Characteristic MR findings in a neonate with incontinentia pigmenti.

Leukoencephalopathy: unusual sonographic finding in a neonate with incontinentia pigmenti.

ncontinentia pigmenti (IP) is a rare X-linked dominant neurocutaneous syndrome chiefly involving the ectodermal tissues, such as the skin, teeth, hair, eyes, and central nervous system (CNS).1 The diagnosis is usually made when the pathognomonic skin lesions are present; these typically appear in 4 stages: erythematous vesicular rash, verrucous patches, swirling hyperpigmentation, and atrophic ...

Incontinentia pigmenti: MR demonstration of brain changes.

PURPOSE To describe the MR findings in eight girls and women with incontinentia pigmenti, from two families. Four had skin lesions and neurologic disease, and four had only skin lesions. METHODS Eight patients had physical examination, family history, electroencephalogram and MR examination of the brain. MR was repeated in the two cases with more severe changes several years after the first s...

Incontinentia pigmenti*

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the line...

[Incontinentia pigmenti].

Four new cases of incontinentia pigmenti are presented, including chromosome studies of one family. An increased number of chromosome breakages was found in the blood of affected as well as unaffected members of this family.

Incontinentia Pigmenti