Granulomatous slack skin: a rare subtype of mycosis fungoides.

We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.

Granulomatous slack skin: a rare subtype of mycosis fungoides

Abstract: We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.

Líquen plano pilar: a importância do diagnóstico precoce / Lichen planopilaris: the importance of early diagnosis

O líquen plano pilar é desordem rara da ordem das alopecias cicatriciais primárias. Descreve- se um caso de paciente do sexo feminino, portadora de placas eritêmato-descamativas e plugues foliculares em região frontoparietal bilateralmente, associados a teste de tração positivo. Após biópsia compatível com líquen plano pilar, a paciente foi tratada com prednisona associada a clobetasol, apresentando repilação da área acometida. O tratamento dessa patologia é um desafio devido à escassez de dados sobre eficácia das terapêuticas e constante recidiva. Trata-se de quadro irreversível se não for tratado precocemente. É descrito um caso clássico de líquen plano pilar com boa resposta terapêutica, destacando- -se a importância do diagnóstico precoce, já que em fase inicial a maioria das alopecias cicatriciais é não cicatricial, devendo, por esse motivo, ser manejada como emergência em tricologia.

Lichen planopilaris is a rare disorder that belongs to the primary scarring alopecia type. The present study describes the case of a female patient bearing desquamative erythematous plaques and follicular plugs bilaterally in the frontoparietal region, associated with positive pull test. The biopsy's result was consistent with lichen planopilaris and the patient was treated with prednisone associated with clobetasol, with regrowth of the hair in the affected area. The treatment of this pathology is a challenge due to the lack of data on efficacy of therapies and constant recurrence. The picture is irreversible if not treated early. This paper describes a classic case of a case of lichen planopilaris with good therapeutic response, highlighting the importance of early diagnosis, due to the fact that most cicatricial alopecias do not produce scarring in their initial stage and should for this reason be managed as an emergency in trichology.

Carcinoma de células de Merkel: apresentação clínica, dermatoscópica e imuno-histoquímica de um tumor raro / Merkel cell carcinoma: clinical, dermoscopic and immunohistochemicalaspects of a rare tumor

Carcinoma de células de Merkel é tumor cutâneo neuroendócrino raro e altamente agressivo. Objetiva-se neste artigo alertar para a possibilidade desse diagnóstico, geralmente não considerado hipótese inicial em neoplasias cutâneas. Relatamos dois casos de pacientes do sexo feminino, idosas com queixa de nódulo único eritematoso na face. Aventaram-se hipóteses diagnósticas de carcinoma basocelular e melanoma amelanótico. À dermatoscopia foi evidente a presença de telangiectasias O exame histopatológico da biópsia incisional diagnosticou carcinoma de células de Merkel. Essa neoplasia ocorre tipicamente em pacientes brancos, acima de 65 anos, manifestando-se como nódulo eritêmato-violáceo, de crescimento rápido, sendo a imuno-histoquímica essencial para o diagnóstico.

The Merkel cell carcinoma is a rare and highly aggressive neuroendocrine skin tumor. The purpose of this paper is to warn of the possibility of this diagnosis, usually not considered as an initial hypothesis in cutaneous neoplasias. The authors describe two cases of elderly female patients with complaints of a single erythematous nodule on the face. The diagnoses of basal cell carcinoma and amelanotic melanoma were considered. The presence of telangiectasias was evident at dermoscopy. The incisional biopsy's histology evidenced Merkel cell carcinomas. This neoplasia typically occurs in Caucasian patients with over 65 years of age, emerging as an erythematous-purplish nodule of rapid growth, with immunohistochemistry being essential for the diagnosis.

Cutaneous histoplasmosis as a complication after anti-TNF use--Case report.

