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Genetics

Kmetz EC, Shashidhar Pai G, Burges GE.

Medical University of South Carolina, Charleston, SC, USA.

 Pediatr Dermatol 2009;26:83–6.

Editor’s note: Incontinentia pigmenti (IP) is a rare, X-linked, dominant genodermatosis that is usually lethal prenatally in males. The skin, hair, nails, teeth, eyes, and central nervous system can be variably abnormal in affected females. The four classic cutaneous stages are: perinatal inflammatory vesicles, verrucous patches, a distinctive pattern of hyperpigmentation, and dermal scarring.

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