腹部远心性脂肪营养不良18例分析

Clinical analysis of 18 patients with lipodystrophia centrifugalis abdominalis

目的探讨腹部远心性脂肪营养不良的临床和病理特点。方法回顾性分析18例腹部远心性脂肪营养不良患者的临床和组织病理资料。结果18例患者，女12例，男6例；发病年龄3～19岁，平均年龄6．8岁；病程1—3年。皮损多数为质软的萎缩斑，可见其下静脉，其中有1例萎缩斑周围有红斑。12例皮疹累及下腹部，11例累及腹股沟，8例累及外阴，2例累及胸部，2例皮损局限于颈部，1例累及腋窝。所有患者的皮损均无明显自觉症状。18例患者萎缩斑处的组织病理均符合腹部远心性脂肪营养不良的诊断。给予口服维生素E和白芍总苷胶囊，外用多磺酸黏多糖乳膏、0．03％他克莫司和吡美莫司软膏等治疗，但无明显疗效。结论腹部远心性脂肪营养不良的诊断主要依靠临床和病理检查。

Objective To study the clinical and pathological features of lipodystrophia centrifugalis abdominalis （LCA）. Methods The clinical and histopathological findings from 18 patients with LCA were analyzed retrospectively. Results Of the 18 patients, 12 were girls and 6 were boys, with the mean age at onset being 6.8 years （range, 3-19 years）. The most common lesions were soft and atrophic patches with visible veins below them. Erythema was observed around the atrophic patches in one patient. The lesions were mainly located in the lower abdomen （12 patients）, followed by the inguinal region （11 patients）, vulva （8 patients）, chest （2 patients）, neck （2 patients）, and axillary fossa （1 patient）. Histopathologic findings in these lesions were consistent with LCA. A variety of treatments were given, including systemic therapies such as vitamin E and total glucosides of paeony capsulesm, as well as topical therapies such as mucopolysaccharide polysulfate cream, tacrolimus 0.03% ointment, pimecrolimus cream, and so on, whereas no apparent improvement was observed. Conclusion The diagnosis of LCA is mainly based on clinical and pathological findings.