NEMO/IKKγ-Deficient Mice Model Incontinentia Pigmenti

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

Incontinentia Pigmenti

Incontinentia pigmenti (IP) is an uncommon X-linked dominant genodermatosis characterized by four cutaneous stages and frequent association with dental (90%), central nervous system (33%) and ocular (35%) anomalies. The exact pathogenesis of this disorder remains unknown.Herein, we report a newborn girl with inflammatory vesiculobullous and warty skin lesions and a positive family history of IP...

Neonatal incontinentia pigmenti.

DESCRIPTION A3-day-old female baby born by normal delivery following an uncomplicated pregnancy presented with blisters on her legs. These blisters were present since birth and were diagnosed as staphylococcal skin infection and treatedwith oral flucloxacillin and the baby was discharged home. She was referred to our hospital on day 3 as the rash was getting worse. The baby was feeding well and...