Piebaldism refers to the absence of mature melanin-forming cells (melanocytes) in certain areas of the skin and hair. What is piebaldism? Piebaldism is a rare inherited disorder of pigmentation characterised by patchy leukoderma (white skin) and white hair (poliosis) present at birth.

Understanding the Context

Piebaldism is caused by genetic mutations, also known as pathogenic variants. Genetic mutations can be hereditary, when parents pass them down to their children, or they may occur randomly when cells are dividing. Piebaldism | About the Disease | GARD - Genetic and Rare Diseases ... In piebaldism, the genetic mutation prevents pigment-bearing cells called melanocytes from dispersing evenly in the skin of a developing fetus.

Key Insights

The melanocytes move away from surfaces on the front and middle of the body, leaving behind characteristic white patches. What is piebaldism and what causes it? What is piebaldism? Piebaldism is a genetic condition, typically present at birth, in which a person develops an unpigmented or white patch of skin or hair.... Piebaldism is a rare congenital pigmentation skin disorder characterized by the presence of hypopigmented and depigmented skin areas (leukoderma) on various parts of the body, preferentially on the forehead, chest, abdomen, upper arms, and lower extremities, that are associated with a white forelock (poliosis), and in some cases with ...

Final Thoughts

This topic will review the pathogenesis, clinical manifestations, diagnosis, and management of piebaldism. Other disorders of hypopigmentation are discussed separately.