소아 복벽원심성 지방위축증 1례

Abstract

Lipodystrophia centrifugalis abdominalis infantilis(LCAI), an atypical form of progressive lipodystrophy, is a disease named by Imamura et al in 1971. The unique and distinct features of this disease are onset by age 3, development of the lesion in the groin, axillae, or their surroundings, presence of slightly inflammatory areas around the depressed one, and histologically, it shows loss of subcutaneous fat in the depressed area with panniculitis in the surrounding area. The depressed lesions are found to enlarge centrifugally for several years and to involve a large part of the abdominal and/ or chest wall. However the enlargement is found to stop spontaneously by age 13, and the depressed area tends to undergo recovery thereafter. The patient is 9-year-old girl. She had multiple well-defined depressions of skin on abdomen, left inguinal area with deformity of labia major which was noticed 4 years prior to first diagnosis. There was no family history, no physical abnormality except skin lesion. Complete blood cell count, urinalysis, liver function test and chest X-ray within normal limit. Biopsy specimen from surrounding erythematous area revealed slightly atrophy of the epidermis, a moderate lymphocyte infiltration around skin appendages in mid-part of dermis, decreased amount of subcutaneous adipose tissue with mild lymphocyte infiltration. She has been treated with topical steroid. But there was no improvement of the depressed lesion except surrounding erythema.