X-linked recessive polyfibromatosis manifesting with spontaneous keloid scars and Dupuytren's contracture.

We present a patient with keloid scars and Dupuytren's contracture comprising the polyfibromatosis syndrome; four members over three generations of his family are also affected. To our knowledge this is the first familial case reported of polyfibromatosis with this specific phenotypic presentation and probable X-linked recessive inheritance.