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Facts about the skin from DermNet New Zealand Trust. Topic index: A B C D E F G H I J K L M N O P Q R S T U V W X Y Z


Piebaldism

The name piebaldism is derived from a combination of the “pie” in the magpie (a bird of black and white plumage) and the “bald” of the bald eagle (the US national bird that has a white feathered head). Hence the major characteristic of piebaldism is a white forelock (a patch of white hair directly above the forehead).

What is piebaldism?

Piebaldism is a rare inherited condition characterised by:

The condition is present at birth and usually remains unchanged throughout life.

Piebaldism Piebaldism Piebaldism
Piebaldism Piebaldism
Piebaldism

What is the cause of piebaldism?

Piebaldism is due to an absence of melanocytes in affected skin and hair follicles. This is caused by mutations of the KIT proto-oncogene. 14 point mutations, 9 deletions, 2 nucleotide splice mutations, and 3 insertions of the KIT gene are believed to be mutations causing piebaldism. The severity of the condition correlates with the site of the mutation within the KIT gene.

Piebaldism is an autosomal dominant genetic disorder meaning half of an affected person's children will also have the condition.

Piebaldism is one of the cutaneous signs of Waardenburg syndrome.

How is the diagnosis made?

Skin biopsy from patients with piebaldism demonstrates complete lack of melanocytes and melanin pigment. In vitiligo, lesions appear later in life and their configuration and distribution is quite different. If deafness is apparent and the distance between the eyes is greater than normal then the diagnosis of Waardenburg syndrome needs to be considered.

What is the treatment for piebaldism?

Piebaldism is a benign disorder. However, patients with the condition are at risk of sunburn and other disorders caused by overexposure to the sun. Patients must be educated about use of sunscreens, sun protective measures, and sun avoidance during peak hours of UV exposure, and self examination of the skin to detect any sun damage that may increase the risk of skin cancer.

Patients who are self-conscious about their appearance may benefit from the following treatments:

Related information

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Author: Vanessa Ngan, staff writer

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If you have any concerns with your skin or its treatment, see a dermatologist for advice.