Piebaldism is a rare autosomal dominant genetic disorder characterized by localized and stationary hypomelanosis of skin and hair secondary to an absence of melanocytes in the involved skin. Depigmentation in piebaldism shows a characteristic
distribution that involves the forehead, ventral chest, abdomen, and extremities. Cases of piebaldism have been reported I association with extracutaneous abnormalities such as heterochromia irides, osteopathia striata, deafness, mental
retardation, and
Hirschschsprung's disease. (Kor J Dermatol 1996;34(1) : 151~154)
|