| piebaldism | Autosomal dominant, congenital disorder characterised by localised hypomelanosis of the skin and hair. The most familiar feature is a white forelock presenting in 80 to 90 percent of the patients. The underlying defect is possibly related to the differentiation and migration of melanoblasts, as well as to defective development of the neural crest (neurocristopathy). Piebaldism may be closely related to waardenburg's syndrome. (12 Dec 1998) |
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Synonyms : Cutaneous Albinism, Partial Albinism
| piebaldism |
a congenital autosomal dominant pigmentary disorder of the skin due to absence of functioning melanocytes and melanin, resulting in patchy areas of depigmentation or hypopigmentation, often occurring in association with white forelock. Called also albinismus circumscriptus and localized or partial albinism. Cf. leukoderma and vitiligo.
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