An unusual case of an acquired acral partial lipodystrophy (Barraquer-Simons syndrome) in a patient with extrinsic allergic alveolitis

Abstract

Conflicts of interest: none declared. Lipodystrophies are a heterogeneous group of rare disorders characterized by the loss of subcutaneous fat of part or all of the body. Lipodystrophies can be inherited or acquired. Acquired forms of lipodystrophy can be generalized, partial, localized or associated with human immunodeficiency virus (HIV). We report the case of an acquired partial lipodystrophy in an unusual acral distribution associated with extrinsic allergic alveolitis. A 54‐year‐old woman presented with a 4‐year history of wasting of the subcutaneous fat of her arms, pelvic girdle and thighs. Her abdomen and face were spared. Loss of fat resulted in marked redundancy of the overlying skin and a weight loss of approximately 5 kg, but was not associated with dermopathies. A striking step was evident at the margin of the affected and unaffected skin at the arms and thighs (Fig. 1a,b). There was no fat accumulation in other parts of the body. A photograph taken on a holiday a year before the onset of the acral lipodystrophy shows subtle semicircular lipoatrophy of the thighs, at the time unnoticed by the patient (Fig. 2). She declined a magnetic resonance imaging (MRI) scan to evaluate the extent of her lipodystrophy. A year after the onset of the lipodystrophy, she was also diagnosed with extrinsic allergic alveolitis. She was commenced on prednisolone and azathioprine, on which her pulmonary condition improved significantly. Her lipodystrophy remains unchanged.