Abstract: Summary Folliculotropic mycosis fungoides is a variant of mycosis fungoides with a worse prognosis than the classic form. It can be associated with follicular mucinosis. We report a case of folliculotropic mycosis fungoides developing in the months following methotrexate therapy in a psoriatic patient. This lymphoma did not regress after stopping the antipsoriatic treatment. There is a known relationship between the use of immunosuppressive therapies and the development of lymphoproliferative malignancies. However these lymphomas are mainly B-cell non-Hodgkin lymphomas associated with Epstein-Barr virus. In our patient the short period of time from the beginning of the immunosuppressive treatment to the occurrence of the T-cell lymphoma does not support a strong causal relationship between the drug intake and the development of mycosis fungoides. To the best of our knowledge, this is the first case of folliculotropic mycosis fungoides in a patient treated with methotrexate for psoriasis.
Abstract: Summary Acute generalized exanthematous pustulosis (AGEP) is a rare disorder, usually drug-induced, and is clinically characterized by widespread, non-follicular aseptic pustules. Although spontaneous resolution usually occurs once the causative drug has been withdrawn, more severe cases often require treatment with systemic corticosteroids. We report a 63-year-old woman who developed AGEP after a 30-day course of hydroxychloroquine. Extensive re-exacerbation of AGEP after an 18-day course of methylprednisolone led us to switch the treatment to oral ciclosporin with a prompt and satisfactory improvement. Ciclosporin has many inhibitory effects on the main cell population (T cells) involved in AGEP. In particular, a significant reduction in producton of interleukin-8 by T cells is a possible explanation of the rapid remission observed in this case. To our knowledge, this is the first reported case of AGEP successfully treated with ciclosporin.
Abstract: Linear and whorled nevoid hypermelanosis is a sporadic pigmentary anomaly occurring within the first weeks of life, characterized clinically by swirls and streaks of macular hyperpigmentation following the lines of Blaschko. Histologically it shows only epidermal melanosis. Underlying chromosomal mosaicism has been demonstrated in only a few published cases. Progressive cribriform and zosteriform hyperpigmentation is considered to be the localized variant of linear and whorled nevoid hypermelanosis. We report a retrospective study on 16 children referred consecutively over a 10-year period for evaluation of segmental, linear or swirled hyperpigmentation distributed along the lines of Blashko, consistent with a diagnosis of linear and whorled nevoid hypermelanosis. Associated abnormalities were found only in one out of six patients with the diffuse form (linear and whorled nevoid hypermelanosis-type) and in none of the remaining 10 children presenting the unilateral form (progressive cribriform and zosteriform hyperpigmentation-type). A long-term follow-up did not disclose further abnormalities. The authors discuss the nosologic position of this entity with respect to hypomelanosis of Ito. Linear and whorled nevoid hypermelanosis and hypomelanosis of Ito should be not considered single entities, but be rather grouped as a heterogeneous collection of nonspecific pigmentary disorders caused by genetic mosaicism. Skin findings in these diseases can differ according to the pigmentation in the normal cell line and whether the second line contains more or less melanosomes than the normal skin of the individual exhibiting mosaicism.
Abstract: Folliculitis spinulosa decalvans is an uncommon condition characterized by follicular hyperkeratosis, followed by scarring alopecia. We report a 12-year-old boy affected by keratotic papules of the scalp and keratosis pilaris of the limbs who developed erythema, pustules, and scale crusts on the scalp associated with scarring alopecia. Histologic examination showed follicular and interfollicular hyperkeratosis, follicular plugging, mild inflammation, and focal scarring. A transient remission of the inflammatory changes on the scalp was obtained after treatment with isotretinoin. The follicular spinulous hyperkeratosis persisted. A severe relapse of the scalp inflammation was observed during a 2-year follow-up.
Abstract: Rotavirus infection is one the most frequent cause of diarrhea among infants and children. Although it is not associated to specific dermatologic clinical pictures, recently, different clinical manifestations have been reported in association with this infection. They include exanthema, Gianotti-Crosti syndrome, and Acute Infantile Hemorrhagic Edema. The condition can be diagnosed microbiologically with stool cultures. The prognosis is excellent in healthy, immunocompetent individuals.
