Diabetes with partial lipodystrophy following sclerodermatous chronic graft vs. host disease

The importance of adipose tissue in metabolism, as a target for insulin action and a secretor of metabolic regulatory proteins, is increasingly recognized. Lipodystrophic conditions are often associated with significant insulin resistance. The commonest acquired form occurs with highly active antiretroviral therapy (HAART) for human immunodeficiency virus infection. Other medical conditions and drugs also have the potential to cause chronic subcutaneous fat damage.

We describe an unfamiliar partial lipodystrophy in a young woman, associated with markedly insulin-resistant diabetes, acquired following allogeneic bone marrow transplantation for childhood leukaemia complicated by late sclerodermatous chronic graft vs. host disease (GVHD). Clinical examination revealed scarring and lipodystrophy affecting mainly legs, thighs, buttocks and forearms but sparing her face, neck and thorax. Her serum adiponectin level was markedly reduced.

However, although thiazolidinediones lower insulin resistance and increase subcutaneous peripheral fat in Type 2 diabetes, pioglitazone treatment had little effect on either serum adiponectin, glycaemic control or the lipoatrophy. In this case, effective glycaemic control was best achieved using a combination of metformin and highly concentrated soluble insulin injections.