Background: Darier's disease is a rare, dominantly inherited genodermatosis; there have not been any large clinical studies of patients with this disease.
Objective: Our purpose was to document the clinical features in a large group of patients with Darier's disease.
Methods: Data were collected from 163 affected persons.
Results: The onset usually occurred between the ages of 6 and 20 years. The disease has a predilection for the skin in seborrheic areas; 96% had acral signs; 6% had hypertrophic flexural involvement; and 13% had oral mucosal lesions. There was no remission. Topical therapy sometimes provided relief of symptoms but had no effect on the progress of the disease. Oral retinoids were effective, but long-term therapy was tolerated poorly. Most patients did not have other medical problems.
Conclusion: Although Darier's disease is a chronic and unremitting burden, most patients manage to lead a relatively normal life.