Three cases of Darier's disease in a family showing marked heterogeneous clinical severity.

We report a Japanese family in which two sisters and their mother with Darier's disease showed apparent heterogeneous clinical severity. The elder sister had extensive skin lesions typical of Darier's disease on the scalp, face, neck, axillae and chest. The skin lesions of the younger sister were less severe. The mother had had skin lesions due to Darier's disease with moderate severity since her teenage years. The lesions had gradually involuted as she grew older, leaving only postinflammatory pigmentation around the neck. Thus, spontaneous remission of skin lesions, rare in Darier's disease, and heterogeneous clinical severity between and within generations were noted in this family.