Diaz-Perez J L, Connolly S M, Winkelmann R K
Arch Dermatol. 1980 Feb;116(2):169-73.
Fourteen children with generalized morphea involving all levels of the skin and soft tissues were examined. The term "acral pansclerotic morphea" describes the distribution and the multiple levels of sclerosis. Lymphocytic inflammation and hyaline pannicultitis were observed on biopsy specimens in some cases. Laboratory data were characterized by a polyclonal elevation of gamma-globulin level and by peripheral eosinophilia. Pulmonary changes in five patients and esophageal changes in one imply that acral pansclerotic morphea may be assoicated with mild nonprogressive visceral change. Although cyclophosphamide may retard the process, no satisfactory treatment for progressive, mutilating acral pansclerotic morphea has been found.
对14例累及皮肤和软组织各层的泛发性硬斑病患儿进行了检查。“肢端全硬化性硬斑病”这一术语描述了硬化的分布和多个层次。在一些病例的活检标本中观察到淋巴细胞炎症和透明变性脂膜炎。实验室数据的特征为γ球蛋白水平的多克隆升高和外周血嗜酸性粒细胞增多。5例患者出现肺部改变，1例出现食管改变，这意味着肢端全硬化性硬斑病可能与轻度非进行性内脏改变有关。虽然环磷酰胺可能会延缓病情进展，但尚未找到针对进行性、致残性肢端全硬化性硬斑病的满意治疗方法。
