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Phacomatosis Pigmentokeratotica : A 4-month-old Infant with Rare Melanocytic–Epidermal Twin Nevus Syndrome-Case Report
Ching-Ying Wu , Wen-Yu Chang , Chieh-Shan Wu , Gwo-Shing Chen
Dermatol Sinica 25: 261-264, 2007

Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an
organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous
nevus, and other extracutaneous anomalies. Such a combination of nevus sebaceous
with melanocytic lesions may be explained by the concept of twin spotting. We
describe a 4-month-old boy with a speckled lentiginous nevus arising from linear
sebaceous nevus, intractable seizure, visual impairment, and hearing loss. To the best of
our knowledge, this is the first case reported in Taiwan. Since cases of malignant
degeneration of both nevus components have been reported, adequate follow-up of
those with phacomatosis pigmentokeratotica is necessary.

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