Picture of Darier's Disease
Darier's disease, or keratosis follicularis, is a pruritic, autosomal dominant inherited disease with multiple discrete scaling, crusted, and pruritic papules; it is malodorous and disfiguring and also involves the nails and mucous membranes. It is characterized by premature and abnormal keratinization and loss of epidermal adhesion with acantholysis. It usually persists throughout the lifetime of the patient. Primary lesions are reddish, scaling and crusted papules that, when stroked, feel warty. Where crusts have been removed there are slit-like erosions that are later covered by hemorrhagic crusts.