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Histoid leprosy (HL) was originally described by Wade in 1963 and is regarded as a rare variant of lepromatous leprosy (LL). These characteristic clinical lesions are firm, deeply adhered nodules with features reminiscent of dermatofibromas or keloids in a background of apparently healthy skin. The main histopathological findings described are the presence of spindle cell histiocytes immersed in a richly collagenized background, usually forming a nodular pattern of infiltration with sharply delimitation and positive staining for acid-fast bacilli. The classical form of HL lesions should be devoid of foam histiocytes and globi. However, we and other authors noticed that in most of the cases, despite characteristic clinical features, histopathology depicts a mixture of LL and HL patterns. Therefore, we present a case with clinical features similar to HL in which an excisional scalpel biopsy of a nodule demonstrated features of classical LL in the center of the lesion and features of HL in the periphery, highlighting that a proper biopsy technique could enhance the ability of the dermatopathologist to histopathologically diagnose cases of HL. In cases in which HL is clinically suspected, we advocate replacing the usual 4-mm incisional punch biopsy by a broader elliptical scalpel biopsy, encompassing the totality of the lesion whenever possible to achieve a reliable representation of the pathologic process.

INTRODUCTION Heparin-induced bullous hemorrhagic dermatosis (HBHD) is an unusual cutaneous adverse reaction to heparin characterized by acute onset of hemorrhagic bullae. METHODOLOGY An exuberant case of HBHD is reported, and a revision of previously reported cases indexed on PubMed is provided. Cases were tabulated to the following parameters: sex, age, type of heparin and route of administration, associated medical procedure, number of days between heparin start and onset of lesions, site of the lesions, level of blister on histopathology, inflammatory infiltrate on histopathology, direct immunofluorescence findings, heparin-related intervention, number of days to resolution of skin lesions, associated hemorrhagic event, and outcome. CASE REPORT A 21-year-old woman with end-stage renal disease was receiving intravenous unfractioned heparin (UFH) during hemodialysis section for the past 3 months. Four hours after using for the first time an arteriovenous fistula punctured on her right wrist, the patient noticed the onset of vesicles and blisters on the right forearm containing citrus or serohemorrhagic exudate, which became overt hemorrhagic in 24 hours. Histopathology depicted a nonacantholytic subcorneal blister containing erythrocytes and plasma without any significant dermal inflammatory infiltrate. RESULTS Sixty cases of HBHD were included. HBHD affected predominantly men, with a male/female rate of 2.75. The age range was from 21 to 94 years, with an average of 70.8 and a median of 72 years. Nine patients used UFH, and 54 patients used low molecular weight heparins (3 patients used both). The lapse of time between the start of heparin and the onset of skin lesion varied from 6 hours to 240 days, with an average of 17.3 days and a median of 7 days. Limbs were affected in most of the cases. The level of the blister was subcorneal in 10 patients, intraepidermal in 30, subepidermal in 8, and both intraepidermal and subepidermal in 1. In 33 cases, there was no significant dermal inflammatory infiltrate. Pure lymphocytic inflammatory infiltrate was present in 10 cases. Eosinophils were found within the dermal inflammatory infiltrate in 2 cases, neutrophils in 2, and a mixture of eosinophils and neutrophils in other 2. Direct immunofluorescence was performed in 18 cases, all of them with negative results. DISCUSSION We hypothesize that mechanical trauma, skin fragility, and the anticoagulation effect of heparin might contribute concomitantly to the development of the lesions and speculate that subepidermal blisters in HBHD could be formed by rupture of the floor of a former intraepidermal blister.

Erythema Nodosum Leprosum (ENL) occurs due to the immunological complication of multibacillary leprosy and is characterized by painful nodules and systemic compromising. It is usually recurrent and/or chronic and has both physical and economic impact on the patient, being a very important cause of disability. In addition, ENL is a major health problem in countries where leprosy is endemic. Therefore, adequate control of this condition is important. The management of ENL aims to control acute inflammation and neuritis and prevent the onset of new episodes. However, all currently available treatment modalities have one or two drawbacks and are not effective for all patients. Corticosteroid is the anti-inflammatory of choice in ENL but may cause dependence, especially for chronic patients. Thalidomide has a rapid action but its use is limited due the teratogenicity and neurotoxicity. Clofazimine and pentoxifylline have slow action and have important adverse effects. Finally, there is no pattern or guidelines for treating these patients, becoming more difficult to evaluate and to control this condition. This review aims to show the main drugs used in the treatment of ENL and the challenges in the management of the reaction.

