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Definition

A genodermatosis usually prenatally lethal in males. In affected females, it causes abnormalities of the skin, hair, eyes, nails, teeth, skeleton, heart, and central nervous system. The prominent skin signs occur in four classic cutaneous stages: perinatal inflammatory vesicles, verrucous patches, a distinctive pattern of hyperpigmentation and dermal scarring.

Acronym

IP

Synonyms

Bloch-Sulzberger syndrome
Familial incontinentia pigmenti male-lethal type
Familial incontinentia pigmenti type II
IP2

 
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