Lipodystrophia centrifugalis abdominalis in Korea

Lipodystrophia centrifugalis abdominalis infantilis is a rare disorder described exclusively in Asians. Thirty Korean patients (4 women, 20 girls, and 6 boys) with a clinical and/or histopathologic diagnosis of this disorder were evaluated. The mean age of onset was 6.2 years. Infantile or congenital onset was seen in only three girls and two boys. In 50% of females, onset was after 5 years of age. All patients had involvement of the lower abdomen and groin both as the site of onset and as the main site. Histopathologic analysis showed loss of fat with or without an infiltrate of lymphocytes and histiocytes in the subcutis. Only one patient responded to treatment, an adult female who received photochemotherapy with softening of the lesions and symptomatic improvement. In previous reports, the age of onset of this disorder was at an age younger than 5 years but older than infants, making the term lipodystrophia centrifugalis abdominalis juvenilis more appropriate. In our series, 50% of females had onset after 5 years of age and 12.5% after 18 years of age. In one woman, the lesion that developed at 4 years of age progressed until adulthood. Our study of affected Korean patients showed a fourfold female predilection and later onset in female patients.