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

Abstract: 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 Corresponding author: Sun Jianfang, Email: fangmin5758@aliyun.com 【Abstract】 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.

Key words: Scleroderma, localized, Child, Skin manifestations, Pathologic processes, Therapy, Disabling pansclerotic morphea