Incontinentia pigmenti with remarkable cutaneous manifestations

Abstract

Background Incontinentia pigmenti (IP) is a genetically determined dysplastic ectodermal disease involving the cutis, hair, teeth, micro-vascular system and central nervous system. Successive skin manifestations develop in four steps, the first one occurs during early infancy or manifest at birth. IP is a dominant mediated genetic disease of the X chromosome due to abnormalities of the IKBKG gene. The purpose of the study is to report cases of incontinentia pigmenti with variable cutaneous presentation.   Methods Twelve patients with incontinentia pigmenti were evaluated by case series descriptive study during the period from 2017-2023 years. All demographic and clinical information were recorded. Full cutaneous examination was carried out.   Results The analysis of twelve patients with incontinentia pigmenti showed that all patients were females, with positive family history in one patient and their ages ranged from 2-6 months. Eleven patients showed warty vesicular erythematous rash in a swirled and whorled pattern with some blaschkoid line features in one side of body except one patient showed generalized rash while in one patient the rash was just generalized whorled pigmentation with no blistering warty rash. More than one stage of rash evolution was noticed in 11 patients often with warty blistering plaques. The warty blistering, the rash was resolved after topical steroid cream.   Conclusion Twelve female infants with incontinentia pigmenti were assessed, mostly with unilateral warty erythematous blistering rash. The sequential stages of the disease were not revealed but in contrast more than one stage was demonstrated at the same time.