Piebaldism

Piebaldism is a rare autosomal dominant disorder of melanocyte development in which the pigment-producing cells called melanocytes are absent from certain areas of the skin and hair. Typical features are a congenital white forelock, a triangular depigmented patch on the forehead, and scattered patches of normally pigmented and lighter skin, though the degree and pattern vary widely even within one family. It differs from true albinism because eye pigmentation is normal and the affected cells keep the ability to make pigment but have that function switched off, and it is unrelated to vitiligo. It is usually recognised at birth, and it has been associated with the genes KIT and SNAI2. In some people it occurs together with more serious developmental problems such as Waardenburg syndrome and Hirschsprung disease.