Darier's Disease

A 42-year-old woman presented with a papular eruption that first appeared during childhood. Severe pruritus, which worsened during the summer, accompanied the lesions. The patient reported that family members, including her mother and brother, had a similar skin problem. A biopsy of a characteristic lesion on the patient's chest revealed diffuse acantholytic dyskeratosis, which confirmed the clinical diagnosis of Darier's disease.

Dr Amor Khachemoune of Georgetown University Hospital, Washington, DC, comments that Darier's disease, or keratosis follicularis, is an uncommon genetic disorder that is inherited as an autosomal dominant trait. It affects both sexes equally; onset is usually during late childhood.

Early lesions are small, firm, flesh-colored papules that cause severe pruritus and discomfort. Over the ensuing years, the papules develop yellow, malodorous crusts and coalesce to form vegetative plaques. Typically, a symmetrical distribution of lesions involves the face, neck, shoulders, chest, back, and limb flexures. Patients often have distal V-shaped notching of the fingernails.

Therapeutic options are palliative. Topical retinoids, such as tazarotene, and adapalene, a retinoid-like agent, can be tried; the systemic retinoids, isotretinoin and acitretin, are the drugs of choice for severe disease. Cyclosporine may be used to control acute, severe flares. Laser excision, excision and grafting, and dermabrasion have been used to treat hypertrophic lesions.

Improvement in this patient's condition was noted after cyclic treatment with tazarotene gel.