there had been no relapse, corticosteroid therapy was slowly and progressively reduced. Nine months later, the patient exhibited new papular lesions of the glans penis and progressive relapse of asthma. Treatment with cyclophosphamide (Endoxan(R)) combined with systemic corticosteroids was initiated.DISCUSSION: In view of the concomitance of necrotic granulomatous vasculitis in the cutaneous biopsies, severe, recent corticosteroid-dependent asthma, eosinophil-rich pulmonary infiltrate, allergic rhinitis, chronic sinusitis and the onset of a neuropathy, the diagnosis of Churg-Strauss syndrome was retained. The absence of hypereosinophilia in the blood during diagnosis can be explained by the oral corticosteroid therapy at low doses because of the asthma and sinusitis. However, hypereosinophil blood levels had been reported in several prior hemograms. Damage to the genital organ is exceptional during Churg-Strauss syndrome and is usually of granulomatous prostatitis form. Only one other case has been reported. Our observation is original in that it was isolated to the genital skin, which led to the diagnosis of Churg and Strauss granulomatous angeitis with systemic involvement.
Abstract: INTRODUCTION: Colchicine is a treatment used for aphthous stomatitis but its efficacy has never been proved. We report the results of an open trial of 54 patients treated with colchicine for aphthous stomatitis. The aim of our study was to assess the long and short term efficacy and tolerance of this molecule in this disease. PATIENTS AND METHODS: The case reports of 64 patients presenting with severe, recurrent aphthous stomatitis treated with colchicine between 1986 and 2000 were analyzed retrospectively. Only immunocompetent patients exhibiting idiopathic aphthous stomatitis or Behçet's disease were included. Ten patients were excluded: 4 cases of Behçet's disease treated with colchicine and systemic corticosteroids, 4 cases of aphthous stomatitis secondary to a hematological cause and 2 patients who were lost to follow-up after 3 month's treatment. Colchicine was prescribed at a dose of 1 to 1.5 mg/d for at least 3 months. All the patients were assessed after 3 month's treatment and all were contacted by phone in May 2000. Our end-point criteria were: the frequency and duration of the episodes, intensity of pain and impact on the quality of life of the patients. RESULTS: Fifty-four patients were included: 39 women (mean age=44 +/- 16.8) and 15 men (mean age=49 +/- 13.5). The disease had progressed for a mean of 11.6 +/- 13.5 years. After 3 month's treatment, 12 patients (22 p. 100) no longer had aphta and were in complete remission, 22 patients (41 p. 100) were significantly improved, since the frequency and duration of the lesions had decreased by at least 50 p. 100 and the latter were no longer painful. Treatment failed or tolerance was poor in 20 patients (37 p. 100). Patients were monitored and assessed for a mean of 4.7 years (range: 6 months to 13 years). Six patients were lost to follow-up. Three patients (6 p. 100) were considered as cured, since they were still in complete remission after suspension of colchicine respectively 6, 24 and 72 months later. Fifteen patients (31 p. 100) had still improved. Among the latter, 10 continued colchicine for a mean of 27 months, and 5 patients stopped treatment, which they considered tedious, after a mean of 16 months. These 15 patients noted a significant reduction in the frequency and duration of the lesions. Moreover, the subjective end-point criteria had improved significantly for them not to wish to change treatment. Thirty patients (63 p. 100) were not improved by the treatment. Some benign adverse events were noted in 10 patients (18.5 p. 100) and led to suspension of treatment in 4 cases. DISCUSSION: This study, which is the first in size (n=54) and duration of follow-up (4.7 years), showed that colchicine is an efficient preventive treatment of severe aphthous stomatitis for 63 p. 100 of patients after 3 month's treatment and that this improvement was lasting in 37 p. 100 of them. Colchicine should therefore be proposed in first intention in severe recurrent aphthous stomatitis, since it is effective, well tolerated and easy to use.
Abstract: Primary melanoma (MM) could be a good model to test an intuitive concept: a cancer that is growing fast in its early phase is likely to have a high aggressiveness. Since MMs are visible tumors, many patients can provide information to indirectly assess the kinetics of their lesion. A prospective study was designed to assess if the kinetics of the visible growth of a primary MM, as described by the patient, could be a noninvasive prognostic marker. The ratio of MM thickness to delay between MM appearance and MM removal was used as a surrogate value for the kinetics of the MM growth. To assess the delay between MM appearance and removal, 362 patients with self-detected invasive MM fulfilled a detailed questionnaire, which provided 2 types of estimations of this delay and thus 2 melanoma kinetics indexes (MKI and MKI*). After a median follow-up of 4 years, univariate and multivariate analyses assessed whether relapse-free survival was linked to MKI or MKI*. MKI was significantly predictive of relapse-free survival (HR = 1.84 [1.51-2.25]) and relapse at 1 year (RR = 2.93 [1.84-4.69]), independently from Breslow thickness. MKI was retained in multivariate prognostic models, just after thickness and before other usual markers. MKI* was also a significant independent risk marker, although less predictive. In this model, the initial growth kinetics of a cancer reflects its aggressiveness and a high index predicts a short-term relapse. The "subjective" data obtained from patients about their MM history, although usually neglected, can thus provide a better prognostic marker than many "objective" tests.
