腹部离心性脂肪营养不良的临床和病理特点

刘洁; 周涛; 方凯; 马东来; 刘跃华

doi:10.3969/j.issn.1674-9081.2012.04.014

腹部离心性脂肪营养不良的临床和病理特点

Clinical and Pathological Characteristics of Patients with Lipodystrophia Centifugalis Abdominalis

摘要

摘要:
目的探讨腹部离心性脂肪营养不良(lipodystrophia centrifugalis abdominalis, LCA)的临床和病理特点。
方法回顾性分析2009年7月至2012年7月间北京协和医院皮肤科确诊的5例LCA患者的临床和组织病理资料, 总结其特点。
结果 5例患者平均发病年龄7.2岁(4~13岁), 病程1~3年; 皮损表现为境界清楚的腹部萎缩性斑片, 皮下血管清晰可见; 患者均无自觉症状; 皮损组织病理可见皮下脂肪消失或显著减少; 治疗以口服活血化瘀中药及维生素E为主。
结论 LCA临床较少见, 具有典型的临床和病理特征, 其诊断通常不难, 但目前尚无有效的治疗方案。

Abstract:
Objective To study the clinical and histopathological features of lipodystrophia centrifugalis abdominalis (LCA).
Methods The clinical and histopathological data of 5 patients with LCA diagnosed at Peking Union Medical College Hospital form July 2009 to July 2012 were analyzed retrospectively.
Results The mean age of onset was 7.2 years (4-13 years). The disease courses ranged from 1 to 3 years. In most cases, the asymptomatic, circumscribed skin atrophy involved the abdomen and inguinal fold. The underlying blood vessels were visible. Histological examination revealed decrease or loss of subcutaneous fat in the atrophic area. The patients were treated with oral traditional Chinese medicine (for promoting blood circulation) and vitamin E.
Conclusions LCA is a rare lipodystrophic disease. The diagnosis of LCA is mainly based on clinical findings and pathological features. Unfortunately, no effective treatment for this disease has been available.

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