Partial lipodystrophy associated with a type 3 form of membranoproliferative glomerulonephritis

Facial Lipoatrophy Following Systemic Lupus Erythematosus

“…Some authors suggest that viral factors, environmental factors, or genetic factors may trigger an immunologic process. 8 In vitro studies suggest that C3NeF causes lysis of adipose tissue expressing factor D. 20 The pathogenesis of facial lipoatrophy in our patient is not related to the presence of C3NeF. The chronology of events is intriguing: The facial lipoatrophy immediately followed the disappearance of lupus lesions.…”

“…9 A benign, febrile, childhood illness, particularly measles, may precede the beginning of this syndrome. 3,8 Women are affected three times as often as men. This syndrome has been associated with insulin resistance, diabetes, dyslipidemia, acanthosis nigricans, hirsutism, and sometimes cirrhosis.…”

“…Mesangiocapillary glomerulonephritis has also been associated with the presence of C3 nephritic factor, with or without acquired partial lipoatrophy. 4,8 We describe a woman with systemic lupus erythematosus who developed lipoatrophy limited to the face, without evidence of C3NeF serum's antibody. This is the first reported case of partial lipodystrophy appearing after the diagnosis of systemic lupus erythematosus.…”

Partial lipodystrophy presenting with myopathy

“…Our patient went on to develop epilepsy in early childhood, but it was only when she developed more typical features of PLD several years later, including the lipoatrophy itself, that the diagnosis became apparent. Her subsequent clinical history is somewhat more typical of the Barraquer-Simon syndrome, with fatty liver, hyperlipidaemia, hyperinsulinaemia, and glucose intolerance being well described (Senior and Gellis 1964, Piscatelli et al 1970, Havel 1972, Boucher et al 1973, Chartier et al 1987, Robertson and Wright 1989, Bourke and Powell 1992, Kavanagh et al 1993. She has clinical and biochemical findings in keeping with polycystic ovarian syndrome, although multiple cysts were not seen on ultrasound at 15 years of age.…”

“…Abnormalities of the complement system, particularly the alternate pathway, have been found in PLD and may precede the development of glomerulonephritis (Sissons et al 1976, 130 Chartier et al 1987, O'Mahoney et al 1990, Power et al 1990, Mathieson et al 1993, Walport et al 1994. Patients with PLD may have increased susceptibility to infection (Piscatelli et al 1970b).…”