Piebaldism
Definition:

Piebaldism is a genetic and rare disorder characterized by the presence of white forelock. Caused by lack of pigmentation, it may also affect the eyebrow, eyelash hair, face, trunk and extremities. It usually manifests at birth and remains throughout a patient's lifetime.


Diagnosis:

Skin biopsies will demonstrate that patients lack completely melanin pigment and melanocytes.


Treatment:

Even if the disorder poses no serious threats, patients are advised to be careful of exposing themselves too much to the sun as this condition increases the risk of sunburns and other related skin diseases. Other remedies include dermabrasion of affected areas and application of cell-suspensions, cosmetic camouflage and melanocyte transplant. It is important to educate patients on how this disease is transmitted.


Symptoms and Signs:

Persons affected with piebaldism have white patches on their forelock and skin on the frontal scalp, among other parts of the body. Usually, surrounding these white patches are narrow borders of hyperpigmented skin.


Causes:

Piebaldism arises when there is a lack of melanocytes in the hair follicles and affected skin. The problem is caused when mutations of the KIT proto-oncogene occur. The mutation's site affects the severity of the disease. As an autosomal dominant genetic disorder, piebaldism manifests in half of the patient's children.


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piebaldism



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