Abstract: Application of low-frequency ultrasound has been shown to enhance transdermal drug transport of large molecules such as insulin. In this study, we investigated the dependence of ultrasound-induced transdermal delivery of insulin on ultrasound parameters. Insulin was delivered in vivo to hairless rats using 20 kHz ultrasound applied over a range of ultrasound intensity, application time and pulse length. Change in blood glucose levels of the animals was monitored to assess insulin transport. The results showed a threshold below which no detectable changes in blood glucose level was observed for each ultrasound parameter. Moreover, our findings indicated that sonophoretic enhancement is dependent on energy dose and length of ultrasound pulse that is consistent with a cavitation-based mechanism. The more significant effect of lowering glycemia was obtained with application of less than 15 min ultrasound and was similar to subcutaneous injection of 0.5 U of insulin. Pretreatment of hairless rat skin with ultrasound followed by application of insulin resulted in no significant modification in blood glucose level, indicating that transdermal transport of insulin mainly occurred during sonication. Sonophoresis may therefore potentially be applied for non-invasive and painless delivery of insulin in the treatment of insulin-dependent diabetes.
Abstract: Phonophoresis or sonophoresis is the use of ultrasound to increase percutaneous absorption of a drug. The technique has been widely used in sports medicine since the sixties. Controlled studies in humans in vivo have demonstrated absence or mild effects of the technique with the parameters currently used (frequency 1-3 MHz, intensity 1-2 W/cm(2), duration 5-10 mins, continuous or pulse mode). However, it was demonstrated in 1995 that administration of macromolecules with conserved biological activity was feasible in animals in vivo using low frequency ultrasound. This led to new research into this method of transdermal administration. The aim of this review is to present the main findings published with low frequency and high frequency ultrasound over the last ten years, and to discuss the respective roles of thermal, cavitational and non-cavitational effects on the reduction of the skin barrier. Particular attention is paid to the biological effects on living skin which might be of importance for tolerance and practical use in humans.
Abstract: The use of low-frequency ultrasound has been proposed to enhance the transdermal transport of various drugs, a technique referred to as sonophoresis. The aim of the present study was to determine the safety of low-frequency sonophoresis on human and rat skin by evaluating their structural modifications after ultrasound exposure. Human skin samples and hairless rats were exposed to 20 kHz ultrasound in vitro and in vivo, respectively. Ultrasound was used with average intensities ranging from 0.25 to 7 W/cm(2) in pulsed or continuous mode. Hairless rats were also exposed to a heat source mimicking the temperature versus time profile during sonication. Skin samples were observed under optical and electron microscopy to detect any structural changes. Human skin samples exposed to intensities lower than 2.5 W/cm(2) showed no modification. For hairless rats, slight and transient erythema was observed after 2.5 W/cm(2) exposure, whereas deep lesions (dermal and muscle necrosis) were observed 24 hr later. These lesions were also observed when a plastic film was placed between the coupling medium and the animals' skin during sonication. In contrast, no histologic lesion could be seen when a heat source was applied to animal skin. Low-frequency ultrasound induces delayed and deep lesions in hairless rat skin at 2.5 W/cm(2) which are not only attributable to the increase in temperature at the skin surface during ultrasound exposure. By using the same ultrasound conditions, human skin seems to be less sensitive in vitro.
Abstract: Acute and long term effects are frequent after radiotherapy. They may alter the general status and quality of life of the patients. Chronic radiodermatitis may result in ulceration and in transformation into a squamous cell carcinoma. There is a correlation of the frequency of acute dermatitis with the total dose. Chronic radiodermatitis may develop after repeated small doses of ionizing radiation for cardiac catheterization and coronary angioplasties. The other prognostic factors for the level of acute and late skin reactions are volume of tissue treated, total daily dose, fractionactions schemes ... but there are some variation in the degree of reaction in patients treated with identical radiotherapy schedules. There is a patient-to-patient variability. Several diseases as systemic sclerosis, some genetic diseases, perhaps some drugs may increase the cutaneous reactions. So both acute and chronic irradiation injury is a complex process with many regulations. Chronic fibrosis may be caused by mechanism of cell activation (and particularly fibroblasts). Cytokines e.g. transforming growth factor beta (TGF-beta) might be involved in the induction of fibrosis. Treatment use emollients. Superoxide dismutase was used as an ointment for radiofibrosis therapy and obtains a reduction of the fibrosis. In late phases plastic surgery or sometimes cryosurgery can be used.
Abstract: Acute bronchial mucosal sloughing related to Toxic Epidermal Necrolysis (Lyell syndrome) is widely reported in literature. On the contrary severe respiratory involvement is rare in post-infectious or toxic Epitheliolysis (Stevens-Johnson syndrome). There is no well-known predictive sign of bronchial epithelium involvement. An 18-year-old patient was admitted for Stevens-Johnson syndrome related to sulfasalazine (salazosulfapyridine). There were no respiratory signs. An acute respiratory failure occurred 36 hours after from admission due to an obstructive and desquamative necrosis of the tracheobronchial epithelium. We purpose that a fiberoptic laryngoscopy should be performed even in non-dyspneic patients suffering from Stevens-Johnson syndrome if hypersecretion is present. Fiberoptic bronchoscopy can be helpful in these cases.
