Abstract: Isotretinoin is the most effective treatment for severe acne, but there are several adverse effects associated with its use, some of them very exceptional (<1/10000). We report one case of hypoacusia and tinnitus in a 15-year-old boy treated with isotretinoin during 6 weeks, who quickly improved after isotretinoin withdrawal. Also, we comment other publications about hearing alterations in patients treated with isotretinoin and other retinoids.
Abstract: Cutaneous melanoma in childhood (CMC) is rare; therefore, its prognostic factors and biologic behavior, and the effectiveness of adjuvant techniques for CMC remain mostly unknown.
Abstract: The development of a second haematological disease during the course of systemic mastocytosis is a well-known phenomenon. In most of the cases, they consist of myelodysplasia or myeloproliferative disorders. The association with lymphoproliferative disorders has also been described, but it is uncommon and the relationship is not well established. We report a patient diagnosed with systemic mastocytosis who three years later developed a splenic marginal zone lymphoma with villous lymphocytes.
Abstract: The withdrawal of marketing authorization for efalizumab by the European Medicines Agency in February, 2009 provided a unique opportunity to assess the course of disease in patients who were not subject to the selection criteria and biases that were common in the pivotal trials. The aim of this study was to evaluate the course of psoriasis following forced suspension of efalizumab in a group of patients treated in normal clinical practice. As secondary objectives, we sought to assess the relationships between clinical characteristics, treatment response, and disease course during efalizumab treatment and 12 and 24 weeks after suspension.
Abstract: Poststeroid panniculitis is a rare disorder usually reported in children after a sudden decrease or withdrawal of corticosteroid therapy. We report a case in an adult, a finding very rarely reported in English literature. The case report is about a 34-year-old man with multiple erythematous, firm and tender subcutaneous nodules on both thighs and legs after the withdrawal of long-term doses of dexamethasone prescribed before and after surgery for a frontoinsular anaplastic oligodendroglioma. Histopathologic study revealed mainly lobular and also septal panniculitis with fat necrosis and characteristic needle-shaped clefts in radial arrangement within fat cells and multinucleated giant cells. The lesions resolved in about 5 months, after weight loss and application of topical corticosteroids for 1 month, leaving only residual hyperpigmentation.
Abstract: During the last decades, the progressive ageing of the population has resulted in a rising skin cancer incidence. Although previous studies detected no higher morbidity for dermatological surgery in senior patients, their exclusion from optimal surgical treatment remains as a common clinical practice.
Abstract: Melanoma is an uncommon tumour in childhood. Only isolated cases in pregnant patients younger than 18 years old have been previously described, therefore the biological behaviour of cutaneous melanoma in this group of age remains largely unknown. We report a single-institution experience with three patients who developed cutaneous melanoma before the age of 18 years and became pregnant concomitantly or during the course of the disease. High tumour thickness was attributed to later diagnosis and could be responsible for the aggressive biological behaviour in these patients. This delay was in part due to patients considering changes in nevi to be normal during pregnancy. The effect of pregnancy on the prognosis of patients with melanoma, particularly the role of hormonal and immunological factors on clinical outcome, survival and risk of developing metastases, as well as the differences between adolescent and adult populations are still controversial. Since there are no specific treatment strategies for this group of patients, treatment recommendations should be established according to adult experience.
Abstract: Cutaneous melanoma in childhood is rare; therefore, its prognostic factors and biologic behavior and the effectiveness of adjuvant diagnostic techniques in this group remain mostly unknown.
Abstract: Interferon is used to treat hepatitis C virus infection and its cutaneous side effects are well known. Recently, interferon-induced sarcoidosis has been reported. We report a new case of sarcoidosis during pegylated interferon alfa and ribavirin treatment with an unusual presentation in a woman with previous episodes of erythema nodusum and nodular vasculitis related to HCV.
Abstract: Background. Eosinophilic dermatosis of hematologic disorders is a reactive process that may cause a variety of clinical manifestations. Methods. We report a patient who had outbreaks of skin lesions since the onset of chronic lymphocytic leukaemia. Results. The cutaneous eruptions began as eosinophilic panniculitis and after changed to insect bite-like lesions. Conclusion. We think that eosinophilic panniculitis and insect bite-like lesions may be part of the spectrum of the same entity in patients with hematologic disorders.
Abstract: Angiolipomas are either encapsulated or non-encapsulated fatty tumours. They are distinguished from other lipomas by the excessive degree of vascular proliferation and because they are mixed with mature adipocytes. They commonly occur in the trunk and extremities and are rare in the maxillofacial area. Only 36 cases in the head and neck have been reported in the literature. The authors report the first non-infiltrating intramasseterine angiolipoma, and a case of non-infiltrating angiolipoma of the cheek. These tumours appear as homogenous low-density areas on CT with no contrast enhancement. MRI gives better tumour delineation and clear definition of the location and longitudinal extent of the mass. Histopathology demonstrates mature adipose tissue and the proliferation of numerous small branching blood vessels. Management of angiolipomas requires complete surgical excision.
Abstract: Various treatment options are available for use in moderate-to-severe psoriasis and election is dependent upon the clinical criteria applied by the attending physician. We undertook a survey among dermatologists to assess the treatment of moderate-to-severe psoriasis currently used in clinical practice in Spain.
Abstract: Actinic cheilitis (AC) is a precancerous lesion of the lip. Treatment of AC is indicated for prevention of squamous cell carcinoma (SCC), although the exact transition rate of AC to SCC is unknown. Carbon dioxide (CO(2)) laser vaporization seems to be an adequate therapy for AC, but there are no references about the evolution rate of AC to SCC after this treatment.
Abstract: This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20 000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25 % of cases, the patients' skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist.
Abstract: Diethylthiourea, like other thioureas, is often used by the rubber industry and in the manufacture of neoprene. We present a patient who suffered allergic contact dermatitis to diethylthiourea in a neoprene wader and who required admission to hospital and systemic treatment. We review the literature on allergy to diethylthiourea. Thioureas are not included in the standard GEIDAC (Spanish Contact Dermatitis Research Group) battery of patch tests. In these cases, it is necessary to use a special battery of rubber allergens, which includes thiourea compounds, for diagnosis of the disease and to ensure that cases of contact allergic dermatitis to thioureas do not go undiagnosed.
Abstract: Congenital melanocytic nevi are very common lesions that nevertheless pose many controversial questions. A systematic review of the literature suggests that the risk of developing melanoma on giant congenital melanocytic nevi (GCMN) is lower than previously thought given that, in the most recent series of GCMN, only 2 % of patients developed melanoma and most did so before the age of 5 years. Therefore,prophylactic surgery should be considered on an individual basis according to the degree of clinical suspicion of melanoma and the esthetic and functional consequences.In extensive reviews of series of biopsies of melanoma, small congenital melanocytic nevi have been associated with 7 % to 8 % of cases. Many authors believe that these might represent a significant risk of malignant conversion from 10 years onwards and so recommend regular control visits during infancy and prophylactic exeresis in puberty.
Abstract: INTRODUCTION: The term spitzoid melanoma (SM) is reserved for a rare group of tumors with striking resemblance to Spitz nevus, often developing in children diagnosed in retrospect after the development of metastases. OBJECTIVES: To determine the biological significance of SM and to analyze the effectiveness of adjuvant diagnostic techniques. MATERIALS AND METHODS: A retrospective, observational study of 38 cases of SM in patients younger than 18 years. Histological type, Clark level and Breslow thickness, radial and vertical growth phase, mitotic count/mm(2), ulceration, regression, vascular and perineural invasion, satellitosis, cytology and associated nevi were reviewed. An immunohistochemical analysis with HMB45 and Ki67 was performed in 10 cases. These features were correlated to patient's stage and outcome. RESULTS: Analysis of histological and immunohistochemical features should allow accurate diagnosis in most cases. Given the low mortality rate, no conclusions about the prognostic significance of histological parameters of the primary tumor could be established. CONCLUSION: We report the largest series of SM from a unique center. Although these patients may have a better prognosis than adults, some patients with SM develop metastasis and die, particularly after age 11 years. Therefore, we recommend using the same treatments as in adults.
Abstract: Treatment of eruptive vellus hair cysts (EVHC) is often unsatisfactory. Laser treatment has been described as an adequate treatment in a few reports. Pulsed carbon dioxide (CO(2)) laser has been used effectively for facial EVHC and erbium:yttrium-aluminium-garnet laser has been used to treat truncal EVHC with variable outcomes. We report our experience with CO(2) laser and lateral manual pressure to extract the cysts as an effective treatment in two cases of EVHC. Good cosmetic results were obtained in both cases. No lesion recurrence was observed after a follow-up of 10 years in the first case and 3 years in the second one. CO(2) laser vaporization and further extraction of cysts by manual lateral pressure might be an effective method to treat EVHC, achieving good cosmetic results and no recurrence.
