Clinical Study of 11 Cases of Piebaldism in Koreans / 대한피부과학회지

ABSTRACT

BACKGROUND: Piebaldism is an uncommon congenital disease inherited in autosomal dominant pattern. It is characterized by stable leukoderma with white forelock and vitiligo-like amelanotic macules usually containing a few normally pigmented or hyperpigmented macules. There have been a few case reports, but no clinical study in Koreans. OBJECTIVE: The purpose of this study was to investigate characteristic clinical features in Korean patients with piebaldism different from that of Caucasian. METHODS: We evaluated 11 patients with piebaldism using retrospective method and telephone survey in regard to sex, family history, clinical features such as distribution of hypopigmented patches, white forelock, normo- or hyperpismented macules within hypopigmented patches, hyperpigmented macules in normal skin, associated systemic disease, and effect of treatment. RESULTS: Ten patients showed autosomal dominant features but one occurred sporadically. The ratio of male to female was 38, and only 6 patients had white forelock. The most common site of hypopigmented patches was the lower leg(in all patients), followed by abdomen, left foot and left buttock, and chest. All patients had normo- or hyperpismented macules in hypopigmented patches, and 4 had hyperpigmented lesion in normal skin. Down's syndrome was associated in one case. We treated 3 cases with epidermal graft. CONCLUSION: Most cases were consistent with other western reports. But our patients revealed female predominance(38), lower incidence of white forelock(about 55%), frequent lower leg involvement(100%), and uncommon distribution of skin lesion such as foot and buttock. These differences reflected characteristic clinical features of piebaldism in Koreans different from those of Caucasians.