Abstract: The effect of low-frequency sonophoresis on fentanyl and caffeine permeation through human and hairless rat skin was studied in vitro. Experiments were performed using 20 kHz ultrasound applied at either continuous or discontinuous mode and with an average intensity of 2.5 W/cm(2). The results showed that low-frequency ultrasound enhanced the transdermal transport of both fentanyl and caffeine across human and hairless rat skin. This was explained by both increasing flux during sonication and shortening the lag time. Discontinuous mode was found to be more effective in increasing transdermal penetration of fentanyl while transdermal transport of caffeine was enhanced by both continuous and pulsed mode. Histological and electron microscopy studies showed that human and hairless rat skin was unaffected by ultrasound exposure. Further studies will be necessary to determine the relative contribution of ultrasound parameters in low-frequency ultrasound-induced percutaneous enhancement of drug transport.
Abstract: BACKGROUND: Granuloma faciale is a rare condition of unknown pathogenesis. Treatment often gives less than satisfactory results. We report two cases successfully treated with cryosurgery. CASE REPORT: A 42-year-old woman had a round 65 x 40 mm erythematous violet-colored papulous plaque on the right cheek. Histology reported a dense inflammatory infiltration of the superficial and mid derma around dilated vessels under a normal epidermis. The infiltration was composed of histiocytes, lymphocytes, plasma cells, neutrophils and eosinophils and was compatible with granuloma facial. Surgical treatment was performed but was followed by recurrence within a few months. Class I local steroids, cotton swab cryotherapy and oral dapsone remained ineffective. Cryosurgery however led to cure without recurrence at 1 year follow-up. The second patient was a 52-year old man with an oval reddish-brown papulous plaque on the right preauricular area. The plaque measured 40 mm in diameter and histology favored granuloma facial. Three cotton swab cryotherapy sessions and one surgical excision procedure were followed by recurrence. Cryosurgery provided successful cure without recurrence at 3.5 years follow-up. DISCUSSION: Most destructive treatments for granuloma facial, including pulverization cryotherapy, surgery, and CO(2) or argon laser are unsuccessful in preventing recurrence. Cryosurgery was proposed in 1977 but few cases have been reported since. Medical treatments have been variously successful with most authors reporting unsatisfactory results. In our two cases, surgical excision was rapidly followed by recurrence and in one case dapsone was ineffective. Cryosurgery provided rapid regression of the lesion and recurrence-free cure at 1 and 3.5 years follow-up respectively. Cryosurgery is an effective treatment for readily recurrent eosinophilic granuloma facial and should be proposed as first intention therapy.
Abstract: A prospective survey was conducted to assess the role of patients in the melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a comprehensive questionnaire including a psychological instrument. Main outcome measures were the delay before medical intervention and the tumor thickness. Of 590 melanomas, 70.8% were detected by patients and this proportion was higher in females. Relatives were involved in the detection of half of the cases. Median delays before the patient realized he had a suspicious lesion, before this lesion was seen by a doctor, and before the melanoma was removed were 4 months, 2 months, and 1 week, respectively. Delays up to several years were observed in some cases. The rate of self-detection tended to be lower, the delays before seeking medical advice to be longer, and the tumor thickness to be higher in old people, in males, in lower-educated individuals, in those living out of towns, and in people with a low awareness about melanocytic tumors than in other cases. Conversely, individuals with a high number of atypical nevi, those who were aware to be at risk, and those who regularly visited a dermatologist tended to detect their melanoma more rapidly. No specific psychological traits were associated with a late reaction, although negligence and anxiety tended to prolong the delays. Knowledge about melanoma was poor in many patients, especially in males, and wrong beliefs were widespread. This study provides the targets of future education programs.
Abstract: A prospective survey was conducted to assess physician responsibility in melanoma prognosis. Consecutive patients with primary melanoma were interviewed and examined using a standardized questionnaire. Main outcome measures were medical components of the delay before tumor resection and tumor thickness. Of 590 melanomas, 29.1% were coincidentally detected by physicians and their tumor depth was lower than in melanomas detected by patients (p < 0.001). Physician sensitivity for melanoma diagnosis was evaluated at 86%. Median time intervals to propose resection and to perform removal of melanoma were short: 0 (mean 103) and 7 (mean 68) days, respectively. Melanomas were managed in an inappropriate way in 14.2% of cases. Location on acral areas and absence of pigmentation were associated with longer medical delays and more frequent inappropriate medical attitudes. Melanomas located on hardly visible areas were less frequently detected by physicians than those on visible areas. Medical delays were shorter, doctor's attitude was more frequently appropriate, and melanoma thickness was lower (p < 0.001) when the patient visited a dermatologist (54.7%) than when he or she visited a general practitioner (33.4%). Our study shows that doctor responsibility accounts for only a small part of the total delay before melanoma removal. However, systematic total examination and better training of doctors, especially about unusual forms of melanoma, could still improve melanoma detection.