Abstract: BACKGROUND: The treatment of venous malformations remains controversial. Traditional surgical excision is only possible in a few cases. Numerous sclerosing agents have been used, but none of them are ideal or absolutely safe for the treatment of venous disorders. In isolated cases, an expectative control would be a good option. OBJECTIVE: To report our experience with CO(2) laser vaporization as palliative treatment in five cases of head and neck venous malformations with lip involvement. METHODS: Five patients were treated with a CO(2) laser in superpulsed, focused mode at 2 W/cm(2), with several passes in each session. Patients were aged from 16 to 49 years old (mean: 36.6 years). Three sessions of treatment were performed in two patients while only one session was used in the rest. The follow-up was 6-36 months (mean: 22.8 months). RESULTS: Significant cosmetic improvement with flattening of the lip surface was achieved in all patients. CONCLUSION: Carbon dioxide laser vaporization can be considered as one method of choice for an effective palliative treatment of lip involvement in venous malformations.
Abstract: INTRODUCTION: In the last years, the incidence of syphilis has incremented in Spain and coinfection with HIV occurs in a high percentage. In HIV-infected patients with syphilis, neurological complications, treatment failure and relapse appear to be slightly raised. Therefore, careful follow-up must be carried out because of the risk of developing neurosyphilis. According to the guidelines, lumbar puncture (LP) is indicated in HIV-infected patients with late latent syphilis or syphilis of unknown duration, but it is discussed in HIV-infected patients with early active syphilis. Recent research has been developed in order to determine clinical and analytical findings for identification of patients with high neurosyphilis risk. We review different opinions about this topic and report our experience. METHODS: We have performed LP in all HIV-infected patients with early active syphilis during 2003-2006. RESULTS: Of the eight studied patients, none met criteria for neurosyphilis. Three of eight (38 %) had a peripheral blood CD4 cell count CD4 + <or= 350 cells/microL. Seven of eight (875 %) had RPR >or= 1:32. CONCLUSION: In these patients, performance of LP could be over indicated because of lack of well-established criterion. Our results are in agreement over to recent studies which restrict indication of LP to specific groups.
Abstract: Kikuchi's disease (KD) is a self-limiting histiocytic necrotizing lymphadenitis (HNL). Cutaneous manifestations are frequent and usually show histopathological findings similar to those observed in the involved lymph nodes. HNL with superposed histological features to KD has been described in patients with lupus erythematosus (LE), and a group of healthy patients previously reported as having HNL may evolve into LE after several months. Up to date, features to predict which HNL patients will have a self-limiting disease and which could develop LE have been not identified. In order to clarify the characteristics of skin lesions associated with KD, we report a case of HNL with evolution into systemic lupus erythematosus (SLE) and a review of previous reports of KD with cutaneous manifestations. A 17-year-old woman presented with a 4-month history of fever and generalised lymphadenopathy. A diagnosis of HNL was established based on a lymph node biopsy. One month later, she developed an erythematoedematous rash on her upper body, with histopathological findings of interface dermatitis. After 8 months, anti-nuclear antibodies (ANA) at titre of 1/320, anti-DNA-ds antibodies and marked decrease of complement levels were detected. During the following 2 years, she developed diagnostic criteria for SLE, with arthralgias, pleuritis, aseptic meningitis, haemolytic anaemia and lupus nephritis. To our knowledge, 27 cases of nodal and cutaneous KD have been reported, 9 of which later developed LE. In all these patients, the skin biopsy revealed interface dermatitis. Skin biopsy revealed a pattern of interface dermatitis in all reviewed KD cases, which evolved into LE. Even this histopathological finding was not previously considered significant; it might be a marker of evolution into LE.
Abstract: Background: Bowen's disease is a common form of intraepidermal (in situ) squamous cell carcinoma of the skin and mucous membrane. Treatment with destruction of the epidermis by any method is necessary to prevent invasive squamous cell carcinoma. Objective: To report our experience with CO(2) laser vaporization in the treatment of Bowen's disease and try to identify which factors could have any influence on results. Methods: A total of 44 patients were treated with the CO(2) laser in superpulsed mode, focalized at 2 W/cm(2). Results: In 86.3% of patients, a unique treatment session was required. 'Clearance after one treatment' was achieved in 86.3% of the total series of patients and only 7.9% of these patients developed recurrence of the lesion. In 11.3% of the cases, there was 'clearance after more than one treatment' without clinical recurrences in any of these patients during the follow-up. 'No response' was achieved in 2.2% of the total series of patients. Conclusion: We conclude that the CO(2) laser in superpulsed mode is an effective, efficient, safe, functional and good cosmetics treatment for Bowen's disease and it provides the same recurrence rate as other more aggressive or more expensive modalities of treatment.
Abstract: BACKGROUND: Ulceration is the most common complication of infantile haemangiomas and constitutes an authentic therapeutic challenge because of associated pain, infection, haemorrhage and subsequent scarring. OBJECTIVE: To report our experience with an intense pulsed light (IPL) system in the treatment of ulcerated haemangiomas. METHODS: Case 1: A 4-month-old girl, with haemangioma affecting the entire cutaneous surface of the left limb, developed four ulcerations on the inner aspect of this extremity. Two sessions with an IPL system using a triple pulse mode, a 570-nm lower cut-off filter and a fluence of 38 J/cm2 were performed. Case 2: A 5-month-old girl with ulcerated labial haemangioma that previously failed to respond to intralesional corticoids was treated with an IPL system device. Three sessions using a triple pulse mode with a 570-nm lower cut-off filter and a fluence of 48 J/cm2 were realized. RESULTS: Good results were rapidly obtained after two and four sessions of IPL treatment, respectively. Pain was soon relieved and complete epithelization was obtained by between 1 and 2 months in both patients. CONCLUSION: Although our experience is rare, we believe that IPL devices may be an effective alternative treatment of ulcerated haemangiomas.
Abstract: BACKGROUND: Bowen's disease is a common form of intraepidermal (in situ) squamous cell carcinoma of the skin and mucous membrane. Treatment with destruction of the epidermis by any method is necessary to prevent invasive squamous cell carcinoma. OBJECTIVE: To report our experience with CO2 laser vaporization in the treatment of Bowen's disease and try to identify which factors could have any influence on results. METHODS: A total of 44 patients were treated with the CO2 laser in superpulsed mode, focalized at 2 W/cm2. RESULTS: In 86.3% of patients, a unique treatment session was required. 'Clearance after one treatment' was achieved in 86.3% of the total series of patients and only 7.9% of these patients developed recurrence of the lesion. In 11.3% of the cases, there was 'clearance after more than one treatment' without clinical recurrences in any of these patients during the follow-up. 'No response' was achieved in 2.2% of the total series of patients. CONCLUSION: We conclude that the CO2 laser in superpulsed mode is an effective, efficient, safe, functional and good cosmetics treatment for Bowen's disease and it provides the same recurrence rate as other more aggressive or more expensive modalities of treatment.
Abstract: The use of laser applications in urology has undergone significant advances and is now used in a wide variety of procedures. Improvements in types of lasers, the wavelength of energy used, optical fiber delivery systems, precision of laser applications and cost reduction have served to further improve laser technology and extend the potential applications. The different types of lasers available at the present time appear to be an alternative treatment modality with excellent cosmetic and functional results and low morbidity in the treatment of benign, pre-malignant and malignant lesions in the genital area. The objective of this article is to provide an update on the most important clinical and experimental advancements therapeutic applications of lasers in genital lesions.
Abstract: BACKGROUND: Multiple endocrine neoplasia type 1 (MEN1) is a genetic disease that predisposes to endocrine tumour development. Some cutaneous lesions (angiofibromas, collagenomas, melanosis guttaca, lipomas, melanomas, 'cafe au lait macules') have been associated to this syndrome. We compare the prevalence of cutaneous lesion in affected patients with their non-carrier relatives. PATIENTS AND METHOD: We studied 9 patients with MEN1 and 20 non-carrier, first-degree relatives. Genetic screening was realized in all of them. Patients were examined by dermatologist, and biopsy was performed when necessary. RESULTS: Patients with MEN1 presented hyperparathyroidism (100%), neuroendocrine tumours of pancreas (66%) and pituitary adenomas (44%); their relatives were free of endocrine features of MEN1. The studied cutaneous lesions were more prevalent in affected patients than in non-carriers (55.5% vs. 25%; P = 0.029). Odds ratio of developing cutaneous lesions in MEN1 patients was 6.6 (95% confidence interval, 1.09-40.43). The frequency of angiofibromas was lower (22.2%) than the reported in other studies (43-88%), and we did not find any collagenoma. CONCLUSIONS: MEN1 is associated to some cutaneous lesions and could be useful for detecting MEN1 carriers in an affected family. Cutaneous lesions should be assessed in MEN1 patients.
Abstract: Cutaneous phaeohyphomycosis is a rare opportunistic fungal disease, knowledge of which is important because of the increase in organ transplantation, aggressive treatments for malignancies, and chronic use of corticosteroids. We report 3 cases of cutaneous phaeohyphomycosis: two patients treated with oral corticosteroids and one elderly woman with multiple hospitalizations. They showed several different clinical appearances. Histopathologically, the fungal infection affected the dermis and subcutis in all cases and in one of them, also the epidermis. Exophiala jeanselmei was isolated from the purulent exudate in the three cases. Good response was obtained with surgical and antifungal treatment.
