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 (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...

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...

In the United States 1.4 million children were maltreated in 1988, resulting in an estimated 2000 to 5000 deaths.1 Largely due to the rising awareness and sensitivity to the horrors of child abuse, the number of deaths declined to approximately 1500 in 1993.2 Guidelines have been published to aid in the identification and management of child maltreatment,3 and reporting of all suspicious cases ...

Case presentation of staged surgical treatment of patients with spinal deformity in incontinentia pigmenti syndrome. As a result of surgical procedure proper sagittal balance of the body was restored.

Incontinentia pigmenti is a rare, dominantly X-linked genodermatosis characterized by multisystemic involvement that is lethal prenatally in the majority of affected males and shows great clinical variability when it is expressed in women. Recently it has been shown that mutations of the gene NEMO/IKK-g located in Xq28 cause the expression of the disease, being only one mutation responsible for...