Abstract: We report the cases of five female patients with high skin phototype affected by relapsing, hypochromic, non-scaling macules occurring after the summer on the back and spreading over large areas of skin. Histological features disclosed decreased epidermal melanin. Psoralen plus ultraviolet A (PUVA) treatment proved to be beneficial, but new relapses were noted after stopping treatment. Clinical and histological features were consistent with the diagnosis of 'progressive and extensive hypomelanosis' described by Guillet in persons of mixed racial background. We discuss the differential diagnosis of the latter entity with respect to the other idiopathic acquired primitive hypomelanosis and hypothesize an overlapping with the so-called extensive pityriasis alba (EPA).
Abstract: Imiquimod, the first member of a new class of immune response modifiers, is approved for the treatment of external genital and perianal warts. Recently, many clinical trials highlighted the potential of imiquimod as a treatment for other viral infections and cutaneous neoplasms. We report two cases of facial keratoacanthomas (KA) treated with topical 5% imiquimod cream. Patients were successfully cleared of KAs after treatment for 8 weeks. No recurrence occurred after a 1-year follow-up. Despite the fact that KAs are characterized by the potential for spontaneous regression, it is possible that a faster activation of CD4+ lymphocytes, via interferon release and cytokine secretion takes place after imiquimod application leading to KA regression.
Abstract: Infantile acute hemorrhagic edema (AHE) is a benign condition characterized by a dramatic onset of inflammatory edema and ecchymotic purpura in a target or cockade pattern. It is considered an uncommon form of cutaneous vasculitis occurring in children younger than 2 years of age. The outbreak is frequently preceded by an immunization or various infections. We describe an 11-month-old girl with rosette-shaped purpuric plaques on the face and limbs, clinically consistent with a diagnosis of AHE of the skin, associated with fever and diarrhea. Laboratory investigations showed a rotavirus infection, which has not previously been reported in association with AHE of the skin. The disease had a benign course without relapses. Appropriate microbiologic investigations are advisable to confirm the possible etiologic role of rotavirus.
Abstract: A 27-year-old woman with a 5-month history of recurrent erythema nodosum was found to have Hodgkin's disease. A temporal relationship between the two disorders suggested a causative role of the lymphoma. A review of the literature yielded 15 cases of this association, suggesting that the diagnosis of Hodgkin's disease should be considered in patients with unexplained erythema nodosum.
Abstract: Achromic nevus or nevus depigmentosus is a cutaneous abnormality consisting of a hypopigmented macular lesion which can present as circumscribed irregular, oval, or round or as a unilateral band or streak with a blocklike configuration or arranged along one or more Blaschko lines. When it is systematized, it is indistinguishable from hypomelanosis of Ito. Pigmentary anomalies along the lines of Blaschko can be associated with systemic features. For these reasons, 20 children, referred consecutively for evaluation of segmental, linear achromic nevus, were evaluated to define the incidence of associated abnormal systemic features. Extracutaneous abnormalities were present in 2 of 20 children (10%). The anomalies consisted of pes cavus ipsilateral to the hypopigmentation in one child and mental retardation, seizures, and hemihypertrophy in the second. This study confirms that achromic nevus, even when distributed along Blaschko lines, is commonly a benign lesion that is associated with systemic features in a small minority of cases.
Abstract: The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) is characterized by hyponatraemia due to water retention resulting from the persistent release of antidiuretic hormone (vasopressin). It may occur in a variety of malignant and non-malignant conditions, in particular in association with oat cell carcinoma, pulmonary and cerebral diseases. We report the case of a male patient affected by melanoma of the right temporal region with brain metastasis who developed acute headache, drowsiness, nausea, vomiting and pathological reflexes. Clinical and laboratory investigations led us to the diagnosis of SIADH. Restriction of fluid intake obtained a good clinical improvement with normalization of laboratory alterations; after 2 months the patient experienced a new episode of SIADH which was promptly treated. As melanoma has been occasionally observed in association with SIADH it should be included in the list of tumours that can cause this particular syndrome.
