We sought to describe the clinical characteristics and complications of children with acquired lipodystrophy (LD).

We conducted a retrospective chart review at a tertiary, academic children’s hospital of children clinically given a diagnosis of acquired LD between January 1997 and December 2004.

During the study period, 23 patients were identified. Their mean age at diagnosis was 9.74 ± 3.98 years. Of patients, 61% were girls. The length of the follow-up was 4.8 ± 3.5 years from the time of LD diagnosis. Of patients, 30% had evidence of localized disease (7 of 23), 26% (6 of 23) had localized partial disease, and 44% (10 of 23) had generalized LD. The most common underlying diagnosis was dermatomyositis (78%), alone or in association with other autoimmune diseases (juvenile rheumatoid arthritis 17%). Panniculitis with autoimmunity was noted in 17% of the patients. More than half of the patients had at least one complication attributable to LD such as acanthosis nigricans (22%), hyperpigmentation (22%), hepatomegaly (13%), hypertension (13%), protuberant abdomen (9%), and hyperlipidemia (4%). The only predictor for development of complications was the age of diagnosis of LD, with younger age being associated with increased risk (7 vs 12 years, P = .04).

Limitations were inherent to the retrospective design.

Childhood acquired LD is seen more frequently in the context of autoimmunity. Affected children should be monitored for the development of complications, particularly if given a diagnosis of LD at a younger age.