Incontinentia pigmenti: Report of 3 cases from Sarawak

ABSTRACT

Incontinentia pigmenti, also known as Bloch-Sulzberger syndrome, is a rare X- linked dominant multisystem disease involving ectodermal structures namely cutaneous, ocular, dental, neurological and skeletal systems1. Mutation of the nuclear factor kappa B essential modulator (NEMO) gene in chromosome Xq28 is determined to cause this rare genodermatosis2. The cutaneous manifestations are the most characteristic features of this disorder3. We would like to report 3 cases of incontinentia pigmenti seen in the skin clinic, Sarawak General Hospital.