Abstract: Selective immunoglobulin A deficiency (IgAD) is a primary immunodeficiency disease characterized by low levels (< 7 mg/dl) of serum immunoglobulin (Ig) A and normal serum levels of IgG and IgM. Patients with IgAD have increased risk for recurrent respiratory and gastrointestinal infections, autoimmune disease, asthma and allergy. A 26-year-old woman was admitted with sudden onset of painful cutaneous lesions on her lower extremities, pyrexia and arthromyalgia. Her medical history was remarkable for recurrent respiratory tract infections, self-limited episodes of acute diarrhea, atopy, splenomegaly and a 4-year history of a lung granulomatous lesion. Laboratory and imaging tests ruled out severe life-threatening infection, connective tissue disease and neoplasm. Serum protein electrophoresis showed a low IgA serum level (6.67 mg/dl), with normal serum levels of IgG and IgM, conducting to a diagnosis of selective IgAD. A skin biopsy showed necrotizing vasculitis without any sign of internal organ disease. We report a patient with IgAD and granulomatous involvement of lungs, spleen and medium-sized arteries of the skin. Although IgAD results from a failure of B-cell differentiation, we propose that deregulated immune response with production of cross-reactive antibodies and hyperstimulation of T cells and macrophages could contribute to this widespread granulomatous reaction.
Abstract: Imiquimod is a topically active imidazoquinoline immunomodulator agent. It works as an indirect antiviral and antitumoral and stimulates the production of INF-alpha and various other cytokines. We assayed topical imiquimod in treating early stages of mycosis fungoides. We applied imiquimod 5% cream in four patients with multi-treatment resistant plaques of MF (stages IA and IIB). We applied it on one patient in association with systemic INFalpha-2a. We observed a complete clinical clearance of the lesions in all four patients. In three cases we achieved a complete histopathological clearance and in one case a partial histopathological clearance. The patient treated with imiquimod and systemic INFalpha-2a showed the most spectacular improvement with a rapid total response. We ascribe this improvement to a synergic effect of imiquimod and systemic INFalpha-2a treatment. Before the introduction of imiquimod, this patient had been treated for 2 years with systemic INFalpha-2a alone, without any evidence of clinical response. Imiquimod could be an effective therapy for early-stage disease of CTCL, used alone or in combination with systemic immunomodulatory therapy.
Abstract: Papular elastorrhexis is a rare entity of elastic tissue characterized by multiple white papules usually located on the trunk. We report a case of papular elastorrhexis in a 22-year-old man with sparse lesions and discuss the main controversial facts in this entity: its consideration as an independent disease or as an incomplete form of Buschke-Ollendorff syndrome and the confusion with and its possible inclusion as a variant of elastic tissue naevi.
Abstract: Primary cutaneous mucinous carcinoma is a rare adnexal sweat gland neoplasm that mainly affects elderly people. Differential diagnosis includes mammary and gastrointestinal metastatic mucinous carcinoma (MC) and secondary cutaneous involvement by underlying neoplasms. An 83-year-old woman presented with an 8-year history of slow-growing infiltrate plaque in her right hemithorax, with ulceration on supraclavicular area, right upper limb edema and palpable axillary lymphadenopathies. She underwent partial excision of the tumor and local radiotherapy. Imaging studies showed widespread cutaneous dissemination with enlargement of ipsilateral axillary lymph nodes but without evidence of underlying breast cancer. Histopathological examination showed large amounts of mucin in the dermis including small islands of epithelial cells. They stained positive for cytokeratin 7, carcinoembryonic antigen, epithelial membrane antigen, gross cystic disease fluid protein-15, and c-erbB-2. Lymphatic invasion was demonstrated by D2-40-immunostained sections. A diagnosis of primary cutaneous mucinous carcinoma was made. Our aim was to reevaluate the differential clinical, histopathological, and immunohistochemical criteria for distinguishing primary cutaneous mucinous carcinoma from skin metastases of visceral mucinous carcinoma, especially those arising in breast. We also propose D2-40 as a reliable marker to detect lymphatic invasion that indicates a strong aggressive trend with shorter recurrence-free and predicts nodal metastases.
Abstract: BACKGROUND: Biologic therapies such as adalimumab, a tumour necrosis factor antagonist, are safe and effective in the treatment of moderate to severe chronic plaque psoriasis. OBJECTIVES: To compare a biologic agent with methotrexate, a traditional systemic agent, to define clearly the role of biologics in psoriasis. METHODS: Patients with moderate to severe plaque psoriasis were randomized to adalimumab (80 mg subcutaneously at week 0, then 40 mg every other week, n=108), methotrexate (7.5 mg orally, increased as needed and as tolerated to 25 mg weekly; n=110) or placebo (n=53) for 16 weeks. The primary efficacy endpoint was the proportion of patients achieving at least a 75% improvement in the Psoriasis Area and Severity Index (PASI 75) after 16 weeks. Safety was assessed at all visits through week 16. RESULTS: After 16 weeks, 79.6% of adalimumab-treated patients achieved PASI 75, compared with 35.5% for methotrexate (P<0.001 vs. adalimumab) and 18.9% for placebo (P<0.001 vs. adalimumab). Statistically significantly more adalimumab-treated patients (16.7%) than methotrexate-treated patients (7.3%) or placebo-treated patients (1.9%) achieved complete clearance of disease. The response to adalimumab was rapid, with a 57% improvement in mean PASI observed at week 4. Adverse events were similar across treatment groups. Adverse events leading to study discontinuation were greatest in the methotrexate group, primarily because of hepatic-related adverse events. CONCLUSIONS: After 16 weeks, adalimumab demonstrated significantly superior efficacy and more rapid improvements in psoriasis compared with either methotrexate or placebo.
Abstract: Mastocytosis is a group of disorders characterized by the accumulation of mast cells in different tissues and organs. The skin is the most frequently involved organ (90% of cases) where mastocytosis may show a heterogenic clinical expression. Anetodermic lesions are an unusual clinical presentation of mastocytosis. We report a case of anetodermic mastocytosis in a 26-year-old man, with sparse lesions and a benign course. PUVA therapy obtained excellent results in this case. Darier's sign should be investigated in patients with anetodermic lesions of unclear origin in order to exclude cutaneous mastocytosis.
Abstract: Sweet syndrome is one of the cutaneous processes more frequently associated to systemic diseases. Its association to the systemic inflammatory response syndrome has rarely been described. We report a case of chronic and relapsing Sweet syndrome associated to a chronic and idiopathic systemic inflammatory response syndrome that lasted seven years and proved fatal to the patient. Among the rare cases of Sweet syndrome associated to a systemic inflammatory response syndrome that have been described there have not been any fatal cases as occurred with our patient.
Abstract: Pustular dermatosis of the dorsal hands is a peculiar variant of Sweet's syndrome described by Strutton et al. Nevertheless, not all authors accept this entity. We present a retrospective study of eight cases of neutrophilic vasculitis of the hands. Three patients presented with lesions only on the dorsal hands, three with lesions on the palmar surface and two with lesions on the dorsal and palmar surfaces of the hands. The patients with lesions on the dorsal surface presented a pustular aspect, and the histopathological examination revealed a high degree of vascular damage with presence of vasculitis and, in one case, with fibrinoid necrosis. Therefore, the term pustular vasculitis of the dorsal hands introduced by Strutton et al. should probably be conserved. Our cases may have demonstrated a peculiar clinicopathological disease with its own entity.
Abstract: OBJECTIVE: To report 3 cases of skin rash with a peculiar livedoid pattern that were probably associated with imatinib therapy. CASE SUMMARY: In the first case, a 74-year-old male diagnosed with Philadelphia chromosome-positive (Ph+) chronic myeloid leukemia (CML), treated with imatinib 400 mg/day, developed a skin eruption with a livedoid pattern. Systemic corticosteroids were started, and skin lesions improved. The second case involved a 66-year-old male with Ph+ CML who was treated with imatinib 600 mg/day. After initiation of this treatment, he developed a skin rash with a livedoid pattern. The drug treatment was discontinued and then reintroduced. Topical corticosteroid treatment was started, resulting in total remission of the skin lesions. When the imatinib dose was progressively reintroduced, the skin lesions recurred. The patient died as a result of the progression of his disease. In the third case, a 43-year-old male with Ph+ acute lymphoblastic leukemia was treated with imatinib 600 mg/day. After a few days of treatment, the patient developed a skin rash with a livedoid pattern. He died as a result of probable septic shock. DISCUSSION: Imatinib is a tyrosine kinase receptor inhibitor that inhibits BCR/ABL tyrosine kinase. There have been several published articles on cutaneous adverse reactions related to imatinib therapy. The most common cutaneous adverse event of imatinib is a rash with variable clinical presentation. The Naranjo probability scale indicated a probable relationship between imatinib and the rash in all 3 cases reported here. CONCLUSIONS: Adverse reactions to imatinib that affect the skin occur frequently. They are strongly dose dependent, self-limiting, or easily managed by lowering the dose of imatinib and, if necessary, prescribing short-term therapy with a systemic corticosteroid. Clinicians should monitor patients taking imatinib and institute treatment quickly if a rash develops.