Anti-TNF agents are effective in the treatment of psoriasis. However, they render individuals more susceptible to infections. We report an atypical case of histoplasmosis in an immunosuppressed patient due to anti- TNF therapy. A patient who used anti-TNF for the treatment of psoriasis had had a lesion on the right eyebrow since discontinuation of the medication. The diagnostic hypothesis was basal cell carcinoma, but the histopathological examination was compatible with histoplasmosis.

Actinic comedonal plaque-variant of Favre-Racouchot syndrome: report of two cases.

The actinic comedonal plaque is characterized by papules, cysts and comedones forming a yellowish plaque in areas of chronic sun exposure skin. There are few reports in literature about this entity, considered a rare and ectopic form of Favré-Racouchot Syndrome. We report two cases of lesions located on forearms and thorax. Favré-Racouchot Syndrome is a condition usually restricted to the periorbital area; however, there are reports of similar findings in atypical locations, such as forearms and chest, which are known as actinic comedonal plaque. Ultraviolet radiation exposure is the main factor involved in its pathogenesis. The objective of this study was to provide accurate knowledge of this dermatosis and stimulate dermatologists to provide a correct diagnosis of the condition.

Actinic comedonal plaque-variant of Favre-Racouchot syndrome: report of two cases

The actinic comedonal plaque is characterized by papules, cysts and comedones forming a yellowish plaque in areas of chronic sun exposure skin. There are few reports in literature about this entity, considered a rare and ectopic form of Favré-Racouchot Syndrome. We report two cases of lesions located on forearms and thorax. Favré-Racouchot Syndrome is a condition usually restricted to the periorbital area; however, there are reports of similar findings in atypical locations, such as forearms and chest, which are known as actinic comedonal plaque. Ultraviolet radiation exposure is the main factor involved in its pathogenesis. The objective of this study was to provide accurate knowledge of this dermatosis and stimulate dermatologists to provide a correct diagnosis of the condition.

Melanoníquia estriada secundária a fibroma da matriz ungueal pigmentado simulando melanoma nodular / Melanonychia striata secondary to pigmented nail matrix fibroma simulating nodular melanoma

Melanoníquia é a coloração da lâmina ungueal variando do marrom ao negro. Representa um desafio diagnóstico, pois há diversos diagnósticos diferenciais incluindo entidades benignas e malignas. Não há relatos de fibroma da matriz ungueal pigmentado causando melanoníquia longitudinal. Diante disso, os autores relatam um caso de melanoníquia estriada secundária a fibroma ungueal pigmentado, com achados do exame clínico e dermatoscópico sugestivos de melanoma nodular.

Melanonychia corresponds to color patterns in the nail plate, ranging from brown to black. It is a diagnostic challenge due to the fact it has several differential diagnoses, including benign and malignant entities. There are no reports of pigmented fibroma of the nail matrix causing striata (or longitudinal) melanonychia. In light of this fact, the authors report a case of melanonychia striata secondary to pigmented fibroma of the nail, with clinical examination and dermoscopic findings suggestive of nodular melanoma.

Pseudoxanthoma elasticum: report of two cases.

Pseudoxanthoma elasticum is a rare inherited multisystem disorder that is characterized by a pathological mineralization of the elastic connective tissue, which involves predominantly the skin, eyes and cardiovascular system. Its cause lies on mutations in the ABCC6 gene, which lead to reduction or absence of the transmembrane transport ADP dependent protein (MRP6), causing an accumulation of extracellular material and subsequent deposition of calcium and other minerals in the elastic tissue. The authors report two cases of pseudoxanthoma elasticum, emphasizing its major clinical features and the importance of early diagnosis of the disorder, aiming for adequate therapeutic management of associated complications.

Pseudoxanthoma elasticum: report of two cases

Pseudoxanthoma elasticum is a rare inherited multisystem disorder that is characterized by a pathological mineralization of the elastic connective tissue, which involves predominantly the skin, eyes and cardiovascular system. Its cause lies on mutations in the ABCC6 gene, which lead to reduction or absence of the transmembrane transport ADP dependent protein (MRP6), causing an accumulation of extracellular material and subsequent deposition of calcium and other minerals in the elastic tissue. The authors report two cases of pseudoxanthoma elasticum, emphasizing its major clinical features and the importance of early diagnosis of the disorder, aiming for adequate therapeutic management of associated complications.

