PIEBALDISM

By Gary M. White, MD

Piebaldism


Piebaldism is a genodermatosis in which larger depigmented patches scattered with smaller hyperpigmented macules are present at birth. Melanocytes are absent in the depigmented skin. Piebaldism appears to result from mutations of the KIT protooncogene.

Pie refers to the variegated black and white plumage pattern of the magpie. Bald derives from the greek phalios meaning having a white spot. The term was used in ancient times to describe animals whose bodies were covered in black and white patches.

Clinical           

Congenital, stable, depigmented patches with pigmented macules scattered on the ventral surface of the body is characteristic. Inheritance is AD. 85% of patients have a white forelock. The eyes and ears are normal.

Treatment

Photoprotection is important.  Autologous cultured epidermal grafts induces permanent and complete repigmentation of piebald lesions.  The Erbium:YAG laser allows for rapid and precise disepithelialization hence allowing treatment of large lesions during a single surgical operation.

Additional Pictures

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