Abstract: Acroangiodermatitis or pseudo-Kaposi sarcoma is an angioproliferative, self-limited entity that includes a group of diseases, congenital or acquired, with cutaneous lesions similar to Kaposi sarcoma (KS). This term can lead to confusion because it comprises several entities that are completely different, nonetheless, it has an important clinical value as it guides the diagnosis and management of these patients. We report the case of a 67-year-old patient with lesions of acroangiodermatitis in both forearms secondary to arteriovenous shunts from hemodialysis. Doppler ultrasound showed a former arteriovenous fistula in addition to the one already known. Immunohistochemical study showed CD34+ staining in endotelial cells and absence of HHV-8 expression.
Abstract: BACKGROUND: Epidermal nevi are benign proliferations of epidermis. Numerous treatment modalities have been tried, but no ideal treatment is yet available. OBJECTIVE: To report our experience with CO(2) laser vaporization in the treatment of verrucous epidermal nevi, and to identify which factors could have influence in long-term results. METHODS: A total of 25 patients were treated with the CO(2) laser in the superpulsed mode, focalized at 2 W/cm(2). The patients were between 3 and 41 years old (mean: 17 years). A total of 44% of lesions were <20 cm(2), 40% measured between 20 and 100 cm(2) and 16% were >100 cm(2). The follow-up was 4-79 months (mean: 28 months). In 76% of patients, multiple treatment sessions were required. RESULTS: Good results were achieved in 92% of patients with soft, flattened nevi and in only 33% patients with keratotic nevi. In addition, 87% of the latter had moderate results and 12.5% had poor results. CONCLUSION: We conclude that the CO(2) laser in superpulsed mode is an effective and safe treatment for verrucous epidermal nevi and provides fewer recurrences than other laser therapies. We also believe that the most determining factor for the cosmetic result is thickness of the nevus.
Abstract: The term "Acute Syndrome of Apoptotic Pan-Epidermolysis" (ASAP) designs clinical entities characterized by massive cleavage of the epidermis resulting from hyperacute epidermal basal cell apoptotic injury. It can be seen typically in classic toxic epidermal necrolysis (TEN), but occasionally occurs in non-drug-induced entities called "TEN-like" diseases (e.g., lupus erythematosus (LE), acute graft versus host disease and pseudoporphyria).We would like to highlight the difficulties of establishing differential diagnoses between "TEN-like" LE and drug reactions, especially when LE has not been previously diagnosed. We report a patient with fulminate pattern of epidermal cell injury resulting in a clinical presentation having combined features of drug-induced TEN and acute cutaneous LE with laboratory findings of systemic LE (SLE) and without systemic symptoms or high-risk drug ingestion. Although most cases of ASAP in the setting of LE are drug-induced TEN, there are reported cases of "TEN-like" LE with subacute progression, absence of systemic involvement and lack of drug ingestion. Such cases usually have a previous history of SLE and positive serologic markers. Although some authors observed that these lesions could be related to systemic severity of SLE, this is the first patient reported who progresses to discoid LE and we think it could be a marker of good prognosis.
Abstract: Histiocytoses are a heterogeneous group of diseases, characterized by the accumulation of reactive or neoplastic histiocytes in various tissues. Generalized eruptive histiocytosis belongs to cutaneous non-Langerhans' cell histiocytoses and is a rare, generalized, self-healing disorder that usually follows a benign clinical course. Herein, we report a case of generalized eruptive histiocytosis in a 41-year-old woman with peculiar clinical and histological features. Clinically, the papules showed a marked distribution into the seborrhoeic areas of the trunk, with a great tendency to coalesce. Furthermore, immunohistochemical labelling demonstrated that the histiocytes were positive for CD68, but negative for CD34, S100, CD1a and XIIIa factor. This is the second report of generalized eruptive histiocytosis with a negative XIIIa factor. We discuss the differential diagnoses of the clinical picture and emphasize that this benign cutaneous disorder should be subjected to close follow-up, owing to the possibility of evolution to a more severe type of histiocytosis or the association with underlying diseases. Spontaneous regression was observed in this actual case.
Abstract: OBJECTIVE: To study 26 cases of lupus erythematosus tumidus (LET), a subset of chronic cutaneous lupus erythematosus (CCLE), referred to in the literature as a rare entity. PATIENTS AND METHODS: A retrospective study was conducted of 26 patients diagnosed with LET between 1996 and 2002. The clinical characteristics, histopathologic and laboratory findings, response to treatment, association with other subsets of lupus, course, and diagnostic criteria were analyzed. RESULTS: The incidence by sex was similar. The mean age of presentation was 49.19 years. The clinical presentation usually involved erythematous, edematous plaques located on the face, chest, back, or extremities, related to sun exposure. A dermal lymphocytic infiltrate with a perivascular disposition and differing degrees of mucin deposition was observed in all cases. Minimal epidermal changes were present in 18 cases, and 11 of these also showed minimal dermal-epidermal changes. Only one case showed dermal-epidermal changes without any epidermal alteration. Direct immunofluorescence test was performed in 15 patients, and 11 were negative. All cases showed a benign course without systemic manifestations. The response to topical steroids or antimalarial treatment was excellent, but a seasonal recurrence was usually observed. Discussion No defined criteria for LET are universally accepted. The main controversies are the acceptance of LET as a separate subset of CCLE, and the histopathologic diagnostic features, mainly the presence or absence of epidermal and dermal-epidermal changes in these lesions. CONCLUSIONS: No inflexible histologic criteria should be employed for the diagnosis of LET. This subset of lupus erythematosus is characterized by intense photosensitivity, definite clinical lesions, a benign course, the absence of systemic disease, good response to antimalarial treatment, and a tendency to recur. More studies should be performed in order to establish the true incidence of LET because this subset of CCLE is probably underestimated.
Abstract: INTRODUCTION: The constant increase in the incidence of skin cancer together with the requirement for maximum exploitation of available medical resources has meant that dermatological major surgery on an outpatient basis has greatly increased in recent years. OBJECTIVE: This article reviews the practice of dermatological surgery in an outpatient setting over a 1-year period. Its purpose is to analyse the number of surgical procedures, the type of cutaneous processes treated and the kind of surgical intervention and anaesthesia used. We also assessed the percentage of cancellations, postsurgical hospital admission and postsurgical complications. Clinicopathological correlation and complete tumour removal were also evaluated. MATERIAL AND METHODS: Our clinical experience of major surgery of outpatients at the dermatology department of the Hospital Juan Canalejo in A Coruña (Spain), analysing 565 patients in a non-randomized pilot study running from January to December 2003, is presented. RESULTS: Six hundred and forty-four surgical procedures were performed on a total of 565 patients. The three main cutaneous processes treated were basal cell carcinoma (240), squamous cell carcinoma (117) and melanoma (77). The most frequent type of procedure was direct closure (346), followed by exeresis and flap (133) and partial- or full-thickness skin graft (29), nail apparatus surgery (56) and lip surgery (33). Twelve patients were admitted to hospital after surgery owing to the complexity of their operations or to complications arising during surgery. Five hundred and fifty-three patients were discharged after being kept under observation for a few hours. Seventeen patients (3%) suffered complications following surgery, which consisted of partial implant failure (six cases), infection of the surgical wound (six cases), intense pain (four cases) and haemorrhage (one case). There was a good clinicopathological correlation in 90.78% of non-melanoma skin cancers, of which 92.03% were completely removed. CONCLUSIONS: Medium and high complexity operations for dermatological processes, traditionally performed on hospitalized patients, can be conducted on an outpatient basis. This allows hospitalization costs and waiting lists to be reduced and affords the possibility of achieving better morbidity rates and medical care than in the standard hospital setting.
Abstract: Pancreatic panniculitis is an uncommon complication of pancreatic disease, which is characterized by liquefactive necrosis of the subcutaneous fat and may constitute the first evidence of a pancreatic illness. Herein, we report a case where cutaneous lesions of pancreatic disease were recognized prior to detection of an acute episode of a known chronic pancreatitis.
Abstract: Pegylated liposomal doxorubicin (PLD) is usually used in disseminated HIV-related Kaposi's sarcoma (KS). It is the first-line treatment or second-line therapy in patients who do not tolerate or do not respond to polychemotherapy since 1995, when it was approved by the FDA. We report two cases of disseminated classic KS not associated with immunosuppression in which HHV-8 infection was demonstrated. They showed an excellent response to PLD, with good tolerance and an absence of side effects.
