Mutilating palmoplantar keratoderma with periorificial keratotic plaques (Olmsted's syndrome)

We report the case of a boy, now aged 4 years, with a progressive, painful and disabling palmoplantar keratoderma, associated with well-defined hyperkeratotic plaques around the mouth and nostrils. His mother has an identical palmoplantar keratoderma. The palmoplantar keratoderma failed to improve with etretinate, but considerable benefit resulted from full-thickness excision of the skin of both palms, followed by skin grafting. We believe that he has a distinctive but extremely rare form of hereditary palmoplantar keratoderma, first described by Olmsted.