Abstract Stewart-Treves syndrome is a rare cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Though most commonly this angiosarcoma is a result of post mastectomy lymphoedema, it also develops in Milroy disease, idiopathic, congenital, traumatic and filarial lymphoedema. Despite the rarity of this syndrome and its poor prognosis, early diagnosis associated with radical surgery can provide improved survival. We report a case of angiosarcoma in the lower limb in a patient with chronic lymphedema associated with history of repeated erysipela episodes.

O acantoma de células claras ou acantoma de Degos é uma doença bem individualizada quanto aos seus aspectos clínicos, histopatológicos e imuno-histoquímicos. Sua natureza patológica - se neoplásica ou reativa - ainda é debatida por pesquisadores. De evolução crônica, ocorre predominantemente nos membros inferiores de adultos, em geral como lesão papulonodular única. Entretanto, foram observados casos com lesões múltiplas, por vezes de caráter eruptivo, e com notável variação de tamanho e forma das eflorescências cutâneas. Há relatos de cinco casos com localização exclusiva na aréola e no mamilo, quatro simulando eczema e um como nódulo polipoide; todos ocorridos em mulheres coreanas. O objetivo deste trabalho é apresentar os aspectos clínicos, histopatológicos e imuno-histoquímicos de dois novos casos da doença, com idêntica topografia areolomamilar, observados em mulheres brasileiras.

Clear cell acanthoma or Degos' acanthoma is a distinct disease concerning its clinical, histopathological, and immunohistochemical features. Its pathologic nature - whether neoplastic or reactive - is still under dispute among researchers. The disease shows a chronic course and often presents with a single papulonodular lesion on the lower limbs of adults. However, cases with multiple lesions, sometimes occurring in an eruptive fashion, and with clear variation in the size and shape of the cutaneous lesions have been reported. So far, five cases in which the lesions were exclusively located in the nipple area have been reported, all in Korean women. Four of these cases mimicked eczema and one, a polypoid nodule. The aim of this article is to present clinical, histopathological, and immunohistochemical features of two additional cases in Brazilian women with similar nipple topography.