[Barraquer and Simons lipodystrophy. Complement anomalies and cutaneous leukocytoclasic vasculitis].
In a 56-year old woman progressive partial lipodystrophy began at the age of 6 years on the face, thereafter extending slowly down to mid-thigh level (fig. 1 and 2), with moderate hypertrophy of the subjacent fatty tissue and a fatty macroglossia (fig. 3). Histological examination of the lipodystrophic skin not only showed an absence of fatty tissue, but also abnormalities at the dermis-epidermis junction with hyaline bodies (fig. 4). At the age of 23 she developed purpura, predominantly on the legs, which rapidly became chronic (fig. 5); histological examination showed leucocytoclasic vasculitis of dermal vessels (fig. 6) with granular deposits of C3 on the vessels and of IgM at the dermis-epidermis junction. Episodes of polyarthralgia and headaches were frequent. Regressive neuritis of the external popliteal nerve occurred when she was 53-year old. Renal function tests proved normal, but renal biopsy was not performed. There was no diabetes mellitus, but an oral glucose tolerance test and a somatostatin insulin glucose test elicited definite resistance to insulin. A search for a serum factor inhibiting insulin receptors was negative. Permanent abnormalities in serum were a very deep fall in C3, a pronounced fall in CH50 and a low C4 level. Besides, a C3 nephritic factor (NeF) at a high level and circulating immune complexes were present (table I); a mixed IgM-IgG cryoglobulin was found intermittently (fig. 7). Clearance of the immune complexes by splenic macrophages was extremely slow. During a series of plasma exchanges, serum C3 increased transiently, whereas serum C4 remained unchanged (fig. 8).(ABSTRACT TRUNCATED AT 250 WORDS)
Perrot
H
H
Clinique Dermatologique, Hôpital Edouard-Herriot, Lyon.
Delaup
J P
JP
Chouvet
B
B
D000936:Antigen-Antibody Complex / Q000378:metabolism; D001706:Biopsy; D003176:Complement C3 / Q000172:deficiency*; D003178:Complement C3 Nephritic Factor / Q000097:blood*; D003181:Complement C4 / Q000172:deficiency* / Q000235:genetics; D003169:Complement Inactivator Proteins / Q000097:blood*; D005260:Female; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications; D008060:Lipodystrophy / Q000150:complications* / Q000276:immunology / Q000473:pathology; D008875:Middle Aged; D011693:Purpura / Q000150:complications* / Q000276:immunology / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000276:immunology / Q000473:pathology
D000936:Antigen-Antibody Complex; D003176:Complement C3; D003178:Complement C3 Nephritic Factor; D003181:Complement C4; D003169:Complement Inactivator Proteins
Lipodystrophie de Barraquer et Simons. Anomalies complémentaires et vasculite leucocytoclasique cutanée.