title: [Progressive lipodystrophy (Barraquer-Simon syndrome): differential diagnosis and clinical aspects].
abstract: "Progressive lipodystrophy" or "partial lipodystrophy of the cephalothoracic type" is a rare, acquired condition of unknown aetiology with onset in childhood and a complete loss of subcutaneous fat of face, neck, trunk and upper extremities. The disease is more common in females than males and causes a disfigurement of the face; that it cannot be regarded only as a harmless variation, is above all due to a typical concomitant disease, membranoproliferative glomerulonephritis with hypocomplementaemia. Laboratory investigations nearly always reveal a complement-activation by the "alternative pathway" with consumption and lack of complement-component C3, a finding which allows a clear distinction between partial lipodystrophy and congenital or acquired forms of total lipodystrophy ("Berardinelli-Seip-Syndrom", lipoatrophic diabetes) and other circumscript lipodystrophies.
mesh_terms: D000273:Adipose Tissue / Q000473:pathology; D000328:Adult; D001706:Biopsy; D003937:Diagnosis, Differential; D004576:Electromyography; D005260:Female; D006801:Humans; D008060:Lipodystrophy / Q000175:diagnosis* / Q000473:pathology; D009136:Muscular Dystrophies / Q000175:diagnosis; D012867:Skin / Q000473:pathology
vernacular_title: "Lipodystrophia progressiva" (Barraquer-Simon-Syndrom): Differentialdiagnose und Klinik.