Piebaldism has been recognized since the late 17th century as a heritable, stable, patterned hypomelanosis lacking extracutaneous manifestations. The earliest awareness of piebaldism dates from classical Greek and Roman literature, but the first extensive work was that of Simon who, in 1861, collected 32 cases from 1698 to the mid-19th century.1 By 1855, 31 pedigrees had been reported and clearly demonstrated autosomal dominant inheritance.2
Within these centuries, the clinical picture characteristic of piebaldism became well established. Most characteristic is a white forelock occurring in 80% to 90% of piebald individuals3; the white-to-silvery hairs arise in a triangular, elongated or diamond-shaped, oft-symmetrical, chalk-white macule that points posteriorly into the scalp and descends to the nasal bridge and medial eyebrows with associated leukotrichia. The posterior aspect of this macule may reach the bregma. In up to 20% of cases, however, cutaneous depigmentation is the only finding; this is characteristically