Fibromixoma acral superficial em paciente do sexo feminino: um relato de caso / Superficial acral fibromyxoma in a female patient: a case report

Descreve-se caso de paciente do sexo feminino de 75 anos, apresentando há três anos lesão única, assintomática, nodular, de consistência fibroelástica, localizada no primeiro pododáctilo esquerdo. Indicada a exérese cirúrgica da lesão, o exame anatomopatológico e a imuno-histoquímica revelaram fibromixoma acral superficial. Esse tipo de lesão foi descrito pela primeira vez em 2001, e hoje há aproximadamente 100 casos na literatura. Trata-se de tumor mesenquimal benigno, de lento crescimento, com predileção por regiões ungueais e periungueais. Não há relatos de transformação maligna, e a recorrência tem sido associada à ressecção incompleta.

The authors describe the case of a 75-year-old female patient, bearing a single asymptomatic, nodular lesion of fibroelastic consistency for three years, located on the left hallux. Following surgical excision of the lesion, the pathological examination and immunohistochemistry revealed a superficial acral fibromyxoma. This type of lesion was first described in 2001 and today there are approximately 100 cases in the literature. It is a benign mesenchymal tumor of slow growth, with a predilection for ungual and peri-ungual regions. There are no reports of malignant transformation and recurrence has been associated with incomplete resection.

Onicomatricoma: tumor raro do aparelho ungueal: relato de três casos / Onychomatricoma: a rare tumor of the nail apparatus: report of three cases

Os onicomatricomas são tumores benignos raros que se originam a partir da matriz ungueal e do estroma subjacente. São geralmente assintomáticos e de crescimento lento. Acometem igualmente homens e mulheres de meia idade, comprometendo com maior frequência os dígitos das mãos. As características clínicas fundamentais para o diagnóstico são: faixa longitudinal amarelada de espessura variável, estilhaços hemorrágicos, estrias longitudinais associadas à hipercurvatura transversal e projeções digitiformes emergentes da matriz ungueal. Os autores relatam três casos desse tumor acometendo pododáctilos, enfatizando seus principais aspectos clínicos, achados dermatoscópicos e tratamento cirúrgico.

Onychomatricomas are rare benign tumors originating from the nail matrix and underlying stroma. They are usually asymptomatic and slow growing, affecting both middle-aged men and women, and more frequently involve the digits of the hands. Key clinical features for diagnosis are: yellowish longitudinal band of variable width, splinter hemorrhages, longitudinal grooves associated with the transverse overcurvature and fingerlike projections emerging from the nail matrix. The authors report three cases of this tumor affecting toes, emphasizing main clinical aspects, dermoscopic findings, and surgical treatment.

Hanseníase históide símile: desafio diagnóstico / Histoid like leprosy: diagnostic challenge

Em 1963, Wade descreveu a hanseníase histoide, que acometia pacientes previamente tratados com dapsona.Caracteriza-se por lesão com aspecto queloideano, na histopatologia vemos histiócitos fusiformes e um grande número de bacilos. Relata-se caso de hanseníase dimorfa virchoviana com padrão de lesão históide, um verdadeiro desafio diagnóstico, a fim de atentar para manifestações atípicas dessa doença e reforçar a importância da confirmação histológica de casos suspeitos de hanseníase históide.

In 1963, Wade described Histoid Leprosy, that affected patients previously treated with dapsone. This type of leprosy is characterized by keloid-like lesions, in which spindle histiocytes and a large number of bacilli can be seen in the histopathology. A case of borderline lepromatous leprosy with histoid pattern is described, a true diagnostic challenge, in order to attempt to atypical manifestations of the disease and reinforce the importance of histological confirmation in suspected cases of histoid leprosy.

