先天性角化不良6例

Six cases of dyskeratosis congenita

报道6例先天性角化不良,均有典型的临床表现,即皮肤异色症样皮损、指趾甲萎缩脱落和粘膜白斑等。其中1例伴有再生障碍性贫血。5例患者的皮损曾行组织病理学检查,其改变均与本病相符。

Six cases of dyskeratosis congenita were reported.All six patients presented with widespread reticulated and hyperpig-mented patches with poikilodermatous appearances,nail atrophy and shed leukoplakia,the characteristics of dyskeratosis congeni ta.One patient also had aplastic anemia.Skin biopsy was obtained from five cases,revealing pathological changes consistent with dyskeratosis congenita.