Abstract: INTRODUCTION: Several diseases with follicular occlusion have been described in HIV-infected patients and can appear isolated or associated with each other in an independent disease named 'HIV-associated follicular syndrome'. Worsening of cutaneous lesions during the course of infection in patients with previous acne vulgaris, late onset or severe forms of acne have been reported in HIV-infected patients. PATIENTS AND METHODS: A prospective study of 335 HIV-infected patients was performed in our hospital. A meticulous dermatological exploration in all patients was made, and multiple data were stored into a Microsoft Access 97 program. Patients diagnosed as having acne were considered separately, and we studied their characteristics. A statistical analysis with SPSS 9.0 (Statistical Package for the Social Sciences) was performed. RESULTS: Thirty-six patients (10.8%) were diagnosed as having acne. Papulopustular lesions were the most frequent clinical presentation. Most lesions were localized on the face or on the back, and 80% of patients had mild to moderate acne; 40% of them required no treatment and the remainder was treated with topical measures, oral antibiotics and isotretinoin. 85% of cases reported the onset of acne during adolescence or before HIV infection and without any relationship with this disease. No relation between acne intensity and stage of HIV infection was observed. A multivariate analysis showed that the most important variable was age. DISCUSSION: In contrast to previous reports, no greater intensity or modifications in acne lesions were observed in our HIV-infected patients during the course of the disease. In the majority of cases, cutaneous lesions started before HIV infection, during adolescence, and the most important factor for suffering from acne was young age. In contrast to data reported in the literature, no relation of acne lesions to CD4 lymphocyte count or AIDS case criteria was found in our patients. CONCLUSIONS: Acne in HIV-infected patients has characteristics similar to those in non-HIV-infected patients. The age is the most important factor for the appearance of lesions that usually develop during adolescence. Acne lesions are not modified by HIV infection, and no relationship with the severity of HIV infection has been observed.
Abstract: A 74-year-old woman with chronic auricular fibrillation, arterial hypertension, hypercholesterolemia, ischemic cardiopathy, and peripheral arteriopathy presented with purpuric lesions on the lower limbs (Fig. 1) and, to a lesser extent, on the anterior area of the chest. The mucous membranes were not affected. In 1989, she was diagnosed with anemia that evolved until 1998, when a bone marrow biopsy revealed a myelodysplastic syndrome unclassified in French-American-British Group (FAB). The patient has required periodic transfusions since February 1999. A skin biopsy of the purpuric lesions revealed a leukocytoclastic vasculitis; the lesions cleared with topical corticosteroid treatment. In May 1999, the patient presented with inflammatory and painful lesions localized on the vulva (Fig. 2), which had evolved over several days, without fever. No lesions were observed in other locations. A cutaneous biopsy showed an intense dermal edema and a diffuse and polymorphous dermal infiltrate involving the follicular structures. Exocytosis, spongiosis, and mucin deposits, demonstrated by Alcian blue stain, were observed in the follicular epithelium. Mature neutrophils were predominant in the dermal infiltrate, but a small number of eosinophils and immature cells were also present (Fig. 3). The myelogenous origin of the immature lining cells was further confirmed by positive staining of intracytoplasmic granules with naphthol-ASD chloroacetate sterase (Leder's stain). Vasculitis was not observed. Routine laboratory tests revealed 3030 leukocytes/mm(3) (60% neutrophils), a hemoglobin level of 8.4 g/dL, and 92,000 platelets/mm(3). Treatment with 30 mg/day of prednisone was started, and the lesions cleared slowly within 4 weeks. A new bone marrow biopsy in September 1999 showed a similar appearance to that taken in 1998. The patient died in January 2000 as a result of pneumonia with cardiac and respiratory failure. A 66-year-old man presented with a febrile syndrome that had evolved over 5 days, and painful and pruritic cutaneous lesions on the face and posterior neck (Fig. 4). Three months before, the patient was diagnosed with chronic myelogenous leukemia in acceleration phase. Examination revealed an edematous and erythematous face with pustular lesions on the surface, also involving the neck and the upper part of the back. The histopathologic examination revealed an intense edema and abscesses in the dermis. The infiltrate of these lesions was composed of mature neutrophils with the presence of abundant immature cells with a myelogenous aspect (Fig. 5). Analytical studies revealed 26,130 leukocytes/mm(3) (42% blasts). No specific treatment for Sweet's syndrome was administered and the lesions showed an improvement within 5 days. Eight days after admission, the patient died as a result of acute hemorrhage, before treatment for leukemia was initiated.
Abstract: BACKGROUND: Erythroplasia of Queyrat is an uncommon carcinoma in situ that usually arises on the mucosa of the glans penis or prepuce as red, shiny, sharply demarcated velvety plaques. The treatment is often difficult and associated with significant recurrence rates, especially in cases with urethral involvement. OBJECTIVE: To evaluate carbon dioxide laser treatment in a case of erythroplasia of Queyrat with urethral involvement. MATERIALS AND METHODS: We report a patient with erythroplasia of Queyrat involving the urethra who was treated with carbon dioxide laser vaporization. RESULTS: Treatment with carbon dioxide laser vaporization resulted in an excellent cosmetic and functional status outcome for the glans penis and urethral lesions. CONCLUSION: Carbon dioxide laser vaporization is probably the initial elective treatment for erythroplasia of Queyrat with urethral involvement in young immunocompetent patients. A follow-up is necessary, and new sessions of treatment should be quickly performed if recurrence occurs.
Abstract: Necrobiotic xanthogranuloma (NXG) with paraproteinemia is a rare non-x histiocytosis with conspicuous lesions mainly located on the periorbital skin. A 68-year-old woman, with a previous history of lymphoplasmacytic lymphoma associated with IgG monoclonal gammopathy over a period of almost 4 years, presented typical lesions of NXG on the periorbital regions and left buttock. Treatment with dioxide carbon laser resulted in great improvement of cutaneous lesions, and no evidence of relapse after a 12-month follow-up. The association of NXG with lymphoplasmacytic lymphoma has not been previously described to our knowledge. The treatment of these lesions represents a true challenge for the clinician and palliative treatment of cutaneous lesions of NXG with CO(2) laser may constitute an alternative treatment in selected cases.
Abstract: BACKGROUND: Angiokeratoma circumscriptum naeviforme (ACN) is an unusual type of localized angiokeratoma that occurs more frequently in females and is usually located on the buttocks or thighs, showing a unilateral distribution. ACN usually causes large lesions, which may require laser ablation because they often are too extensive to perform surgical excision. Carbon-dioxide laser is a known alternative for treating angiokeratomas. OBJECTIVE: Report of two cases of ACN treated with carbon-dioxide laser vaporization. PATIENTS AND METHODS: A 28-year-old woman with a hyperkeratotic, violaceus plaque on her left buttock and a 24-year-old woman with a similar lesion on her right buttock were treated with a carbon-dioxide laser. Two laser passes were performed on each lesion within a single session. RESULTS: A successful cosmetic aspect of treated areas was obtained, with minimal pigmentary or textural changes. After a 2-year follow-up in the first patient and a 6-month follow-up in the second patient, no recurrence of the lesions was observed. CONCLUSIONS: Our results, obtaining relevant cosmetic improvement after a few sessions of treatment, with low morbidity and minimal secondary effects, suggest that continuous-wave carbon-dioxide laser vaporization is a safe and effective treatment for ACN.
Abstract: The decreased incidence of infectious diseases in developed countries may make their diagnosis difficult. Cutaneous tuberculosis is an example of this fact. A 44-year-old man presented with two painful abscesses on his lower extremities, which developed into chronic ulcers. A cutaneous biopsy revealed necrotizing granulomas in the dermis. Ziehl-Neelsen and periodic acid-Schiff stain were negative. Mantoux test was positive. Tc-99m scintigraphy showed increased uptake in the bone tissue of the left ankle and right tibiae, without direct relation to cutaneous lesions. Chest X-ray showed micronodular, apical, bilateral infiltrates, reduced volume of the right lung, and cavitation of the right superior lobe. Mycobacterium tuberculosis was grown from sputum and skin biopsy samples. Isoniazid, rifampin and pyrazinamide treatment for 2 months, followed by isoniazid and rifampin for 12 months, resulted in complete resolution. The clinical features of cutaneous tuberculosis in our patient were characteristic of tuberculous abscesses. Some uncommon findings, such as the low number of lesions, negative acid-fast resistant stains in cutaneous biopsy samples and his preserved general state of health, may be explained by a higher competence of the immune system than is usual in this clinical subset of disseminated tuberculosis. Cutaneous tuberculosis should be included in the differential diagnosis of cutaneous abscesses in immunocompetent patients.
Abstract: BACKGROUND: Port-wine stains (PWSs) are capillary malformations that usually show progressive stasis of the vascular channels and cause slow hyperplasia of the soft and hard tissues. When these lesions involve the lower lip, macrocheilia may be developed along the time. Vascular-specific lasers are not adequate to correct these three-dimensional tissue deformities, and surgical management becomes necessary, resulting in considerable morbidity and aesthetic disturbances. OBJECTIVE: To report a case of macrocheilia secondary to PWS treated by combination of surgery and carbon dioxide laser. METHODS: A 51-year-old man with macrocheilia of the lower lip and severe functional impairment, secondary to long evolution PWS, received treatment with carbon dioxide laser vaporization and minimal surgical correction, resulting in significant improvement of the lower lip hypertrophy, good aesthetic and functional status, and preservation of the muscular function. CONCLUSIONS: Combined carbon dioxide laser and surgery treatment may constitute a valuable alternative in treatment of macrocheilia secondary to PWS because bleeding risk is minimized and improves the preservation of muscular function and aesthetic results in relationship to conventional surgical approaches.
