A unique variant of Darier's disease

A 45-year-old black woman presented with a chief complaint of an increasing number of "light spots" on her face, upper trunk, and legs. She had a 4-ye ar history of a pruritic eruption on the dorsum of her hands. The eruption was particularly pruritic in the summer months. Other family members, inclu ding her sister and her daughters, reportedly had a similar dermatologic pr oblem. The patient had been previously evaluated and biopsied by another de rmatologist. The earlier biopsy was nondiagnostic, however, and she present ed for further evaluation of this problem. On physical examination. the patient had hypopigmented macules along her ja wline (Fig. 1), lateral neck, and upper chest. She had similar hypopigmente d macules on her thighs. She had hyperkeratosis of the palmoplantar surface of her hands and feet. The dorsum of her hands had numerous coalescing, sh iny, flat-topped, hypopigmented papules (Fig. 2), and several of her finger nails had distal, V-shaped notching. A punch biopsy from a papule on the dorsum of her hand was obtained. The ep idermis had corps ronds present with focal areas of acantholysis above the basal layer (Fig. 3). The dermis had sparse, superficial, perivascular infi ltrates composed of lymphocytes and histiocytes. These changes were consist ent with our clinical diagnosis of Darier's disease (keratosis follicularis ).