Incontinentia Pigmenti is a multisystem disorder predominantly affecting females and characterized by skin changes in three stages – vesiculobullous, verrucous and hyperpigmented. Not all stages occur necessarily, though. Sixty percent of the patients display abnormalities of the ectodermal tissue: Nail dystrophy, dental abnormalities, hair anomalies are common. The incidence of ocular abnormalities is high and may approach 40%. The disorder is thought to be due to an X-linked dominant gene with lethality in affected males.
Incontinentia Pigmenti, Bloch-Sulzberger Syndrome
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