Barraquer-Simons lipodystrophy, Raynaud's phenomenon and cutaneous vasculitis

We report a 32-year-old man who presented with a 2-year history of unexplai ned weight loss and symptoms of Raynaud's phenomenon. Clinical examination and investigations were consistent with a diagnosis of acquired partial lip odystrophy (Barraquer-Simons disease). This patient also exhibited an assoc iated cutaneous vasculitis and peripheral perniotic changes. He had normal renal function and glucose tolerance and no immunological abnormality has b een detected in his serum to date. The absence of C3-nephritic factor in th e presence of overt lipodystrophy suggests that there may be another factor or immunological mediator responsible for the subcutaneous changes seen in patients with lipodystrophy.