LONG-TERM FOLLOW-UP OF CUTANEOUS CHANGES IN SIBLINGS WITH MANDIBULOACRAL DYSPLASIA WHO WERE ORIGINALLY CONSIDERED TO HAVE HEREDITARY SCLEROSING POIKILODERMA

Mandibuloacral dysplasia is a rare syndrome characterized by mandibula r hypoplasia, delayed cranial suture closure, dysplastic clavicles, ab breviated, club-shaped terminal phalanges, acroosteolysis, atrophy of the skin over the hands and feet, and poikilodermatous skin changes. W e describe the cases of two siblings with features of mandibuloacral d ysplasia who as children were considered to have hereditary sclerosing poikiloderma. On their reevaluation as adults, the clinical features of their condition were perceived to be compatible with mandibuloacral dysplasia.