Abstract: BACKGROUND: Extracutaneous involvement is rare in the classical form of Kaposi sarcoma (KS). OBSERVATION: We report a case of bone involvement revealed by bone pain. Magnetic resonance (MR) images demonstrated the local invasion of bone from cutaneous lesions. Bone biopsy confirmed bone involvement. The patient was treated with vindesine. Bone pain progressively disappeared. CONCLUSION: Bone involvement has rarely been reported in classical KS though 4.5% of the patients were affected when it was systematically sought. Treatment of symptomatic lesions requires radiotherapy or chemotherapy.
Abstract: BACKGROUND: D-penicillamine can induce autoimmune disease, particularly in patients with associated immune disorders. CASE REPORT: A 67-year old woman who had been taking D-penicillamine for 15 months for rheumatoid arthritis was hospitalized due to the development of a bullous eruption and proximal muscle deficiency. Search for intercellular antisubstance antibodies in serum was negative. The skin biopsy histology revealed intra-epidermal cleavage in the mucosal body and direct immunofluorescence revealed epidermal frame-marking with anti-IgG and anti-C3 antibodies. Other tests revealed muscular cytolysis, and anti-acetylcholine receptor antibodies. The electromyogram showed neuromuscular block without muscle deficiency and muscle biopsy showed moderate myositis. D-penicillamine was interrupted and was followed by cure of the pemphigus and aggravation of the myositis, requiring high-dose systemic corticosteroid therapy. DISCUSSION: This patient developed D-penicillamine induced pemphigus, a rather frequent observation. The desmoglein immunolabelling favored drug-induced pemphigus and the course was rapidly favorable after withdrawal. Pemphigus had developed simultaneously with signs of myasthenia and polymyositis. Polymyositis and myasthenia are also known complications of D-penicillamine therapy. The association of these three complications suggests that D-penicillamine can unmask certain antigens or have an immunomodulator effect.
Abstract: Diagnosis of exercise-induced anaphylaxis is based on conjunction between a specific factor: a specific or nonspecific food allergy and exercise. The authors report observation of a patient who presented with exercise-induced anaphylaxis associated with food allergy to spinach, but also with a cross reaction with latex.
Abstract: BACKGROUND: Cutaneous periarteritis nodosa (PAN) is distinguished from systemic PAN by the lack of visceral involvement. The aim of this study was to describe the clinical presentation, laboratory findings, clinical course, and treatment in cutaneous PAN. PATIENTS AND METHODS: We retrospectively reviewed the files of patients hospitalized for vasculitis in our Dermatology unit where approximately 20 cases of vasculitis are seen each year. Inclusion criteria were skin signs suggestive of PAN and a histological image of leukocytoclastic vasculitis of an arteriole. RESULTS: Nine cases of cutaneous PAN were treated in our unit between 1976 and 1997. Follow-up ranged from 32 months to 22 years. No cases of systemic PAN had been diagnosed during this period. These 9 cases of cutaneous PAN all had the same clinical presentation: nodules on the lower limbs in all cases associated with nodules on the upper limbs in half of the cases. Neuropathy was found in 3 of the 9 cases. No systemic involvement was observed. The most frequently used treatment protocol was general corticosteroid therapy (0.5 mg/kg/d prednisone or prednisolone). Immunosuppressive drugs, colchicine, dapsone, non-steroidal anti-inflammatory drugs and intravenous immunoglobulins were also used with efficacy. DISCUSSION: Cutaneous PAN is a particular form of vasculitis associating skin signs with locoregional neuromuscular involvement. The differential diagnosis with other types of vasculitis is sometimes a difficult task. The clinical course is the fundamental diagnostic clue in cutaneous PAN. A benign course and the absence of visceral involvement allow initiating a symptomatic treatment such as colchicine. The development of neuromuscular signs may warrant the use of general corticosteroid therapy.
Abstract: OBJECTIVE: To test the basic assumption of campaigns for early diagnosis of melanoma, ie, prognosis is correlated with the delay in the diagnosis. DESIGN: Prospective study of the correlation between delays to diagnosis, assessed using a questionnaire, and the Breslow thickness as a prognosis marker. SETTING: Dermatology departments in France. PATIENTS: Five hundred ninety consecutive patients, referred within 12 weeks after resection of cutaneous melanoma. MAIN OUTCOME MEASURES: Assessment of 5 successive time intervals from the first time the patients realized that they had a lesion until the resection of the melanoma, and results of the correlation between each time interval and tumor thickness (Breslow). RESULTS: There is a positive but weak correlation between tumor thickness and the delay to identify a lesion as suspicious (r = 0.17; P = .009). However, this delay tends to be short for the thickest tumors. There is a negative correlation between tumor thickness and the delay to seek medical attention (r = -0.20; P<.001). This delay was shorter for nodular melanoma. No correlation is found between melanoma thickness and physicians' delays. CONCLUSIONS: Poor prognosis can be accounted for by aggressive rapidly growing tumors rather than by delays. In well-informed populations, campaigns for early diagnosis of melanoma may thus no longer have a major impact on prognosis, unless they are focused on subgroups less accessible to information and medical care.
