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A papulose linfomatóide (PL) constitui uma variante rara dos linfomas cutâneos de células T com a presença de amplo infiltrado intracutâneo de células T positivas para CD30. O exame histológico sugestivo de doença altamente maligna e agressiva opõe-se ao curso crônico-recidivante, muitas vezes auto-limitado, presente na maioria dos casos. São apresentados os relatos de dois pacientes. Uma mulher com 64 anos de idade e história de 10 anos de PL recebeu terapia Puva em creme, sendo a doença controlada. No segundo caso, uma mulher de 42 anos apresentava história de 18 anos de PL tratada com Puva e, posteriormente, com fotoquimioterapia extracorpórea (FEC). Durante a FEC, observou-se rápida disseminação metastática que não pôde ser controlada por poliquimioterapia. A paciente foi a óbito após um ano, em razão de metástase no sistema nervoso central.Lymphomatoid papulosis (LP) is a rare variant of cutaneous T-cell lymphomas with large CD30-positive intracutaneous T-cell infiltrate. There is a discrepancy between histology suggesting a highly malignant and aggressive disease and the chronic relapsing mostly self limiting course in most cases. We report two patients. A 64-year-old woman with a 10-year history of LP was treated with cream PUVA that was able to control the disease. The other case, a 42-year-old woman had an 18-year history of LP treated by PUVA and later by extracorporeal photochemotherapy (ECP). During ECP a rapid metastatic spread developed that could not be controlled by polychemotherapy. Eventually she died due to a central nervous system metastasis one year later.

A 48-year-old female with an atypical plaque-like lesion of the lower leg is presented in this article. Histologic investigation revealed a rare low-grade fibromyxoid sarcoma (pT1a cN0 cM0; stage Ia) of suprafascial localization. Staging of the patient did not reveal metastatic spread. The tumor was surgically removed with wide safety margins. The defect was closed using a mesh graft transplant and vacuum-assisted closure. Healing was complete. Regular follow-up for at least 5 years is recommended. Besides the rareness of this tumor, this case is also remarkable because of the localization on the lower leg and the suprafascial soft tissue.