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.
5 June 2012 in Journal of Dermatological Treatment
Introduction: Hidradenitis suppurativa (HS) is a chronic disease with painful, inflamed lesions in the apocrine gland-bearing areas of the body and unsatisfactory treatment. TNFα blockers have been proposed as promising treatments, but only few randomized, short-term, small controlled trials have been conducted. The aim of this study was to evaluate long-term response of HS patients treated with infliximab (IFX). Material and methods: A long-term, prospective study of 10 patients with moderate–severe refractory HS treated with IFX was performed, including assessment of therapy safety, disease severity, and activity. Previous reports on IFX treatment for HS were reviewed. Results: Lack of response was observed in 20% and relapse in 50% of patients, after a median period of 37 weeks. The median number of doses administered was 7.5 during 49 weeks. No life-threatening adverse events were detected. Systematic review of 61 previously published cases showed lack of response was associated with previous surgery, young age at diagnosis, and long time of evolution of the disease. Conclusions: Long-term IFX therapy might be an efficient, well-tolerated, safe option for patients with short-time evolution, severe HS. Relapse is common after 8 months of continuous treatment, especially in patients with more severe disease and in those treated with IFX in monotherapy.
29 August 2012 in Journal of the European Academy of Dermatology and Venereology
Context.—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. Objective.—To review the most useful, evidence-based practice criteria for establishing the diagnosis of CMC, for which universally accepted criteria are lacking, in order to facilitate the interpretation and comparison of the results from different institutions, and to perform systematic reviews and meta-analysis. Data Sources.—A comprehensive review of the most relevant previous single-institution series reported in the literature since 1990, including our cumulative experience of 137 cases of primary cutaneous and mucosal melanoma in patients younger than 18 years. Special characteristics of melanoma in children are discussed, regarding clinical settings and risk factors, helpful histologic features, and immunohistochemical patterns for diagnosis and prognosis. Conclusions.—Careful analysis of histologic features as well as the additional information provided by immunohistochemistry should allow for a correct diagnosis in most cases of melanoma in children. Although it seems that pediatric patients with melanoma have higher survival probability than adults, still a number of children will develop metastasis and die of their disease, particularly when melanoma is diagnosed after puberty. Until further studies more accurately determine the prognosis, a prudent approach to CMC diagnosis and therapy seems to follow the same principles as those established for adult melanoma.