Abstract: Sjören syndrome favors the development of lymphoma, particularly in the salivary glands with MALT lymphomas. The differential diagnosis with benign lymphoepithelial sialadenitis can be difficult. A 78-year-old woman had an oculo-buccal sicca syndrome for 10 years and developed parotid hypertrophy. The first biopsy, performed 7 years before the present investigation had showed chronic lymphoepithelial sialadenitis. A second biopsy showed MALT lymphoma. Search for extension revealed a second gastric localization of the lymphoma. This patient had a particular immunophenotype, showing a CD5+ tumoral population frequently observed in mantel lymphomas and usually lacking in MALT lymphomas. Recently, however, another case similar to our own, has been reported in the literature. The observations raise the problem of distinguishing between mantel lymphoma and MALT lymphoma.
Abstract: BACKGROUNDS: Multiple cutaneous cylindromas are uncommonly associated with extracutaneous cylindromas. An association between cutaneous cylindromas and salivary gland cylindromas would probably not be fortuitous. CASE REPORT: A 66-year-old man developed multiple cutaneous cylindromas and cylindromas situated in the parotid and submaxillary gland. Histology was similar for both localizations. DISCUSSION: This association was probably not fortuitous as the histology was similar, other cases have been reported in the literature, and recent complaint concerned the development of multiple cutaneous cylindromas and salivary gland cylindromas. Salivary gland cylindromas should be searched for in patients who develop cutaneous cylindromas as they are more aggressive in nature.
Abstract: BACKGROUND: Radiodermatitis after cardiac catheterization procedures has been reported in only a few cases in the literature. This side effect may not however be uncommon after long or repeated endocavitary explorations. CASE REPORT: We observed a case of chronic radiodermatitis which developed after cardiac catheterization, in a patient treated with corticosteroids and ciprofibrate for lupus. The patient had undergone only one heart catheterization. A second catheterization performed when the patient had stopped ciprofibrate did not provoke new lesions. DISCUSSION: This observations would suggest that ciprofibrate could participate as a radiosensitizing factor in the development of radiodermatitis.
Abstract: BACKGROUND: Tufted angioma, described by Wilson Jones in 1976, is a benign acquired vascular tumor occurring in children or young adults, usually located on the neck or the upper part of the thorax. Pathology examination confirms the diagnosis showing well-limited lobules in the dermis composed of tight clusters of capillaries without atypical cells. CASE REPORT: An 81-year-old woman consulted for a large extensive angiomatous lesion involving the neck and shoulder which had developed over two years. Histopathology reported tufted angioma. Treatment with pulsed dye laser was unsuccessful. DISCUSSION: Different treatments have been proposed for tufted angiomas: surgery, cryotherapy, interferon, argon laser and pulsed dye laser. This is a second cases of unsuccessful treatment, perhaps due to deep extension of the angioma.
Abstract: OBJECTIVE: We conducted a prospective evaluation of the contribution of high-resolution ultrasound imaging prior to cryosurgery for basocellular carcinoma and in search for recurrence. PATIENTS AND METHODS: All patients seen between 1992 and 1994 at the skin tumor clinic and treated by cryosurgery were included. Ultrasound imaging using 20 MHz prototype was performed prior to cryosurgery and 2 months later. RESULTS: Among 101 patients treated, 112 basocellular carcinomas were treated by cryosurgery. Ultrasound imaging provided good visualization of the tumor limits in all cases. The ultrasound aspect was anechogenic, often with rare areas of highly dense echoes. The tumor limits described by ultrasound imaging were larger than the clinical limits in 32% of the cases. In 8 of the 16 cases of recurrent tumors, the ultrasound examination revealed the recurrence first. In the other 8 cases, clinical manifestations were confirmed by ultrasonography. In our series, recurrence of basocellular carcinoma was statistically more frequent when the depth of the tumor was 3 mm (ultrasonographic measurement) or when the lateral limits established by ultrasound assessment were greater than the clinical evaluation. DISCUSSION: These findings demonstrate that high-resolution ultrasound imaging of basocellular carcinomas prior to cryosurgery: 1) visualizes tumor limits allowing adapted cryosurgery, 2) identifies factors with predictive value for recurrence, 3) can identify recurrences early. Ultrasound imaging of the skin is a useful non-invasive technique for pre- and post-therapeutic assessment of skin tumors and could be a particularly useful tool for "blind" cryosurgery destruction of skin tumors.
Abstract: INTRODUCTION: Mycobacterium chelonae is an ubiquitous mycobacterium which is exceptionally pathogenic. It can cause localized skin infections which are difficult to treat because of multiple drug-resistance. CASE REPORT: A 87-year-old woman with bullous pemphigoid was treated with long-term prednisone and developed a skin infection due to M. Chelonae. Treatment with clarithromycin was successful, but relapse occurred 6 months after withdrawal of the antibiotic. DISCUSSION: Clarithromycin is effective in single drug regimen for M. Chelonae infection, but the duration of treatment should be long when immunosuppression cannot be avoided.
