An ultrastructural study of lipodystrophia centrifugalis abdominalis infantilis, with special reference to fibrous long-spacing collagen

Lipodystrophia centrifugalis abdominalis infantilis (LCAI) in a 2.5-year-ol d Japanese girl is reported, Clinically she had a curved cutaneous depressi on with a slightly elevated erythematous border on the left abdomen beyond the left groin. The regional lymph node was palpable. Histologic examinatio n showed a decrease of the fat, an inflammatory infiltrate mainly composed of lymphocytes, and septal fibrosis in the subcutaneous tissue, Immunohisto chemical analysis found that infiltrating lymphocytes were mainly positive against CD4, Ultrastructurally nuclei in the adipocytes were crescent shape d and located toward the periphery of the cell, Some banded structures with a periodicity of 150 nm, and with intraperiodic bands, referred to as fibr ous long-spacing collagen (FLSC), were observed in the septal area of the f atty tissue, These results indicated that FLSC was correlated with the brea kdown of fibrillar collagens in LCAI.