Cútis laxa granulomatosa: relato de caso / Granulomatous slack skin: a case report

RESUMO

A cútis laxa granulomatosa é variante de linfoma T cutâneo com características histopatológicas semelhantes às da micose fungóide, além da presença de infiltrado granulomatoso e perda de fibras elásticas. Caracteriza-se clinicamente por placas endurecidas, eritêmato-vinhosas, que evoluem para flacidez cutânea, acometendo preferencialmente as regiões axilar e inguinal. Em mais da metade dos casos há associação com linfoma de Hodgkin. Relata-se o caso de paciente de 47 anos com cútis laxa granulomatosa, com manifestação sistêmica, que respondeu parcialmente à terapia com interferon-alfa e corticosteróides.

ABSTRACT

Granulomatous slack skin is a cutaneous T cell lymphoma variant with histopathologic findings similar to those of mycosis fungoides, in addition to granulomatous infiltrate and loss of dermal elastic tissue. Clinically, it begins with indurated purplish plaques that evolve into areas of hanging bulky skin masses, preferentially affecting axillary and inguinal folds. In more than half of cases there is an association with Hodgkin’s disease. Here, we report the case of a 47-year-old woman with granulomatous cutis laxa and systemic manifestations that partially responded to therapy with interferon alpha and steroids.