Juvenile dermatomyositis with lipodystrophy (Q92505)

Juvenile dermatomyositis with lipodystrophy is a chronic systemic autoimmune disease characterised by early-onset proximal weakness and characteristic skin rashes, associated with loss of subcutaneous fat in a localised or generalised distribution, frequently with resultant metabolic abnormalities such as insulin resistance, diabetes and hyperlipidaemia. These patients have a loss of mature, functional adipocytes, as opposed to an absence of lipid in otherwise normal adipocytes.