Steatocystoma multiplex is a rare genetic disorder characterized by the presence of hamartomatous malformations at the junction of the pilosebaceous duct. It consists of encapsulated cystic lesions in the dermis, with adjacent sebaceous gland. When associated with inflammation, resembling hidradenitis, it is called steatocystoma multiplex suppurativa, a condition rarely reported. This is the first case of steatocystoma multiplex suppurativa reported in the Brazilian literature. Female patient, 23 years old, with papular and nodular cystic lesions that started in the armpits and groin, later spreading to the trunk, lower limbs, anticubital fossa, face and scalp. The presence of papular-nodular lesions associated with disseminated hidradenitis-like lesions in flexural areas and the histopathological diagnosis of steatocystoma defined the diagnosis of steatocystoma multiplex suppurativa.

Dermatofibroma is a benign fibrohistiocytic tumor, common and easily diagnosed when classical clinicopathologic features are present. The atrophic variant of dermatofibroma is of uncertain origin. This lesion is characterized clinically by a flat or atrophic and depressible surface. Histopathological features show reduction of the thickness of the dermis and elastic fibers. We report a typical case of this uncommon and probably underdiagnosed variant.

Blue nevi are benign melanocytic lesions located in the deeper reticular dermis, consequence of failure of melanocytic migration into the dermal-epidermal junction from the neural crest. Lesions are usually asymptomatic and solitary, but may present in a multiple or agminated (grouped) pattern. The agminated subtype is formed when bluish-pigmented lesions cluster together in a well-defined area. Lesions can be flat or raised. We report the case of a patient who presented multiple bluish macules (1-3 mm in diameter) grouped on the left upper back. Dermoscopy and anatomic pathological examination were consistent with blue nevus.

Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Granulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

A hidradenite supurativa é doença inflamatória de difícil tratamento. Recentemente, a terapia anti-TNF alfa, com anticorpos monoclonais ("terapia biológica"), tem sido apontada como uma alternativa. No entanto, ensaios clínicos avaliando a eficácia destas drogas na hidradenite supurativa, ainda não foram publicados. Apresentamos um caso no qual o uso do infliximabe não resultou em melhora clínica expressiva.

Acrometastasis is a rare occurrence, especially when affecting the hands. It represents around 0.007-0.2% of all metastatic lesions. The most common site of origin is the lung, accounting for 40-50% of all cases reported in the literature. Kidneys and breasts are other sites also associated with neoplastic lesions that disseminate to the hands. More rarely, the site of origin may be the gastrointestinal tract or other systemic tumors or sarcomas. Early diagnosis is difficult, since the condition may be asymptomatic or may mimic tenosynovitis, arthritis, paronychia, pyogenic granuloma or a local infection. In the present paper, the authors report on a patient with the diagnosis of acrometastasis on both hands originating from a basaloid carcinoma of the anal canal. Response to radiotherapy was poor. Keywords: Colonic neoplasms; Granuloma, pyogenic; Neoplasm metastasisResumo: As acrometástases, principalmente para as mãos, são incomuns e representam cerca de 0,007-0,2% de todas as lesões metastáticas. O pulmão é o sítio de origem mais comum, colaborando com 40-50% dos casos relatados na literatura. Os rins e mamas são outras localizações também relacionadas a neoplasias que metastatizam para as mãos, além de, mais raramente, trato gastrointestinal, outros tumores sistêmicos e sarcomas. Seu diagnóstico precoce é difícil, pois pode ser assintomático, se assemelhar a tenossinovite, artrite, paroníquia, granuloma piogênico ou infecção local. No presente relato, os autores apresentam paciente com diagnóstico de acrometástase, em ambos os quartos quirodáctilos, oriunda de carcinoma basaloide de canal anal, com pobre resposta à radioterapia.

A hanseníase é uma doença infectocontagiosa, com período de incubação médio de dois a cinco anos, causada pelo Mycobacterium leprae, o qual possui tropismo para a pele, as mucosas e os nervos periféricos. Quando manifestada em crianças abaixo de quinze anos, reflete a intensidade e longo período de exposição à grande carga bacilar. Representa, então, um importante evento de alerta que aponta para uma dificuldade no controle da doença. Os autores relatam três casos de hanseníase, em menores de quinze anos, provenientes do Município de Itaguaí, Rio de Janeiro. Discutem-se as implicações epidemiológicas da detecção de novos casos nessa faixa etária e o papel fundamental do exame de contatos e da busca do caso fonte no controle da Hanseníase.