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[儿童致残性全硬化性硬斑病]

[Incapacitating pansclerotic morphea in childhood].

作者信息

Ferrandiz C, Henkes J, González J, Peyri J

出版信息

Med Cutan Ibero Lat Am. 1981;9(5):377-82.

PMID:7038353
Abstract

An 8-year old boy with generalized morphea involving all levels of the skin and soft tissues with disabling course, policlonal elevation of gammaglobulins and peripheral eosinophilia is examined. Neither Raynaud's disease nor sings of systemic scleroderma were present. The biopsy specimen showed thickening and hyalinization of collagenous tissue with moderate lymphocytic and plasma cell infiltration, mainly in the subcutaneous region and fascia. A diagnosis of Disabling Panscleroti morphea of children (Díaz-Pérez et al., 1980) was done. With this case and others reported in the literature, the authors compare this peculiar picture with the usual type of morphea, systemic scleroderma and eosinophilic fasciitis.

摘要

对一名8岁男孩进行了检查,他患有泛发性硬斑病,累及皮肤和软组织的各个层次,病程致残,γ球蛋白多克隆升高,外周血嗜酸性粒细胞增多。既无雷诺病,也无系统性硬皮病的体征。活检标本显示胶原组织增厚和玻璃样变,伴有中度淋巴细胞和浆细胞浸润,主要位于皮下区域和筋膜。诊断为儿童致残性全硬化性硬斑病(迪亚兹-佩雷斯等人,1980年)。作者将该病例及文献中报道的其他病例的这种特殊表现与硬斑病、系统性硬皮病和嗜酸性筋膜炎的常见类型进行了比较。

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