Incontinentia pigmenti in a term neonate: an atypical presentation

Diffuse cortical necrosis in a neonate with incontinentia pigmenti and an encephalitis-like presentation.

Incontinentia pigmenti is a rare neurocutaneous disorder that may present with neurologic symptoms, in addition to a characteristic vesicular rash within the first days of life. We describe a neonate girl presenting with a rash and an encephalopathy who was first thought to suffer from a viral infection and was only later recognized as being affected by incontinentia pigmenti. Cerebral MR imagi...

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*

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