Incontinentia Pigmenti: An Iranian Case Report

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 system, 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 affecting 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 system during the initial assessment and the final diagnosis was IP.