Piebaldism

Piebaldism is a rare skin disorder which resembles and presents with vitiligo spots. Is a autosomal dominant disorder of melanocyte development characterized by a congenital white forelock and multiple symmetrical hypo pigmented or depigmented macules.

Piebaldism is due to an absence of melanocytes in affected skin and hair follicles as a result of mutations of the KIT proto-oncogene.

Piebaldism is an autosomal dominant genetic disorder of pigmentation characterized by congenital patches of white skin and hair that lack melanocytes. Piebaldism results from mutations of the KIT proto-oncogene, which encodes the cell surface receptor Tran membrane tyrosine kinase for an embryonic growth factor, steel factor

The white hair and patches of such patients are completely formed at birth and do not usually expand thereafter.

Piebaldism is a benign disorder. The white forelock is evident in 80-90% of those affected. Both hair and skin in the central frontal scalp are permanently white from birth or when hair color first becomes apparent. The forelock and white skin may have a triangular shape.

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White spots may be observed on the face, trunk, and extremities and tend to be symmetrical in distribution and irregular in shape. They represent a focal lack of melanocytes.

This depigmented skin may show a narrow border of hyper pigmentation or island of pigmentation and has white hair that is otherwise normal-appearing emanating from it.

White patches of hair may be located other than frontally in some patients. The only pigmentation change of skin or hair may be a white forelock in some patients.

Depigmented skin in piebaldism is generally considered unresponsive to medical or light treatment. Dermabrasion and thin split-skin grafts are preferred surgical procedures. Autologous punch grafting for regimentation in piebaldism is considered in selected individuals.

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