Autosomal dominant familial angiolipomatosis clinically mimicking neurofibromatosis.

Abstract

The autosomal dominant form of familial angiolipomatosis may be mistaken for peripheral neurofibromatosis (NF-1) due to the similarity of the family history and the occurrence of multiple subcutaneous masses, but histopathological examination of the tumors readily distinguishes these two diseases. We report here a case of familial angiolipomatosis, which was initially though to be neurofibromatosis, and the occurrence in this patient of a granular cell tumor similar to such tumors occasionally seen in neurofibromatosis. A review of the literature discloses intriguing parallels between familial angiolipomatosis and neurofibromatosis, suggesting that similar pathogenetic mechanisms may operate in both diseases.