No abstract

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

SYNDROME IN QUESTION CASE REPORTA 65 year-old man presented for evaluation of multiple widespread nodules in his body. The lesions were long standing and began during his childhood. More lesions appeared over time. On physical examination multiple, subcutaneous, soft, mobile and non painful nodules and tumors were observed in the arms, legs and abdomen distorting the affected areas (Figures 1, 2 and 3). His past medical history was remarkable for diabetes mellitus and hepatocarcinoma secondary to chronic HBV infection. He denied alcohol consumption. Similar but less extensive lesions were observed in his two brothers and father. Some lesions were surgically excised because they caused functional discomfort. The histopathological examination showed the presence of globules of mature white adipose tissue surrounded by thin fibrous capsules. The analytical study showed no significant abnormalities including lipid abnormalities. Based on the characteristic clinical history, family history and histopathology the diagnosis of familial multiple lipomatosis (FML) was made.

A criptococose é uma infecção que ocorre maioritariamente em indivíduos imunodeprimidos, afectando 2-10% dos doentes com SIDA. Nesta população, a fase inicial de infecção respiratória passa frequentemente desperce- bida, aumentando o risco de disseminação sistémica e envolvimento multiorgânico. O atingimento cutâneo secundá- rio ocorre geralmente nas fases terminais de infecções disseminadas e potencialmente letais. Os autores apresentam o caso de um homem de 37 anos, VIH positivo, com história de lesões cutâneas na face, tronco e membros superiores com uma semana de evolução, associadas a febre, tosse seca e cefaleias intensas. Os exames complementares de diagnóstico permitiram efectuar o diagnóstico de uma criptococose sitémica com envolvimento cutâneo secundário, tendo o doente efectuado tratamento com anfotericina B e fluconazol sistémicos, com resolução completa de todo o quadro clínico. A importância deste caso prende-se com o facto de as lesões cutâneas serem frequentemente a única manifestação de uma criptococose sistémica, o que aliado à sua clínica altamente inespecífica, enfatiza a importância de um elevado índice de suspeita clínica para o seu correcto diagnóstico e tratamento imediato. PALAVRAS-CHAVE – Criptococose; Imunossupressão; VIH.