Acquired generalised lipodystrophy and type 1 diabetes mellitus in a child: a rare and implacable association

Abstract

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.

Keywords: diabetes; lipid disorders; metabolic disorders.

Conflict of interest statement

Competing interests: None declared.

© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.

Figures

Figure 1

Panels (A) and (B) showing facial appearance at age4 and 7 years. Panels (C)–(F) are the clinical photographs at 13 years of age showing front and lateral aspect, pseudohypertrophy of all muscles, phlebomegaly with generalised loss of subcutaneous fat.

Figure 2

(A) CT axial image showing sheet like hypodense soft tissue in the retroperitonium encasing the aorta, inferior vena cava and bilateral renal vessels (white arrow). (B) MR axial image showing the hyperintense soft tissue in the retroperitoneum.