Darier-white disease

We present a 58-year-old woman with bipolar disorder and with a longstanding history of yellowbrown, hyperkeratotic papules in a seborrheic distribution and nail changes. Her father and paternal grandmother had similar eruptions and also had psychiatric disease. Histopathologic examination showed acantholysis and dyskeratosis, which was consistent with Darier-White disease. Darier-White disease is a rare, hereditary disorder that is inherited in an autosomal dominant manner and that usually presents in adolescence or early adulthood due to mutations in the SERCA2 calcium pump. The resultant alterations in calcium homeostasis affect desmosome assembly and lead to acantholysis and apoptosis, which creates the characteristic eruption. Genetic linkage analysis of affected families shows that a susceptibility locus for bipolar disorder cosegregates with genetic markers that are found in the Darier-White disease region. Treatments, which include topical emollients, mild keratolytics, and topical or oral retinoids, are aimed at improving the appearance of skin, relieving symptoms, and preventing or treating infectious complications.