Abstract: The pathogenetic mechanism of fixed drug eruption (FDE) is still unknown. One of the most common causes of FDE is the use of nonsteroidal antiinflammatory drugs (NSAIDs). Oxicams are in the NSAID group and piroxicam is one of the most used of these drugs. FDE caused by piroxicam is rare but a few cases have been reported. Patch tests are useful for diagnosing some cases of FDE; they give variable results on previously affected skin while no reaction appears on unaffected skin. Some cases of cross-sensitivity among piroxicam and other substances have been reported. We report two new cases of FDE due to piroxicam with negative patch test on normal skin and positive results on affected skin.
Abstract: OBJECTIVE: To report a case of psoriasiform eruption induced by infliximab. CASE SUMMARY: A 46-year-old woman with enterocutaneous fistula secondary to Crohn's disease developed pruriginous, erythematous, desquamative plaques on her elbows, knees, hands, and buttocks after receiving the second and third doses of intravenous infliximab. Histologic examination showed a lichenoid pattern. No new cutaneous lesions appeared after cessation of infliximab therapy. DISCUSSION: Several cutaneous reactions secondary to infliximab, a monoclonal antibody against tumor necrosis factor-alfa, have been described. Psoriasiform dermatitis has not been reported as a cutaneous reaction to infliximab, but there have been several previous reports of psoriasiform dermatitis secondary to other drugs. An objective causality assessment revealed that the adverse event was probable. CONCLUSIONS: This is the first report of a clinico-pathologic dissociated pattern of cutaneous reaction showing a histopathologic picture of lichenoid dermatitis resulting from infliximab treatment.
Abstract: Apert's syndrome is an uncommon disease characterized by synostosis of extremities, vertebrae and skull. A clear association between Apert's syndrome and acne vulgaris with resistance to usual acne treatments has been described. A case of Apert's syndrome treated with oral isotretinoin with good results is reported and the pathogenic mechanisms of acne in this syndrome are discussed.
Abstract: Chromomycosis is a rare mycotic infection that is more frequent in tropical and subtropical regions. Dematiaceous fungi are the causal agents of this mycosis. Several cases of chromomycosis in organ transplant recipients have been reported. We present a case of chromomycosis by Exophiala jeanselmei in a Spanish male who had received a renal transplant several months previously, and was receiving treatment with tacrolimus, prednisone and mycophenolate mofetil. Very few cases of chromomycosis due to Exophiala have been reported, and this is, to our knowledge, the first European case.
Abstract: Acquired trichomegaly of the eyelashes in HIV-infected patients usually appears at the late stage of HIV infection. Eyelash length was measured in a series of 204 HIV patients, and no correlation with CD4 cell count, viral load, Centers for Disease Control and Prevention category, and AIDS case criteria was established. Our data support the finding that eyelash trichomegaly is currently uncommon in HIV-infected patients, perhaps because of antiretroviral therapy or an improvement in their immune situation.
Abstract: Venous lakes (VL) are acquired venous ectasies of the superficial dermal venules, usually observed in older people. Thirty-two adult patients with VL in several localizations were treated by carbon dioxide laser vaporization. Two passes were performed to each lesion within the same session. A continuous and defocused mode, with a power density of 5 W/cm2, was used in the first pass, and a continuous focused mode with the same power density was in the second pass. Photographic controls were performed before and after treatment. VLs were removed, and the smooth surface of the treated area was re-established by a single laser session. Only one case recurred after the treatment. No significant secondary effects were observed. Carbon dioxide laser is a good and safe method for treatment of skin VL. With adequate cautions, excellent cosmetic results can be obtained with a single session of treatment.
Abstract: Neutrophilic pustulosis is currently considered as a part of the spectrum of Sweet's syndrome, and has been associated with inflammatory bowel disease and several other diseases. We report the case of a 34-year-old male who had been suffering from ulcerative colitis (UC) for several years and who experienced the manifestation of a pustular eruption on both forearms and the abdominal wall during an exacerbation of his bowel disease. Both processes were controlled with steroids per os. The histological picture showed an inflammatory infiltrate composed mainly of neutrophils with scattered neutrophilic epidermal abscesses. Certain distinct clinical and histological characteristics have been described as diagnostic of the pustular eruption of UC. This picture should be included in the spectrum of the neutrophilic dermatosis, in our case associated with an ulcerative colitis. This is a well documented clinical and histologic case report of a recognized association of inflammatory pustulosis in the course of ulcerative colitis.
Abstract: Since 1998, many cases of antiretroviral therapy-related paronychia of the toes or fingers and ingrown toenails have been reported. Most of them were related to indinavir. Other indinavir-induced mucocutaneous disorders resembling the adverse effects of systemic retinoid therapy have also been reported. Although there is some uncertainty in the literature regarding a cause-effect relationship, results of several epidemiological and in vitro studies, together with cumulated clinical experience leave no doubt that indinavir causes a retinoid-like effect and nail alterations. Indeed, indinavir is the only antiretroviral drug that produces these disorders, although ritonavir may enhance indinavir-induced retinoid-like effects through pharmacokinetic interactions leading to increased plasma indinavir concentrations. Approximately 30% of patients receiving indinavir show two or more retinoid-like manifestations and 4-9% develop paronychia. These adverse effects are not related to other epidemiological variables such as the patient's sex, age or other risk factors or immune status. They seem to be exposure dependent and, therefore, largely dose-dependent. Chronic paronychia is considered generally to be caused by contact irritants and candidal infection. Nevertheless, indinavir is currently the most frequent cause of chronic or recurrent paronychia in HIV-infected patients. In addition, retinoid-like manifestations such as cutaneous xerosis and cheilitis are frequent mucocutaneous adverse effects related to indinavir. The exact mechanism of indinavir-induced retinoid-like effects is unclear. Hypotheses for pathogenesis include interference with retinoid metabolism by enhancing the retinoic acid signalling pathway, or by increasing retinoic acid synthesis, or by reducing cytochrome p450-mediated retinoic acid oxidative metabolism. Replacement of therapy by an antiretroviral regimen not containing indinavir, while retaining other protease inhibitors and lamivudine, resolves retinoid-like manifestations without recurrences.
Abstract: Methotrexate (MTX) inhibits DNA synthesis by competition with dihydrofolate reductase. Adverse cutaneous reactions to MTX are usually dose-related and have been mainly reported in patients receiving extremely large doses of chemotherapy. Painful erosion of psoriatic plaques has been often reported as an early sign of MTX toxicity, but cutaneous ulceration as a sign of MTX toxicity in patients without psoriasis has only been described in one case. We report a patient with rheumatoid arthritis and without psoriasis who developed cutaneous ulceration on the knuckles as a sign of MTX toxicity. Cutaneous ulceration by MTX toxicity is an exclusion diagnosis and its pathogenic mechanism may be multifactorial, including direct toxicity of the drug in addition to local factors.
Abstract: OBJECTIVE: To report a case of linear immunoglobulin (Ig) A bullous dermatosis (LABD) induced by gemcitabine. CASE SUMMARY: A 59-year-old man was diagnosed with squamous-cell carcinoma of the lung in T4N2M0 stage and treated with cisplatin, vinorelbine, and gemcitabine. Twenty-four hours after the administration of gemcitabine, a symmetric, bullous, herpetiform eruption appeared on his trunk and upper limbs. Histopathologic examination and direct immunofluorescence test were consistent with IgA bullous dermatosis. Cutaneous lesions resolved two weeks after the drug was withdrawn and topical steroid treatment was instituted. DISCUSSION: Drug-induced LABD is a variant of classic or idiopathic LABD. Vancomycin is the most frequently implicated drug, but other agents have been reported to cause LABD. According to the Naranjo probability scale, the relationship of gemcitabine treatment with cutaneous eruption in our patient is possible. CONCLUSIONS: We report the first case of gemcitabine-induced LABD. Clinicians should monitor patients receiving this drug for signs of LABD.
Abstract: BACKGROUND: Superficial white onychomycosis is exceptional in children, although a few anecdotal cases have been reported in the literature. On the other hand, superficial white onychomycosis and proximal subungual onychomycosis, while unusual in the general population, often appear in adult human immunodeficiency virus (HlV)-infected persons. CASE REPORT: A 3-year-old girl with vertically acquired HIV-1 infection presented superficial white onychomycosis involving the second, third, fourth and fifth toes of her left foot, caused by Trichophyton rubrum. She was treated with topical amorolfine once a week, with a good response within 4 weeks. CONCLUSIONS: We report a case of superficial white onychomycosis associated with HIV infection in a 3-year-old girl. To our knowledge, this type of onychomycosis has not been previously reported in HIV-infected prepubertal children.