Abstract: INTRODUCTION: Dapsone is a drug widely prescribed in dermatology. It can lead to undesirable side effects including hematologic disorders. CASE REPORT: A patient with dermatitis herpetiformis was treated with dapsone. Agranulocytosis developed 6 weeks later and was revealed by fever and pneumonia which resolved under antibiotics. The agranulocytosis regressed without specific treatment after drug withdrawal. Blood counts returned to normal 2 weeks later. DISCUSSION: Dapsone-induced agranulocytosis is in common (1/10,000 to 1/20,000). The drug is widely prescribed, particularly for leprosy. Nevertheless, when dapsone is prescribed for dermatitis herpetiformis, the risk of agranulocytosis increases 25-fold reaching 1/400. The indication for dapsone must always made carefully, with surveillance of the blood counts every 15 days during the first 3 months of treatment for dermatitis herpetiformis.
Abstract: Alternaria is a very common and saprophytic fungus. Cutaneous infection is rare and about 71 cases have been described, mainly in Europe in immunocompromised hosts. We report a case of dermal alternariosis occurring in a woman treated with corticosteroids for dermatomyositis. The cutaneous lesion consisted of an erythematous and scaly plaque on the leg measuring 2 x 2 cm. Cutaneous biopsy showed hyphae and round inclusions stained with PAS and Gomori-Grocott within a polymorphous granuloma. Cultures of cutaneous biopsies grew Alternaria sp. HIV1 and HIV2 serology was negative. The patient was treated by local excision and corticosteroids were decreased. One-year follow-up showed no recurrence. Cutaneous alternariosis is an opportunistic infection. the disease has been described mainly in patients treated with systemic corticosteroids (39 cases out of the 71 reported cases) or local corticosteroids (3/71) and in patients suffering from Cushing's syndrome (7/71) but rarely in HIV-infected patients (3/71). Cutaneous fragility induced by hypercorticism is an important cofactor permitting direct inoculation from the environment.
Abstract: INTRODUCTION: Cutaneous polyarteritis nodosa is a subset of polyarteritis nodosa (PAN) characterized by a good prognosis because of the absence of severe visceral involvement. Dapsone, colchicin, non steroidal anti-inflammatory drugs are the first line treatments of cutaneous PAN. Corticosteroids are an alternative therapy. CASE-REPORT: A case of cutaneous PAN followed for 6 years is reported. Dapsone, colchicin, corticosteroid and cyclophosphamide have failed to control cutaneous lesions. Intravenous immunoglobulins 400 mg/kg/d five day monthly for 5 months was successful, but a cutaneous relapse occurred 3 months later which was another time controlled with intravenous immunoglobulins. DISCUSSION: Some cases of necrotizing vasculitis have been treated with intravenous immunoglobulins. This is the first reported case of cutaneous PAN treated with intravenous immunoglobulins. Because of the lack of controlled studies, the indications of intravenous immunoglobulins in vasculitis are restricted to those resistant to corticosteroids and immunosuppressive therapy.
Abstract: Candida parapsilosis is a very common and saprophytic fungus. Its pathogenic role has been reported in intensive care units after invasive instrumentations. We report a case of dermal candidiasis due to Candida parapsilosis occurring in an immunocompromised patient suffering from aplastic anemia treated with corticosteroids. The histopathological aspect was non specific, showing a polymorphous granulomatous reaction in the middermis. Findings of periodic acid-Schiff and Grocott-Gomori stains revealed a large number of pseudohyphae and spores. Candida parasilosis was cultured from a skin biopsy. The route of skin infection is discussed: direct inoculation or septic localization after fungemia.
Abstract: BACKGROUND. Although cutaneous photosensitivity is one of the major criteria used for the diagnosis of systemic lupus erythematosus, this criterion is not precise. OBJECTIVE. To evaluate the interest of photobiological exploration in patients with cutaneous lupus erythematosus, and to compare the results to clinical photosensitivity. METHODS. Nineteen patients consecutively seen in one year, have been tested using a standardized method. RESULTS. Clinical photosensitivity was present in 16 cases. The clinical presentation of cutaneous disease was classified in 3 groups: acute, subacute, and chronic. One patient with no history of photosensitivity had positive photobiological tests, and five patients had negative tests though they experienced clinical photosensitivity. Positive phototests with UVA and UVB were present in 6/8 subacute lupus cases, 3/6 acute lupus cases, 3/5 chronic lupus cases. Histological aspect of cutaneous biopsies from phototest was not characteristic of lupus. Minimal erythematous doses was comparable in lupus group and in controls. No correlation between positivity of phototests and the presence of systemic involvement or anti-Ro/SSA antibodies was established. CONCLUSION. Phototesting is useful to assess photosensitivity in lupus patients.
Abstract: We have studied changes in the elasticity and viscosity of the skin in patients with lymphoedema, using a technique involving vertical extensibility by suction. We measured parameters which included immediate extensibility (Ue, which reflects the elastic properties of the skin), and delayed extensibility (Uv which reflects intracutaneous movements of a viscous type). In grade III lymphoedema, Ue is decreased and Uv is increased. These changes are explained by volume variations and histological alterations, and tend to normalize after treatment. Our study shows that measurement of extensibility is useful in evaluation of volume variations, effects of therapy, and disease evolution, as Ue variations correlate with volume variations and with Uv changes. This technique also provides information which is useful in assessing patients' functional difficulties in relation to skin infiltration, and might be of value in lymphoedema follow-up.
