Segmental Darier’s disease, a rare disorder of keratinization: a case report

Abstract

We present a case of Darier disease which is a rare autosomal-dominant genodermatosis. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas. A 30-year-old male came with complaints of papules that were restricted to the lateral aspect of the trunk on the right side and right underarm area. Histopathological examination showed the acanthosis of the epidermis with premature keratosis and suprabasal acantholysis. Darier’s disease must be distinguished histologically from other acantholytic dyskeratoses. It has been observed that biopsy is fundamental to make the final diagnosis. However, a multidisciplinary approach is required to treat affected patients.

Keywords

• autosomal dominant
• darier disease
• keratosis follicularis
• suprabasal clefts