• | autosomal dominant; congenital depigmentation (presents at birth) |
• | white forelock and depigmented macules |
• | characteristic feature = islands of hyperpigmentation (hyperpigmented macules in white areas and on normal skin) |
• | histology = complete absence of melanocytes |
• | etiology = mutation in c-kit protooncogene C-kit mutation also plays a role in mastocystosis (defect in migration and differentiation of melanoblasts from neural crest) |
• | vs. vitiligo – (piebaldism) typically spares hands and feet, islands of hyperpigmentation, presents at birth, and the white forelock |
• | vs. Waardenburg sx – (piebaldism) lacks heterochromia irides, dystopia acanthorum, congenital sensorineural deafness |
• | bullet - congenital depigmentation with islands of hyperpigmentation, white forelock, from a mutation in c-kit protooncogene |
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