Lesão vegetante em septo nasal como primeira manifestação clínica de hanseníase / Vegetative lesion in the nasal septum as the first clinical manifestation of Hansen´s disease

Apresentamos um caso de paciente com sintomas de obstrução nasal e coriza, tendo como primeira manifestação de hanseníase dimorfa virchoviana uma única lesão vegetante no septo nasal, caso excepcionalmente raro como primeira manifestação de doença. A forma dimorfa é instável e pode evoluir para virchoviana ou tuberculoide. A hanseníase virchoviana tem maior número de bacilos e é caracterizada por múltiplas máculas eritematosas, pápulas, nódulos e placas. As lesões são disseminadas e preferencialmente com distribuição simétrica...

Estudo do perfil epidemiológico e avaliação pós-cirúrgica de oito casos de tumor glômico / Study of epidemiological profile and post-surgical evaluation of 8 glomus tumor cases

Introdução: O tumor glômico é lesão benigna rara que ocorre mais comumente em falanges distais e representa de 1% a 4,5% das neoplasias da mão. O tipo subungueal é mais frequente em mulheres nas terceira e quinta décadas de vida. Objetivo: Este trabalho objetivou avaliar o perfil epidemiológico de pacientes com tumor glômico, sua satisfação em relação ao tratamento, e a recidiva por retirada incompleta. Método: Estudaram-se os prontuários de oito pacientes com diagnóstico de tumor glômico tratados em serviço de dermatologia de 1992 a 2011 avaliando-se sexo, idade, profissão, região acometida, satisfação do paciente em relação ao tratamento e se houve recidiva. Resultados: A média de idade dos casos foi de 63 anos, superior à faixa etária descrita pela literatura. A profissão mais relacionada foi "prendas domésticas". Todos os pacientes ficaram satisfeitos com o tratamento do ponto de vista estético. A melhora da dor foi plena em três dos seis pacientes que retornaram para avaliação. Naqueles em que a melhora não foi total, houve recidiva. A taxa de recidiva por retirada incompleta foi de 50%. Conclusão: Conclui-se a persistência da dor como indicadora de recidiva e percebe-se a efetividade da cirurgia na melhora da dor.

Introduction: Glomus tumors are rare benign lesions that are most often found on the tips of the fingers; they account for 1.0-4.5% of neoplasias on the hand. Subungual glomus tumors occur more frequently in women in their 30s and 50s. Objective: This study evaluated glomus tumor patients' epidemiological profiles, their satisfaction with the treatment and the rate of recurrence due to incomplete removal. Method: The records of patients that were treated for glomus tumors at a dermatology clinic between 1992 and 2011 were evaluated regarding gender, age, profession, affectedarea of the body, satisfaction with the treatment and possible recurrence. Results: The patients (n = 8, average 63 years) were older than those usually described in the literature. The most frequently described profession was that of housewife. All patients were satisfied with the aesthetic results of the treatment. There was a total improvement of the pain in threepatients, while patients with a partial improvement in pain experienced a recurrence. The recurrence rate due to incomplete removal was 50%. Conclusion: The surgery's effectiveness can be measured by the improvement of the pain; persistence of pain can indicate recurrence.

Epidermolytic hyperkeratosis: a follow-up of 23 years of use of systemic retinoids.

Epidermolytic hyperkeratosis is a form of ichthyosis normally resistant to topical treatments. Female patient monitored since 1978 diagnosed with epidermolytic hyperkeratosis. Clinical examination showed generalized hyperkeratosis and scaling. Given that no other treatments were available at the time, the patient was initially treated with keratolytic, systemic vitamin A and moisturizers, with no improvement. In 1986, with the development of oral retinoids, etretinate was introduced. In 1998 this was replaced by acitretin. The patient is receiving 25 mg/day after 23 years of using oral retinoids. Significant improvement of the condition and patient's quality of life has been noted.