Darier-White disease is an uncommon genodermatosis that is commonly under-recognized and is characteristically refractory to treatment. It is most frequently due to an autosomal dominant mutation in an intracellular calcium pump. It can be associated with severe cutaneous infections requiring hospitalization as well as neurologic and psychiatric comorbid disease. There are many treatment modalities described in the literature and systemic retinoids are the most effective. An increased clinical suspicion could possibly lead to a decreased delay in the diagnosis of this disease and an increased quality of life for these patients. We report a case of a man with Darier-White disease followed by a review of the current literature on the pathogenesis, clinical features, diagnosis, and treatment of this cutaneous malady.

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