摘要
目的分析6例致残性全硬化性硬斑病的临床及病理学特点。方法回顾分析2007--2017年中国医学科学院皮肤病医院病理科经临床及组织病理学确诊的6例致残性全硬化性硬斑病患者的临床、病理表现及随访情况。结果6例患者中男4例，女2例；发病年龄3-10岁，平均6．5岁；发病至确诊时间2～10年，平均6．2年。病变部位均出现皮肤萎缩、变薄、紧绷，肢体变细，合并肌肉萎缩，表面可见深部粗大静脉显露；4例出现临近关节的挛缩、畸形及功能障碍；2例出现下肢明显短缩。外周血检查均未见嗜酸性粒细胞升高及高1免疫球蛋白血症。影像学检查：骨皮质光整，骨小梁清晰，未见骨质异常。下肢挛缩皮损组织病理：表皮萎缩变薄，基底层色素增加，真皮中下部、皮下脂肪区域及深部组织胶原纤维明显增生、粗大、硬化，部分均质化改变。结论致残性全硬化性硬斑病常无内脏受累，但往往出现四肢深在性受累，病理上主要表现为真皮及皮下组织胶原纤维明显增生、硬化。
Objective To investigate clinical and pathological features of 6 cases of disabling pansclerotic morphea （DPM）. Methods Clinical and pathological manifestations of and follow-up results in 6 patients, who were clinically and histopathologically diagnosed with DPM in the Department of Pathology, Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College from 2007 to 2017, were retrospectively analyzed. Results Among the 6 patients, 4 were male and 2 were female. The age of onset ranged from 3 to 10 years, with an average age of 6.5 years. The average duration from the occurrence to the confirmation of the diagnosis was 6.2 years （range, 2 - 10 years）. At all the lesional sites, skin atrophy, thining and tightness occurred, and the limbs became thin. Additionally, there were muscular atrophy and visible deep thick veins on the surface of the limbs. The contracture, deformity and dysfunction of the adjacent joints occurred in 4 cases, and the lower limbs were obviously shortened in 2 cases. Peripheral blood examination showed no increase of eosinophils or hypergammaglobulinemia. Imaging examination revealed smooth cortical bone and clear trabecular bone, and no osseous abnormality was observed. Histopathological examination of contracted skin lesions of the lower limbs revealed atrophic and thinned epidermis, hyperpigmentation in the basal layer, hyperplastic, thickened, hardened and partly homogenized collagen fibers in the middle to deep dermis, subcutaneous adipose tissue region and deep tissue of the skin. Conclusions DPM usually does not affect viscera, but often involves deep tissue of the limbs. Histopathologically, DPM is mainly characterized by obviously hyperplastic and hardened collagen fibers in the dermis and subcutaneous tissue.
作者
张韡
陈浩
徐秀莲
姜祎群
曾学思
孙建方
Zhang Wei;Chen Hao;Xu Xiulian;Jiang Yiqun;Zeng Xuesi;Sun Jianfang(Department of Pathology,Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College,Nanjing 210042,China)
出处
《中华皮肤科杂志》
CAS
CSCD
北大核心
2018年第8期561-563,共3页
Chinese Journal of Dermatology
基金
中国医学科学院医学与健康科技创新工程项目（CIFMS-2017-12M-1-107）
关键词
硬皮病
局部性
儿童
皮肤表现
病理过程
治疗
致残性全硬化性硬斑病
Scleroderma
localized
Child
Skin manifestations
Pathologic processes
Therapy
Disabling pansclerotic morphea
