Incontinentia Pigmenti: An Iranian case Report

Shirin  Mohamadi; Fahimeh  Abdollahimajd; Soheila  Sotoudeh

doi:10.18502/igj.v9i1.21882

Shirin Mohamadi Division of Neonatology, Department of Pediatrics, Shohadaye Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Fahimeh Abdollahimajd Department of Dermatology, Shohada-e Tajrish Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
Soheila Sotoudeh Department of Dermatology, Children's Medical Center Hospital, Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran

DOI: https://doi.org/10.18502/igj.v9i1.21882

Keywords: Incontinentia Pigmenti; Neonatal Vesiculopustular Lesions; Neonatal Impetigo; Skin Diseases

Abstract

Incontinentia Pigmenti (IP) is a rare X-linked dominant genetic disorder primarily affecting the skin, with cutaneous manifestations present in all cases. Other ectodermal tissues—including the central nervous sys- tem, eyes, hair, nails, and teeth—may also be involved to varying degrees. In this report, we present the case of a newborn female who exhibited widespread vesiculopustular skin lesions at birth, predominantly affect- ing the upper and lower extremities. At 9 hours old, the newborn with skin lesions suspicious for generalized impetigo, transferred to the NICU at Children’s Medical Center in Tehran, Iran, for further evaluation and management. No abnormalities were observed in the hair, nails, oral mucosa, eyes, or central nervous sys- tem during the initial assessment and the final diagnosis was IP.