Abstract
The clinical manifestations of Darier's disease are polymorphic and diverse. Guttate leukoderma has been described in around twenty patients with genetically pigmented skin. We report a case of widespread guttate leukoderma several years before the classic signs of Darier's disease in a patient with black skin.A 19-year-old woman consulted for characteristic signs of Darier's disease evolving for the previous four years. Examination revealed perifollicular, non-confluent hypopigmented macules and papules in small drop size (1-5mm in diameter) scattered on the trunk, limbs and the jaw and chin. These hypopigmented lesions had been present since the age of six years. Histology of the keratotic papules confirmed the diagnosis of Darier's disease. Histological inspection of a hypopigmented lesion showed hyperkeratosis, acantholysis and a considerable reduction of epidermal melanin pigment.Our observation suggests that a guttate leukoderma could be an early sign, readily accessible to dermatologists, in the diagnosis of Darier's disease in black-skinned patients.
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