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| Fig. 4.6 Darier’s disease. Confluent, crusted papules involving the face, scalp, and chest. |
The diagnosis is based on clinical manifestations and histologic features. The primary lesions are flesh-colored papules that may coalesce into plaques and develop tan, scaly crusts (Fig. 4-6). These keratotic papules are located in “seborrheic areas,” such as the chest, back, ears, nasolabial folds, scalp, and groin. Thick, foul-smelling, warty masses can develop. Flat, wartlike papules may be seen on the dorsa of distal extremities, and 1- to 2-mm punctate keratoses may be present on the palms and soles. Oral and rectal mucosal surfaces often demonstrate small, cobblestone-like papules. The diagnosis can be confirmed by skin biopsies that reveal acantholytic dyskeratosis.
