Abstract

Lipodystrophies are a group of rare diseases characterized by selective loss of adipose tissue. Lipodystrophies have been classified on the basis of whether the disease is familial or acquired, or whether the adipose tissue loss is generalized, partial or localized. Acquired partial lipodystrophy, also known as Barraquer-Simons syndrome or cephalothoracic lipodystrophy, is a rare form of lipodystrophy, of which etiopathogenesis has been closely linked with autoimmunity. Acquired partial lipodystrophy is typically characterized by symmetrical loss of fat from face, neck, upper extremities and trunk with spared or increased adiposity in the lower body. There is a growing body of evidence which suggests that autoimmunity plays a role in the etiopathogenesis of acquired partial lipodystrophy. Here, we report a very rare case of acquired partial lipodystrophy accompanied by autoimmune thyroiditis. Thus, we highlight the rarity of the condition and review the available literature on the subject.

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