piebaldism

[pi´bawld-izm] 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.

pie·bald·ism

(pī-bawld'izm), [MIM*172800] Patchy absence of the pigment of scalp hair, giving a streaked appearance; patches of vitiligo may be present in other areas due to absence of melanocytes; often transmitted as an autosomal dominant trait caused by mutation in the KIT protooncogene on 4q and may be associated with neurologic defects [MIM*172850] or eye changes [MIM*172870]. Compare: Waardenburg syndrome. Synonym(s): cutaneous albinism, piebald skin, piebaldness

A striking focal depigmentation of hair of the anterior scalp margin, which has no clinical significance, and is typically seen in the Waardenburg syndrome, which is characterised by leukoderma, a flattened nasal bridge and cochlear deafness, see there. It may also be seen in tuberous sclerosis and Vogt-Koyanagi syndrome

pie·bald·ness

(pī'bawld-nĕs) Patchy absence of the pigment of scalp hair, giving a streaked appearance; patches of vitiligo may be present in other areas due to the absence of melanocytes. May be associated with neurologic defects or eye changes.
Synonym(s): piebald skin, piebaldism.

piebaldism

1. VITILIGO. 2. Partial ALBINISM. 3. A rare hereditary disease in which there are patchy areas of skin with no pigment.

单词	piebaldism
释义	piebaldism piebaldism [′pī‚bȯl‚diz·əm] (medicine) A pigmentary disorder characterized by patterns of white spots on the skin and caused by an inherited absence of melanocytes. piebaldism piebaldism [pi´bawld-izm] 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. pie·bald·ism (pī-bawld'izm), [MIM172800] Patchy absence of the pigment of scalp hair, giving a streaked appearance; patches of vitiligo may be present in other areas due to absence of melanocytes; often transmitted as an autosomal dominant trait caused by mutation in the KIT protooncogene on 4q and may be associated with neurologic defects [MIM172850] or eye changes [MIM*172870]. Compare: Waardenburg syndrome. Synonym(s): cutaneous albinism, piebald skin, piebaldness A striking focal depigmentation of hair of the anterior scalp margin, which has no clinical significance, and is typically seen in the Waardenburg syndrome, which is characterised by leukoderma, a flattened nasal bridge and cochlear deafness, see there. It may also be seen in tuberous sclerosis and Vogt-Koyanagi syndrome pie·bald·ness (pī'bawld-nĕs) Patchy absence of the pigment of scalp hair, giving a streaked appearance; patches of vitiligo may be present in other areas due to the absence of melanocytes. May be associated with neurologic defects or eye changes. Synonym(s): piebald skin, piebaldism. piebaldism 1. VITILIGO. 2. Partial ALBINISM. 3. A rare hereditary disease in which there are patchy areas of skin with no pigment.
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