Kagoura M, Toyoda M, Matsui C, Higaki S, Morohashi M
Department of Dermatology, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Toyama, Japan.
Pediatr Dermatol. 2001 Jan-Feb;18(1):13-6. doi: 10.1046/j.1525-1470.2001.018001013.x.
Lipodystrophia centrifugalis abdominalis infantilis (LCAI) in a 2.5-year-old Japanese girl is reported. Clinically she had a curved cutaneous depression with a slightly elevated erythematous border on the left abdomen beyond the left groin. The regional lymph node was palpable. Histologic examination showed a decrease of the fat, an inflammatory infiltrate mainly composed of lymphocytes, and septal fibrosis in the subcutaneous tissue. Immunohistochemical analysis found that infiltrating lymphocytes were mainly positive against CD4. Ultrastructurally nuclei in the adipocytes were crescent shaped and located toward the periphery of the cell. Some banded structures with a periodicity of 150 nm, and with intraperiodic bands, referred to as fibrous long-spacing collagen (FLSC), were observed in the septal area of the fatty tissue. These results indicated that FLSC was correlated with the breakdown of fibrillar collagens in LCAI.
报告了一名2.5岁日本女孩的婴儿离心性腹部脂肪营养不良（LCAI）。临床上，她左侧腹部超过左腹股沟处有一个弯曲的皮肤凹陷，边缘有轻微隆起的红斑。可触及区域淋巴结。组织学检查显示脂肪减少，皮下组织有以淋巴细胞为主的炎性浸润和间隔纤维化。免疫组织化学分析发现浸润淋巴细胞主要对CD4呈阳性。超微结构上，脂肪细胞核呈新月形，位于细胞周边。在脂肪组织的间隔区域观察到一些周期性为150 nm且有周期内带的带状结构，称为纤维状长间距胶原（FLSC）。这些结果表明FLSC与LCAI中纤维状胶原的分解有关。
