This paper is reported a rare case of palmoplanter keratoma hereditaria-keratoma hereditaria mutilans and differential diagnosis with various palmoplantar keratodermas and dactylolysis are discussed. 本文报告了一例罕见的遗传性掌跖皮肤角化病—遗传性残毁性角化瘤,并与各种掌跖皮肤角化症、断肢症的鉴别进行了讨论。