Objective In this study, we propose a modification to the simple decompression technique that contains the ulnar nerve in the cubital fossa, thus preventing subluxation during forearm flexion movements. Methods Five consecutive patients with leprosy-associated cubital tunnel syndrome underwent surgery with the modified technique between July 2011 and October 2012. Results The most common symptoms were neuropathic pain and sensory changes (both 60%). On the McGowan scale, three patients maintained their preoperative score and two patients improved by two points, while on the Louisiana State University Health Sciences Center scale, two patients maintained the same scores, two improved by two points, and one improved by one point. Four patients were able to discontinue corticosteroid use. The mean follow-up time was 25.6 months (range 2-48 months). There were no recurrences or subluxations in the long-term. Conclusion This alternative technique resulted in excellent functional results, as well as successful withdrawal from corticosteroids. Furthermore, it resulted in no ulnar nerve subluxations. RESUMO Objetivo Neste manuscrito apresentamos uma modificação da técnica de descompressão simples do nervo ulnar no túnel cubital que impede a subluxação do nervo em movimentos de flexão do antebraço. Métodos Foram incluídos cinco pacientes consecutivos acometidos por síndrome do túnel cubital (Hanseníase) submetidos à cirurgia entre 2011 e 2012. Resultados Os sintomas mais comuns foram dor neuropática e alterações sensitivas (60%). No pós-operatório, três pacientes mantiveram o mesmo escore e dois melhoraram dois pontos na escala de McGowan, enquanto na escala Louisiana State University Health Sciences Center, dois pacientes mantiveram o mesmo escore, dois melhoraram dois pontos e um melhorou um ponto. Os corticosteróides foram descontinuados em quatro pacientes. O tempo médio de seguimento foi 25,6 meses (variação 2-48 meses). Não foram observadas recorrência ou subluxação no longo prazo. Conclusões A técnica alternativa apresentou excelentes resultados funcionais e foi bem sucedida na retirada dos corticosteróides. Ademais, subluxações do nervo ulnar não foram observadas.

Cytomegalovirus is an opportunistic virus that commonly affects immunosuppressed patients. Cutaneous involvement by this virus is rare and occurs in significantly immunocompromised hosts, with a poor prognosis. Skin ulcers may represent the first sign of systemic infection by cytomegalovirus in these patients. Herein, a case of a systemic infection by Cytomegalovirus presenting as genital and oral ulcers in a kidney-transplant recipient is reported.

OBJETIVO: Descrever dois casos de hanseníase em menores de 15 anos, sendo um de paciente com 18 meses de idade e outro de 13 anos, diagnosticados por modos de detecção diferentes, ressaltando a importância de examinar os contatos. DESCRIÇÃO DO CASO: Um dos casos foi diagnosticado precocemente por meio do exame de contatos intradomiciliares, enquanto o outro foi diagnosticado por demanda espontânea após quatro anos de aparecimento das lesões e, apesar de ser contato de um ex-paciente, não foi examinado na época. COMENTÁRIOS: Em países endêmicos, a alta detecção da hanseníase em menores de 15 anos revela a persistência na transmissão do bacilo e as dificuldades dos programas de saúde para o controle da doença. O maior tempo para diagnóstico ocasiona sequelas e deformidades e, dessa forma, a busca dos contatos constitui importante método para o diagnóstico precoce da doença na infância, quando os sinais clínicos nem sempre são fáceis de serem identificados e há grande diversidade de formas clínicas em que a doença pode se apresentar.

Berardinelli-Seip syndrome is a rare autosomal recessive disease characterized by inadequate metabolism and inefficient storing of lipids in fat cells, generating accumulation of fat in organs such as the liver, spleen, pancreas, heart, arterial endothelium and skin. Classically, patients manifest generalized lipoatrophy at birth or until 2 years of age, and in adolescence usually develop marked insulin resistance with rapid progression to diabetes and dyslipidemia. We report the case of a 17-year-old Berardinelli-Seip syndrome patient with eruptive xanthoma associated with severe hypertriglyceridemia. It is worth noting Eruptive xanthoma as a dermatological manifestation that is not generally highlighted in the reports of cases of this genetic metabolic disorder.

Hidradenitis suppurativa is an inflammatory disease with difficult treatment. Currently, the anti-TNF alfa therapy, with monoclonal antibodies ('biological therapy'), has been shown as a new alternative. However, clinical trials assessing the efficacy of these drugs to treat hidradenitis suppurativa have not yet been published. A case in which the use of infliximab did not result in significant clinical improvement is described.

