Betts C M, Bardazzi F, Fanti P A, Tosti A, Varotti C
Department of Experimental Pathology, University of Bologna, Italy.
Dermatology. 1994;189(4):384-91. doi: 10.1159/000246885.
A clinical, histological and ultrastructural study of a 38-year-old woman with generalized progressive hyperpigmentation is reported. Hyperpigmentation started in early infancy, progressed during childhood and has increased to a dark bronze-brown color with 'mottling' in the last 8 years. Histology and electron microscopy revealed epidermal hypermelanosis and 'negroid' distribution of melanosomes in keratinocytes. In our family, autosomal dominant phenotype transmission with 'variable expression' is suggested. The literature on the entity has been reviewed. This case is another rare example of familial progressive hyperpigmentation.
报告了一名38岁全身性进行性色素沉着女性的临床、组织学和超微结构研究。色素沉着始于婴儿早期，在儿童期进展，在过去8年中已发展为深青铜褐色并伴有“斑点”。组织学和电子显微镜检查显示表皮黑素沉着增加以及角质形成细胞中黑素体呈“黑人样”分布。在我们家族中，提示存在具有“可变表达”的常染色体显性表型传递。已对有关该病症的文献进行了综述。该病例是家族性进行性色素沉着的又一罕见实例。
