Multiple sclerotic fibromas of the skin. A cutaneous marker of Cowden's disease

Abstract

Multiple hamartoma syndrome, or Cowden's disease, is a rare genodermatosis with multiple organ system involvement in which malignancy, particularly of breast and thyroid, may develop. Multiple trichilemmomas have been classically regarded as the cutaneous hallmark of this disease. We here emphasize multiple sclerotic fibromas of the skin as another specific cutaneous marker of this entity, which may also be helpful in an early diagnosis.