Abstract: BACKGROUND. The aim of our study was to image psoriasis plaques by ultrasound to assess the changes in psoriasis and to measure and quantify them objectively. MATERIALS AND METHODS. Thirty-one psoriasis plaques were studied in 19 patients. Measurements of skin thickness were obtained with a high resolution B-mode echographic system. RESULTS. Some changes were seen in psoriatic skin. A new structural element was observed: a wide subepidermal nonechogenic band. The other changes were a decrease in dermal echoes that were less intense and less dense, and an increase in the epidermal and dermal skin thicknesses. The skin thickness was increased in all psoriasis plaques as compared to apparently normal skin (P < 0.001). The average increase was 67% for whole skin and 200% for epidermis. CONCLUSIONS. Ultrasound imaging of psoriatic skin allowed the identification of different skin changes induced by psoriasis, and particularly, the differentiation between epidermal and dermal alterations. We presume that epidermal thickness reflects epidermal proliferation and desquamation, and the increase in the dermal and whole skin thickness reflects infiltration. We feel that ultrasound imaging of psoriatic skin is a quantitative method that is as easy and noninvasive as the psoriasis area and severity index (PASI). It could be used for following up patients with psoriasis and could achieve widespread use, especially in research protocols.
Abstract: Infections due to Actinomycosis species are located in the cervico-facial region in 50 to 65 percent of the cases. Extra-cervical cutaneous lesions are exceptional. The most frequently encountered germ is Actinomyces israeli, observed in 85 percent of the cases. We report the case of an Actinomyces meyeri infection which presented as a leg abscess and a pulmonary lesion. There was no cervico-facial localization. There was however a chronic parodontitis. A second germ, Capnocytophaga sp. was isolated from the abscess. This case is of particular interest because of the extracervical localization and the rare species isolated (17 other cases of Actinomyces meyeri infection have been reported). The mechanism of the infection can be better understood in light of pulmonary lesions in the lower right lobe due to inhalation and the coexistence of a buccodental germ in the culture of the leg abscess: buccodental origin of the germ, pulmonary lesion secondary to inhalation, septicaemic dissemination with cutaneous metastases.
Abstract: Vertical cutaneous extensibility was studied before and after 1, 2, 5 and 10 min hydration by application of tap water. After hydration, we noted an increase in all rheological parameters linked to elasticity whereas viscosity and hysteresis parameters were unmodified. These modifications took place from the first minute and increased thereafter. Men and women showed identical values prior to hydration. After hydration, an extensibility gain was noted only in the women, men's extensibility being unmodified. Studying rheological behaviour as a function of age, we showed similar modifications in younger and older groups, the extensibility gain being greater in the older group. Prior to hydration, the stratum corneum was extremely rigid and extensibility was comparable between men and women. Hydration, softening the horny layer, allows a rapid extensibility gain proportional to the reduced thickness of the dermis, especially in women and older subjects.
Abstract: Xanthosiderohistiocytosis is a rare non-Langerhans histiocytosis (4 reported cases). The clinical characteristics include diffuse, sometimes deep, dark-brown infiltrations of the skin. Histological examination reveals abundant deposits of haemosiderin within the histiocyte proliferation. This entity is a clinical form of Montgomery's disease (xanthoma disseminatum) which has been reported in over 100 cases. Our case of disseminated xanthosiderohistiocytosis was particular because it involved the heart and was associated with a monoclonal gammapathy. Five cases have been reported associating xanthoma disseminatum and monoclonal gammapathy, including one case of xanthosiderohistiocytosis. In our case, rapidly increasing levels of monoclonal immunoglobulin suggested an evolution towards a myeloma. A monoclonal gammapathy should be looked for and monitored in cases of xanthosiderohistiocytosis, and more generally xanthoma disseminatum because of the risk of developing lymphoma or myeloma.
Abstract: Autoimmune bullous diseases have been reported associated with different causal factors: drugs, mechanical trauma and physical trauma, particularly ultraviolet light and radiotherapy. In these cases different hypotheses regarding the pathogenesis of blister formation can be supported. In this observation, we report a localized cicatricial pemphigoid with unusual clinical presentation. Moreover, it appeared 9 years after radiotherapy for breast carcinoma and it was preferentially localized on an upper limb lymphoedema. Because of the long time between the treatment of carcinoma with radiotherapy and the onset of pemphigoid, we assume that lymphoedema played a major etiological role in this particular cicatricial pemphigoid.