Objetivo: Descrever ação para o controle da hanseníase por meio da educação em saúde e da busca ativa de casos na faixa etária escolar, em uma instituição pública de ensino do município de Ananindeua, Pará. Métodos: Trata-se de estudo descritivo, conduzido em uma instituição pública de ensino, incluindo estudantes do ensino fundamental. Em outubro e novembro de 2010 foram realizadas palestras na escola sobre hanseníase e outras dermatoses e, em seguida, foi feita uma triagem com os alunos que apresentaram alguma mancha na pele. No total de 532 alunos envolvidos, foram identificados 55 alunos como casos suspeitos para hanseníase. Esses alunos foram conduzidos para consulta dermatológica, sendo realizados o exame clínico e a aplicação de questionários sociodemográficos. Resultados: A face (49%) e os membros superiores (45%) foram as principais regiões do corpo com lesões. Na maioria dos casos investigados tratava-se de pitiríase alba (42%) e pitiríase versicolor (31%). Entretanto, diagnosticou-se um caso de hanseníase (2%), na forma clínica tuberculóide. Conclusão: Considerando a abrangência do estudo, o número de casos de hanseníase encontrado é significativo e reafirma a alta taxa de detecção de hanseníase em menores de 15 anos no município. Ações de vigilância epidemiológica devem ser intensificadas, no sentido de detectar a doença mais precocemente, contribuindo para a redução de estigmas e da restrição à participação social.

Berardinelli-Seip syndrome is a rare autosomal recessive disease characterized by inadequate metabolism and inefficient storing of lipids in fat cells, generating accumulation of fat in organs such as the liver, spleen, pancreas, heart, arterial endothelium and skin. Classically, patients manifest generalized lipoatrophy at birth or until 2 years of age, and in adolescence usually develop marked insulin resistance with rapid progression to diabetes and dyslipidemia. We report the case of a 17-year-old Berardinelli-Seip syndrome patient with eruptive xanthoma associated with severe hypertriglyceridemia. It is worth noting Eruptive xanthoma as a dermatological manifestation that is not generally highlighted in the reports of cases of this genetic metabolic disorder.

Herpes simples crônico é doença capaz de gerar apresentações exuberantes, principalmente em pacientes com sida. O provável mecanismo da formação dessas lesões hiperplásicas consiste na invasão viral dos linfócitos T CD4 situados na epiderme. Diante das dificuldades terapêuticas e da grande taxa de insucesso do tratamento nesses pacientes, novas terapias têm sido citadas na literatura atualmente. O conhecimento da imunopatologia ratificou o mecanismo pelo qual o imiquimode poderia ser eficaz como terapia adjuvante aos antivirais. Relatamos aqui dois casos de pacientes tratados com aciclovir associado ao imiquimode tópico que obtiveram resposta clínica excepcional. Chronic herpes simplex can present as exuberant clinical lesions, especially in HIV patients. The most probable mechanism of formation of these lesions is the invasion of the epidermal CD4 T cells by the herpes simplex virus. Due to the therapeutic difficulties and the high rates of treatment failure amongst these patients, new drugs are currently being discussed on the literature. Studies based on the immunophatology of these lesions have suggested that imiquimod might work as an adjuvant therapy to the antiviral drugs. Here we present two cases of excellent response to treatment with topical imiquimod as an adjuvant drug to acyclovir.

O Dermatomiofibroma está incluído no grupo de lesões neoplásicas mesenquimais benignas de linhagem fibroblástica e miofibroblástica da pele. É uma doença rara, havendo aproximadamente 100 casos descritos na literatura mundial até o momento. Este artigo relata o caso de uma mulher jovem com apresentação clínica típica e diagnóstico histopatológico de dermatomiofibroma. Foram realizadas colorações especiais que mostraram preservação das fibras colágenas e a imunohistoquímica revelou positividade para vimentina e negatividade para actina e S100. Por se tratar de doença rara, os achados histopatológicos são de grande importância, mas a supeição clínica é possível em casos típicos como este. Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.

Dermato myofibroma is included in the group of benign cutaneous mesenchymal neoplastic lesions of fibroblastic and myofibroblastic lineage. It's a rare disease and there are approximately only one hundred cases described worldwide in the medical literature up to now. The present study reports the case of a young woman with typical clinical cutaneous lesion and histopathological diagnosis of dermato myofibroma. Special stains were carried out which showed preserved collagen fibers and immunohistochemistry was positive for vimentin and negative for actin and S100. As it is a rare disease, the histopathological findings are of great importance but clinical suspicion is possible in typical cases such as this one.

Leprosy is an infectious disease with an average incubation period of two to five years. It is caused by Mycobacterium leprae, mainly affecting skin, mucous membranes and peripheral nerves. When it occurs in children under fifteen, it reflects an intense and long contact period, with a high bacillary load. Therefore, it is considered an important alert sign that points to the challenge of controlling the disease. The authors report three cases of leprosy in children under fifteen that occurred in the Itaguaí district, Rio de Janeiro. The epidemiologic implications of new cases detected at this age and the fundamental role of examining the patients' close contacts and the possible identification of source cases as an effort for leprosy control are discussed.

Hidradenitis suppurativa is an inflammatory disease with difficult treatment. Currently, the anti-TNF alfa therapy, with monoclonal antibodies ('biological therapy'), has been shown as a new alternative. However, clinical trials assessing the efficacy of these drugs to treat hidradenitis suppurativa have not yet been published. A case in which the use of infliximab did not result in significant clinical improvement is described.