Darier's disease associated with multiple café-au-lait macules.

Darier's disease, or keratosis follicularis, is an autosomal dominant inherited skin disease characterized by keratotic papules predominantly on the upper trunk and scalp, palmar pits, and nail dystrophy. It has been reported in association with basal cell carcinoma and other skin cancers, neuropsychiatric disorders, salivary gland obstruction, renal and testicular agenesis, bone cysts, and psoriasis. We describe the first report of a patient with multiple café-au-lait macules, but no personal or family history of neurofibromatosis, who developed Darier's disease.