Acquired Generalised Lipodystrophy Lipoatrophic Diabetes or Lawrence Syndrome

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Acquired generalised lipodystrophy (AGLD) is a rare, juvenile-onset lipodystrophy, first fully described by Lawrence in 1946 [1], who reported on a young female subject with 'lipodystrophy, and hepatomegaly with diabetes, lipaemia and other metabolic disturbances.' To date, approximately 80 patients with AGLD have been reported [2]. Like others LDs, AGLD is prevalent in females. Lipoatrophy develops over a number of years, in childhood or in adolescence, so that the onset of the condition is later than that of congenital generalised lipodystrophy (CGLD). Extended areas of subcutaneous fat are involved, including the face, arms, and legs. Less frequently mesenteric, retroperitoneal, perirenal and mediastinal fat depots are involved, while retroorbital fat seems to be spared. Muscle mass, evaluated by dual energy X-ray analysis (DEXA), is preserved or even increased compared to age-, sex- and body mass index (BMI)-matched subjects. Therefore, in spite of the generalised atrophy of fat tissue the BMI in the majority of lipodystrophic patients falls into the low-normal range. Resting energy expenditure, independent of hyperthyroidism, is increased. This syndrome is associated with hyperinsulinaemia, insulin resistance, and acan-thosis nigricans, soon resulting in overt, non-ketotic diabetes mellitus. Hyperlipidaemia, namely hypertriglyceridaemia, is a further metabolic abnormality associated with AGLD. Liver steato-sis, autoimmune hepatitis, splenomegaly, and, ultimately, liver cirrhosis occur in some 20% of patients, often beginning in childhood [3, 4]. Other autoimmune diseases, such as Sjogren's

syndrome and dermatomyositis, have been reported in association with AGLD, suggesting an immunomediated fat loss as a possible pathogenetic basis [3]. Panniculitis, characterised by a granulomatous infiltration of adipose tissue, may be an early manifestation of the disease [5]. Acute viral infections often precede AGLD onset. A higher frequency than casually expected of astrocy-tomas of the third ventricle have been reported. Low levels of leptin and adiponectin have also been described in these patients [6].

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