The surgical treatment of hypertrophic intertriginous Darier's disease.

Abstract

Darier's disease is a rare autosomal-dominant genodermatosis characterised by persistent hyperkeratotic papules and plaques in seborrhoeic areas. The flexural areas of the body are especially difficult to treat, and they are often resistant to current topical and systemic treatment strategies. To our knowledge, this article provides the first description of a successful surgical treatment of a severely debilitating, hypertrophic Darier's disease of intergluteal area, resistant to oral retionids. A 25-year-old male patient was successfully treated with the use of tissue expanders and diseased skin excision. There are no signs of recurrence of the disease in the perianal region 6 months postoperatively, and there is a good result in functional as well as cosmetic terms. The formation of new skin on the excision site was observed, and there were no evident signs of infection. These encouraging results suggest that a patient who responds poorly to conventional treatment in a specific area should be presented to the plastic surgeon at an earlier point in time. Therefore, unnecessary hospitalisations, antibiotic treatments, dose-related adverse effects of oral retionids can be avoided, and eventually a long-term result can be achieved.