Incontinentia Pigmenti

Abstract
A study of 2 institutionalized patients with incontinentia pigmenti, and of 2 other patients with a strong familial history of the disorder, suggests that this is an X-linked genetic disorder. Since all surviving patients are females and since there are frequent male abortions, the trait probably has great penetration in heterozygotic females and intrauterine lethality in hemizygotic males. Besides the characteristic skin lesions, incontinentia pigmenti is often associated with poor dentition, strabismus and microcephaly. It may be a cause of mental retardation.

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