表皮松解性角化过度鱼鳞病一家系

A Family Report of Epidermolytic Hyperkeratosis Ichthyosis

报道表皮松解性角化过度鱼鳞病一家系。家系中无近亲结婚史,2代8人中有5人患病。先证者男,18岁,其母亲46岁,均是出生时即患病,先证者母亲皮疹主要为厚的角质性疣状损害,先证者皮疹主要为糜烂渗出损害,临床与病理均符合表皮松解性角化过度鱼鳞病。口服阿维A胶囊短期内见到明显效果。

A family with epidermolytic hyperkeratosis ichthyosis were reported here. There was no consanguineous marriage in the family which had 5 patients among 8 members in the two generation. A 18-year- old proband presented with EHI since birth with erythematous, erosive lesions over the entire body. The proband mother was 46 years old who presented with horny verrucous plaques. Two cases were in consistence with the clinical and pathological features of EHI. The obvious effects could be obtained in short period by oral administration with retinoid A capsule.