Medical Definition of Piebaldism

Reviewed on 6/1/2022

Piebaldism is a condition that occurs due to the absence of pigment cells (melanocytes) in certain areas of the skin or hair. The condition is caused by mutations in a gene known as the KIT proto-oncogene. It is inherited in an autosomal dominant manner, meaning that only one copy of the defective gene (from either parent) is necessary to cause the disease. Symptoms and signs include patches of skin or hair that are lighter in color than the surrounding areas. Other common symptoms and signs include a white forelock and a triangular depigmented area on the forehead. The condition occurs in all races and is also found in animals.

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References
Jameson, J. Larry, et al. Harrison's Principles of Internal Medicine, 20th Ed. New York: McGraw-Hill Education, 2018.