Abstract: BACKGROUND: Port-wine stain (PWS) is a congenital vascular malformation of the superficial dermal vessels. These vessels become progressively ectatic, with development of nodular and hypertrophic areas on the surface of the lesions. OBJECTIVE: To determine the efficacy of CO2 laser vaporization in the treatment of nodules in PWS. METHODS: Twenty adult patients with PWS who developed nodules and hypertrophy on the surface of the lesions were treated by CO2 laser vaporization. Only one pass was performed to each area of the lesions, using a continuous and defocused mode, with a power density of 10 W/cm2. When the treated lesion was very large, several sessions of treatment were necessary to vaporize its entire surface. Photographic controls were performed before and after treatment. RESULTS: The nodules and hypertrophy were removed and the smooth surface of the lesions was reestablished. The color, usually violaceous, was transformed in a slight erythema. CONCLUSION: CO2 laser is a good method for treatment of nodules and hypertrophy in PWS. With adequate precautions, excellent cosmetic results can be obtained. Other more-specific laser systems may be successively used to treat the remaining erythema.
Abstract: BACKGROUND: Apocrine hidrocystoma is a benign cystic tumour with apocrine differentiation. Although most cases are solitary tumours, multiple tumours may occur. Surgical removal is the usual treatment for apocrine hidrocystoma, but it may be troublesome and disfiguring in cases with multiple tumours. OBJECTIVE: To determine the efficacy and cosmetic outcome of carbon dioxide laser vaporization in the treatment of multiple apocrine hidrocystomas. PATIENTS AND METHODS: A total of 11 lesions in three adult patients were treated with carbon dioxide laser vaporization using a continuous and defocused mode, with a power density of 5 J/cm(2). The lesions were localized lateral to the outer canthus, on the free edge of the eyelids, and on the ear. Only a single session of treatment was performed for each lesion. Photographic controls were taken before and after treatment. RESULTS: The lesions cleared after laser treatment without residual changes, and a successful cosmetic result was obtained. CONCLUSION: Carbon dioxide laser is an efficient method of the treatment for multiple apocrine hidrocystomas.
Abstract: Olmsted syndrome is an uncommon inherited disorder of keratinization that presents mutilating palmoplantar keratoderma, perioral hyperkeratosis, leukokeratosis and alopecia. We report a case of this rare syndrome diagnosed in a 48-year-old woman and confirms the existence of a generalized abnormality in keratin expression. Immunoreactivity in our case suggests an abnormal expression of keratins 5 and 14 similar to the observed in other hyperproliferative disorders.
Abstract: A 72-year-old woman developed three consecutive processes that showed characteristics of different neutrophilic dermatoses. First, she developed a picture resembling granuloma faciale, followed by a Sweet's syndrome-like eruption, and then by a superficial pyoderma gangrenosum. She was later diagnosed with myelodysplastic syndrome. This case demonstrates that neutrophilic dermatoses form a spectrum of entities that do not necessarily occur in isolation.
Abstract: This trial was designed to study the efficacy and tolerability of azelastine in controlling symptoms of chronic idiopathic urticaria, using ebastine as validation group. Fifty-two adult patients were randomised to receive azelastine (4 mg), ebastine (10 mg) or 18 placebo for 21 days. Patients were required to visit the investigating physicians on three different occasions (days 0, 7 and 21). On each of these three study days, investigators assessed itching, wheals and erythema, based on a 4-point scale, and quality of life using a visual-analogue scale and subscale 9 of the Short Form 36 (SF-36) Health Survey. Patients entered daily assessments of itching on diary cards also using a 4-point scale. Furthermore, investigators assessed global efficacy and tolerability of the study medication on day 21 or upon premature discontinuation of the trial. Side effects and compliance were evaluated on each visit. A statistically significant reduction in itching was found for both active treatments compared with placebo. These improvements, which were statistically significant already after 1 day of treatment, continued over the course of 3 weeks. Additionally, both azelastine and ebastine were effective in improving symptoms such as wheals and erythema when compared to placebo. The quality-of-life parameters were unaffected by either treatment. Taste perversion (2 cases) and somnolence (1 case) were the only adverse drug reactions of azelastine. Ebastine, however, seemed to cause more often and more severe symptoms such as fatigue, sleepiness and asthenia. Global assessments of efficacy and tolerability performed by the investigators, also favoured azelastine. In conclusion, both azelastine and ebastine are effective and safe drugs, able to control symptoms of chronic idiopathic urticaria since the first day of treatment, and along a period of 3 weeks.
Abstract: BACKGROUND: Mucin deposition is a common feature in autoimmune collagen diseases including dermatomyositis. Nevertheless, clinical manifestations of mucinosis are uncommon in patients with dermatomyositis. Two cases of mucinosis associated with dermatomyositis are reported. PATIENTS: A 53-year-old woman presented with symmetrical plaques on the upper limbs formed by the coalescence of small, violaceous papules. In addition, she showed the typical cutaneous and muscle features of dermatomyositis. A 44-year-old woman with dermatomyositis of 5 years' evolution developed linear, flesh-colored papules across the flexural creases of her palms and fingers. RESULTS: Skin biopsy of the upper limb lesions in the first patient showed epidermal changes compatible with dermatomyositis and dermal mucin deposition. Histopathologic examination of the palmar lesions of the second patient showed less intense epidermal changes of dermatomyositis and dermal mucin deposition. CONCLUSIONS: Mucin deposition in patients with dermatomyositis may have an unusual clinical presentation, and it should be considered in the differential diagnosis of atypical cutaneous lesions in these patients.
Abstract: Docetaxel and pactitaxel [corrected] are the main drugs pertaining to the taxanes family. Nail alterations associated with docetaxel therapy are not a rare event, but they have rarely been reported secondary to paclitaxel therapy, probably because of its more recent use. We present two cases of onycholysis and nail discoloration secondary to paclitaxel therapy.
Abstract: We report the case of a woman with clinical and histopathological features of reticular erythematous mucinosis syndrome (REM) who six years later developed diagnostic criteria for systemic lupus erythematosus (SLE). Two biopsies performed at the same area initially showed mucin dermal deposits and further development of characteristic dermo-epidermal changes of lupus erythematosus. Our findings suggest that SLE may present with an REM-like condition.
Abstract: Several autosomal dominant skin diseases may manifest cutaneous mosaicism. Two types of segmental arrangement can be distinguished: type 1 is characterized by segmental lesions with similar severity to that observed in the diffuse phenotype, the remaining skin being normal; type 2 is characterized by segmental lesions showing a major degree of severity and milder lesions diffusely arranged. Multiple glomus tumours have recently been included in the group of genodermatoses showing type 2 segmental involvement. A family with 2 cases of multiple glomangiomas arranged in a segmental fashion is reported. A 12-year-old girl presented multiple nodular glomangiomas on her right buttock and thigh, in a band-like distribution. A sister of her paternal grandfather showed plaque-like multiple glomangiomas on her left thigh and various glomangiomas on her right buttock and arm. No other family members were known to be affected. Two new cases of familial segmental multiple glomangiomas are reported, with the particularity that one of these exhibited type 2 segmental manifestation and the other type 1 from a clinical point of view.
Abstract: A 42-year-old man with uveitis and a widespread cutaneous eruption, histopathologically characterized by dermal granulomatous infiltrates with perineurial invasion, was incorrectly diagnosed and treated as having borderline leprosy. Further studies demonstrated secondary syphilis and human immunodeficiency virus (HIV) infection. Standard penicillin therapy resolved his cutaneous and ocular lesions. Reports on clinical and pathological findings of active syphilis in HIV infected patients are scarce but this case and isolated previous case reports suggest that granulomatous infiltrates might be a common feature in secondary syphilis with short evolution in HIV infected patients.
Abstract: Ecthyma gangrenosum is considered as a pathognomonic sign of Pseudomonas aeruginosa sepsis. Lesions similar to ecthyma gangrenosum may be caused by other organisms. We report a case of an ecthyma gangrenosum-like eruption caused by Morganella morganii, a Gram-negative bacillus.
Abstract: We report a case with the clinical and histological features of the reticular erythematous mucinosis syndrome (REM), in which there was moderate, continuous, fine, granular, IgM deposition along the basal layer. Similar direct immunofluorescence results have been reported in only two previous cases.
Abstract: We report an 18-year-old man with the unusual combination of Rothmund-Thomson syndrome (RTS) and Addison disease. He was admitted when he was 26 months old because of short stature, dehydration, metabolic acidosis, hyperpigmentation, and typical skin lesions. Because his growth remained delayed, at age 10 years he was given a trial of recombinant growth hormone. After six years of treatment no improvement in height, bone, or sexual maturation was observed. This fact may be related to a defect in connective tissue metabolism. Chromosomal analysis of peripheral blood lymphocytes revealed increased numbers of breaks and gaps. Fibroblasts cultured from affected skin did not grow. Patients with RTS are prone to developing cancer, but no malignant disease was found in our patient. Early diagnosis and treatment of both endocrinologic and malignant complications are essential for survival of patients with this rare syndrome.