Abstract: Schnitzler's syndrome, first described in 1974, is defined by chronic non-pruritic urticaria, osteocondentation, and a monoclonal IgM dysproteinemia, but without criteria of lymphoproliferative disease. We report a patient with chronic urticaria and macroglobulinemia. In addition, he had double monoconal dysproteinemia IgM kappa (31.3 g/l) and IgA lambda, osteocondensation, and some cutaneous lesions of pseudoxanthoma elasticum. Only 20 cases of Schnitzler's syndrome have been reported hitherto. This is the first case associated with pseudoxanthoma elasticum, which was localized and discovered at the same time as Schnitzler's syndrome. We discuss the possible role of monoclonal immunoglobulin in the occurrence of localized elastorrexhis.
Abstract: Eight patients, six men and two women, had chronic photosensitivity of 2.0 +/- 1.1 years' duration. The clinical and photobiological signs were consistent with a diagnosis of chronic actinic dermatitis syndrome. Photosensitivity was extremely severe, and the minimal erythematous dose (MED) in polychromatic light was dramatically decreased in all eight patients (82 +/- 20 mJ/cm2; range: 25-200 for a normal MED range of 1.000 to 2.000 mJ/cm2). Photopatchtests were positive to phenothiazine in four patients, to fragrance mix and oxybenzone in two patients, to balsam of Peru and musk ambrette in one patient each. Seven patients were treated with corticopuvatherapy. They all were markedly improved after one month of treatment, recovering normal MED and outdoor activities. Corticosteroid therapy was gradually reduced and stopped after 3 months, while puvatherapy was continued for several months. Treatment was withdrawn in three patients. The minimal erythematous dose was monitored after discontinuation of therapy: a progressive decrease of MED was observed, accompanied by a relapse in two of the three patients. This relapse was well controlled by another course of corticopuvatherapy. Four other patients had, after discontinuation of treatment, another course of corticopuvatherapy reinstituted in the early spring. Puvatherapy was stopped at the end of october. One patient did not receive corticopuvatherapy because she was living far from a puvatherapy centre. She was treated with azathioprine for 6 months without improvement, then with azathioprine+prednisolone 20 mg daily during 2 years with slight improvement. Corticopuvatherapy is a very efficient means of treating severe chronic actinic dermatitis.(ABSTRACT TRUNCATED AT 250 WORDS)
Abstract: The purpose of this study was to determine the frequency of contact allergy and photocontact allergy to sunscreens. A consecutive series of 54 patients with suspected clinical photosensitivity were assessed. All had the same standardized photobiological investigation from January 1989 to December 1990, including patch tests and photopatch tests with 6 sunscreen agents. Oxybenzone was found to cause 4 cases of allergic contact dermatitis (with photoaggravation in 2), and 3 cases of photocontact dermatitis (13% of patients). This is probably due to the wide distribution of oxybenzone in sunscreens and other cosmetics, 2 patients with polymorphic light eruption and persistent light reactivity, respectively, were regular sunscreen users. Photobiological investigation is necessary to ensure accurate diagnosis, since sunscreen contact or photocontact allergy may simulate other photosensitivity eruptions.
Abstract: Carpal tunnel syndrome (CTS) was observed in 4 cases of a consecutive series of 16 patients admitted for the initial diagnosis or follow-up of systemic scleroderma from 1986 to 1990. We also observed one case of ulnar nerve compression. Neurological involvement was confirmed by electromyogram, and motor and sensory nerve conduction studies. The neurological signs appeared at the beginning of the disease in two cases and preceded the discovery of scleroderma in two. CTS is not rare in scleroderma and must be carefully sought, both clinically and electrophysiologically. Patients with 'idiopathic' CTS might need clinical follow-up for early diagnosis of scleroderma.
Abstract: The fusidic acid steady-state concentrations in serum (S) and skin suction blister fluid (SBF) after oral doses of 250 and 500 mg administered twice daily for 6 days, as film-coated tablets of sodium fusidate, were studied in eight healthy subjects. The mean peak serum concentrations after the 250- and 500- mg regimens were 39 +/- 5 and 102 +/- 11 mg/l, respectively, obtained between 2 and 3 h after drug intake. The corresponding values for SBF, obtained later, between 2 and 12 h after drug intake, were 21 +/- 5 and 79 +/- 11 mg/l. As measured by the ratio of area under the concentration vs. time curve (SBF/S), fusidic acid penetration is 69-75%, whatever the dose. With either regime, the fusidic acid SBF and serum levels exceed the minimal inhibitory concentration of bacteria usually found in skin infections, especially Staphylococcus species. A dose of 250 mg twice a day appears sufficient to treat these infections and could be tested in clinical studies.
Abstract: Transdermal nitroglycerin is commonly used and may induce contact dermatitis. The frequency of adverse skin reactions is controversial and may vary from 10% to 75%, according to various authors. 33 patients using transdermal nitroglycerin for more than 7 days were examined and patch tested (nitroglycerin 0.5% aq., 2% pet. and TTS in toto). 5 patients (15%) had adverse reactions. The patch tests were all negative in the 33 patients. Contact dermatitis occurs in many cases, about 15% of the cases with the newly available TTS, and even if patients respect the conditions for using TTS. These contact dermatitides are mainly irritant reactions and do not require transdermal nitroglycerin treatment to be stopped. Nevertheless, since some cases of allergic contact dermatitis have been reported, a contact dermatitis from transdermal nitroglycerin should lead to patch testing.
