Summary

Lipodystrophy syndromes are frequently associated with marked degree of insulin resistance and lipoatrophic diabetes. Although acquired generalised lipodystrophy (AGL) has been known to be associated with various autoimmune disorders, type 1 diabetes mellitus (T1DM) is very rarely reported to occur with AGL. Combination of AGL and T1DM can lead to a totally different phenotype with very difficult-to-treat diabetes and progressive complications of both the conditions. We report a case of AGL with T1DM with poor diabetes control despite high doses of insulin, metformin and pioglitazone. Our case further progressed to develop complication of retroperitoneal fibrosis, not hitherto reported with AGL.