Familial Incontinentia Pigmenti in Nigerian Neonate and Mother: A Need For Parental Evaluation in the Management of Genodermatoses

Abstract

Incontinentia pigmenti (I.P) is an uncommon genodermatosis that is mostly characterized by specific sequential skin
lesions along blaschko's lines. These occur in addition to alterations in other tissues of neuroectodermal origin.
Local literature on the inherited form of IP is sparse. We report this condition in a 2 day old female neonate with a
linear vesiculobullous rash that later evolved to become hyperpigmented, scaly and verrucous plaques. Ocular
abnormalities were subsequently found in the neonte. Her 25 year old mother also had a similar history of skin rash
in childhood, which had been replaced with linear atrophic and hypopigmented lesions. An additional finding of a left
accessory nipple was made in the mother.
Despite the unavailability of molecular genetic testing, diagnosis of inherited IP was made in both females based on
clinical findings, and a demonstrated family history of the disease.
This report highlights an uncommonly reported form of IP in our environment as well as the usefulness of parental
evaluation in the management of suspected genetic disorders.
key words: Incontinentia pigmenti, Genodermatoses, Blaschko's lines.