Abstract: A case of multiple miliary osteomas of the face in a 68-year-old female with no personal history of acne or former inflammatory skin disorders is reported. The patient showed multiple painless, stone-like formations at several sites of the face. Histological, echographic and radiological investigations confirmed the presence of bone tissue in the dermal layer. Multiple miliary osteoma of the face is considered the rarest variant of primary osteoma.
Abstract: We report the case of a 26-year-old man affected by a symmetrical keratoderma localized to the interdigital spaces of the fingers. No occupational, traumatic, or irritant factors were discovered. Clinical and histological features were consistent with the diagnosis of symmetrical interdigital hyperkeratosis, a sporadic disorder described by Frei in 1926. We believe this condition to be less rate than the few cases reported in the literature would suggest.
Abstract: We report an infant with a rare form of epidermolysis bullosa simplex characterized by an autosomal recessive pattern of inheritance, severe cutaneous involvement, oral and nail lesions, associated with muscular dystrophy, and a poor prognosis, due to extracutaneous disease. In addition to the usual presentation of this disease, our patient had severe anemia, with immature circulating white cells, and bone marrow histology suggestive of a pre-leukemic state, a finding which has not before been reported in the literature.
Abstract: Congenital symmetrical interdigital hyperkeratosis is a rare disorder described by Frei in 1923, characterized by localized hyperkeratosis of the interdigital spaces of the hands and feet. The authors report the case of a 7-year-old girl affected by this condition. The skin hyperkeratotic lesions appeared localized exclusively to the interdigital spaces of the hands. Histologic examination showed orthokeratotic hyperkeratosis, hypergranulosis and acanthosis. Keratolytics failed to improve the clinical picture.
Abstract: A syndrome characterized by palmoplantar keratoderma, sclerodactyly, and skin cancer was first described in two families by Huriez et al. The pattern of inheritance was compatible with that of an autosomal dominant disorder. We report a patient with this condition and review the literature.
Abstract: The authors report the third case of eosinophilic pustular folliculitis (EPF) associated with a non-Hodgkin lymphoma. The dermatosis occurred after an autologous bone marrow transplantation performed as treatment for the lymphoproliferative disorder. Although EPF was initially described as an idiopathic disease, the association of some cases with immunologic alterations or diseases, such as immunodeficiencies, suggests a possible immunopathologic event in the pathogenesis of EPF.
Abstract: Focal facial dermal dysplasia (or congenital ectodermal dysplasia of the face) is an inherited condition characterized by congenital scarlike lesions on the temples and a wide spectrum of associated facial abnormalities. We report the case of a woman and her son with this disorder. An autosomal dominant inheritance with variable penetrance and expressivity may explain the different clinical expressions of focal facial dermal dysplasia.
Abstract: Twenty-six children with a symmetric papular or papulovesicular acrolocated eruption were studied to evaluate a possible link with viral infection. A history of a recent immunization was present in four patients, two of whom had a polio vaccine virus isolated from their stool samples. There was serologic evidence of a recent Epstein-Barr virus infection in one patient, adenovirus infection in another, a concurrent Epstein-Barr virus and adenovirus infection in a third, and cytomegalovirus infection in a fourth. Viral cultures were positive in two patients for adenovirus and in two others for rotavirus. Although some of the virologic findings could have been coincidental, this study confirms that multiple viral infections can be related to most papular and papulovesicular acrolocated eruptions of childhood. Virologic investigations in the earlier phases of the eruption are recommended to identify the etiologic factors of these lesions.
Abstract: Thirty-five children affected by a recurrent summer and spring papular eruption on the elbows, knees, and backs of the hands were examined retrospectively in order to evaluate the frequency of atopy. It was found that 45.7% were suffering from atopic diseases (atopic eczema, allergic rhinitis, asthma). This high proportion confirms the fact that an underlying atopy may predispose to the occurrence of this dermatosis.
