Pigmenti incontinence. Presentation of a case

Abstract

The incontinentia pigmenti is a genodermatosis that affects the pigmentation of the skin and is usually associated with a wide variety of alterations in eyes, nails, hair, teeth, skeleton, heart and central nervous system. Most cases of pigmenti incontinence occur sporadically as a result of a de novo mutation consisting of the deletion of a large part of the NEMO gene (Nuclear Factor Kappa B Essential Modiffer).

We present a 6-year-old patient with hyperpigmented lesions following the Blaschko lines. She has no history during the perinatal period, was born vaginally without complications, with a normal cognitive development for her age, without neurological aectation, which calls attention because although this disease mainly affects the skin, other associated disorders must be considered, including dental defects, convulsive episodes, mental retardation, ocular abnormalities and childhood neoplasms.