Abstract: The p53 protein is the product of a tumour suppressor gene, which is implicated in many human malignancies. p53 expression was investigated by immunohistochemistry in a series of viral warts (n = 12) from five patients with epidermodysplasia verruciformis (EV), using a monoclonal anti-p53 antibody (DO7). p53 expression was also investigated in a series of common warts (n = 8), flat warts (n = 8), and penile bowenoid papulosis (n = 6) from non-EV patients. Immunostaining was positive in 11 of 12 (92%) EV warts, whereas p53 reactivity was negative in most cases of warts from non-EV patients. Exons 5-8 of the p53 gene were screened by the polymerase chain reaction-single strand conformation polymorphism technique in four EV warts, which were strongly stained for p53, and p53 mutations were not detected. These results suggest an association between p53 accumulation (probably of wild type) and EV warts.
Abstract: Report of a case of complete heart block in a patient with systemic lupus erythematosus (SLE). The cardiac disturbance presented as repeated syncopal episodes and normal cardiac clinical and electrocardiographic examination between these episodes. Circulating anti-U1RNP antibodies were detected and tests for anti-SSA/Ro, anti-SSB/La and antiphospholipid antibodies were negative. Evidence of myocarditis was not found. The diagnosis was just established when the patient developed a syncopal episode during hospitalization. Only one SLE case with a similar clinical course has been reported previously.
Abstract: Herein we report a patient with scleromyxedema associated with seronegative polyarthritis and proximal myopathy. The histopathologic and electron microscopic features are described and compared with previously reported patients with documented scleromyxedema and myopathy.
Abstract: Papular xanthoma was diagnosed in a 14-month-old boy. The eruption cleared spontaneously within four years. Immunohistopathologic study revealed that the predominant foamy histiocytic cells had the phenotypic features of the dermal dendrocyte. Giant multinucleated cells, which showed characteristics of monocyte-derived macrophages, were also present. We suggest classifying diseases derived from dermal dendrocytes into a separate group of histiocytoses.
Abstract: We report the case of a 15-year-old girl who simulated a complex clinical picture including cutaneous lesions, fever, arthralgia, asthenia and oliguria. A diagnosis of probable systemic lupus erythematosus (SLE) was assumed by numerous specialists and steroid therapy instituted. Factitious SLE should be considered for differential diagnosis of SLE, especially when laboratory evidence for autoimmunity is not demonstrated.
Abstract: The efficacy and tolerance of 7-chloro-3-[1-(2,4-dichlorophenyl)-2- (1H-imidazol-1-yl)ethoxy-methyl]benzo[b]thiophene (sertaconazole, FI-7045, CAS 99592-32-2) 2% dermatological cream in two daily applications compared with miconazole 2% cream in two daily applications were studied on 631 patients suffering from superficial cutaneous mycosis (sertaconazole n = 317, miconazole n = 314), in a double-blind, controlled multicentre trial with parallel groups. The therapeutic efficacy was evaluated by clinical assessment of the improvement of the lesion and symptoms, a microscopic test on the presence of hyphae or mycelia in the affected area and a culture test on the presence of active infection. Tolerance and safety were evaluated by a general blood analysis and interrogation of the patient on adverse effects. The rate of clinical cures for both treatments at the end of the follow-up was 95.6% for sertaconazole and 88.1% for miconazole, with the difference being statistically significant. In the comparative analysis of the actuarial curve, it was observed that the patients treated with sertaconazole were cured earlier and in a higher proportion than those treated with miconazole, with the difference being significant. The negative result of the microscope examination and culture test confirmed the superiority of sertaconazole over miconazole, already after 14 days of treatment. At the end of the follow-up, 98.6% of the patients in the sertaconazole group obtained a negative culture test result, as opposed to 91.7% in the miconazole group, with the difference being highly significant.(ABSTRACT TRUNCATED AT 250 WORDS)
Abstract: We examined the prevalence of the antibodies to cardiolipin measured by solid-phase enzyme immunoassay during a prospective study of patients with subacute cutaneous lupus erythematosus (SCLE). Seven of 44 (16%) consecutive patients with SCLE had positive anticardiolipin antibodies; of these only three satisfied the American Rheumatism Association's revised criteria for the classification of systemic lupus erythematosus. Clinical findings probably associated with the positive anticardiolipin antibodies were found in four cases, including clotting abnormalities, thrombocytopenia, hemolytic anemia, livedo reticularis, chilblain lupus erythematosus lesions, migraine, leg venous thrombosis and pulmonary embolism after surgery, and spontaneous abortion. Our data suggest that it is reasonable to screen SCLE patients for these antibodies to confirm the presence of the antiphospholipid syndrome.
Abstract: A 43-year-old woman, with an 11-year history of progressive cutaneous lesions, was considered to have granuloma annulare and was treated as such for 4 years. She then developed chronic meningitis. Serological investigation revealed active syphilis. Appropriate antibiotic treatment led to a rapid resolution of the clinical symptoms. The recent reports of similar cases indicate the importance of an adequate knowledge of clinical manifestations and pathological patterns of syphilis.
Abstract: We have retrospectively studied 35 cases of Kaposi's sarcoma in 460 patients with AIDS (incidence of 7.6%) during a period of 10 years. All of them were males, with a mean age of 38 years. 88% of the cases belonged to the homosexual risk group. The tumor was the diagnostic criteria of AIDS in 25 patients. At the moment of the diagnosis, 4 patients were at stage I, 23 at stage II, 1 at stage III and 7 at stage IV, according to the Mitsuyasu's classification; 7 patients had systemic symptoms. The tumor was localized at the skin (34 cases), mucosa (16), digestive tract (7), lung (6) and ganglion (4). The immunological study revealed lymphopenia in 74% of patients, reduction of T4 lymphocytes ( < 0.5 x 10(9)/L) in 93% and inverted T4/T8 ratio in 96%. Sixteen patients received antitumoral treatment (8 with chemotherapy, 7 with interferon and 5 with radiotherapy). The response was stabilization of lesions in 8 cases, partial remission in 2 and progression in 3; in other 3 cases, such response was not assessed. The mortality was 48% and the average survival, 13 months. Opportunistic infections were the cause of death in most patients. Our results confirm the clinical and evolutive characteristics of the Kaposi's sarcoma associated to AIDS; disseminated cutaneous affectation with frequent visceral affectation, poor response to treatment and low survival associated to the presence of opportunistic infections. The lower incidence of tumor observed in our study is related to the different distribution of the risk groups for HIV in our country.
Abstract: Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. The various forms of onset of neonatal lupus erythematosus syndrome are emphasized.
Abstract: We report a 12-year-old girl with nodular skin lesions, fever, pancytopenia, coagulation defects and a fatal outcome within 4 months. Histopathology revealed proliferation of histiocytes with haemophagocytosis in skin, perivisceral fat and haemopoietic organs. The symptoms were most consistent with cytophagic histiocytic panniculitis.
Abstract: We report the case of a 52 year-old male in the chronic phase of chronic myeloid leukaemia, with Philadelphia chromosome due to t(9;22) in the karyotype. He was treated with courses of busulfan and hydroxyurea. Fourteen months after initial presentation, the patient developed fever, non-productive cough, maculonodular violaceous painful skin lesions and bilateral pulmonary infiltrates visible on a chest roentgenogram. Laboratory data, repeated bone marrow aspiration and biopsy and karyotype analysis showed findings similar to those of the initial diagnosis. A biopsy taken from one of the trunk lesions was consistent with Sweet's syndrome. Oral methylprednisolone therapy was initiated at doses of 64 mg daily, and the skin lesions and fever were rapidly resolved. When we reduced the steroid dose, skin lesions and fever recurred. Two further courses of steroid therapy were given with similar results. Finally we treated him with naproxen (750 mg daily for 1 month) with a rapid and stable response. This drug should be considered as an alternative treatment for patients with Sweet's syndrome not responding to corticosteroids or for immunocompromised hosts.
Abstract: We report a case of multiple cutaneous indeterminate cell proliferative lesions in an adult without any other organ involvement. All lesions regressed spontaneously over 5 years without recurrence over a 4-year period of follow-up. The electron-microscopic and immunohistochemical features of the cellular infiltrate were those of the indeterminate cells. This case supports the hypothesis that indeterminate cell proliferative disorder is a wide spectrum of conditions with variegated clinicopathologic presentation and with different biological behavior.
Abstract: Report of a case of multicentric reticulohistiocytosis with clinical, histopathologic, histochemical and immunohistochemical study. Neutral mucins and lipids were demonstrated in the cytoplasm of dermal histiocytes. The monocyte-histiocytic nature of the cells was shown by means of acid phosphatase, ATP-ase, muramidase and alpha-1-antitrypsin. Due to the immunohistochemical results, the relationship with Langerhans cells was rejected. No secretory activity of immunoglobulins was shown and the proliferation qualified as benign. The cells among the histiocytic population were typified as mature T lymphocytes.
Abstract: We report a case of systemic mastocytosis in a 21-year-old male with skin, liver and bone marrow involvement. During the clinical course he developed important gastrointestinal manifestations including a peptic ulcer probably caused by mast cell mediators. In this article we also review the most remarkable characteristics of mastocytosis, especially their systemic forms.
