Incontinentia Pigmenti - One case, two diagnoses

Incontinentia Pigmenti is a rare X-linked genodermatosis caused by mutations in the IKBKG gene. It is a systemic disease mainly involving tissues of ectodermic origin, manifesting itself primarily as skin lesions but also as changes in the hair, nails, teeth, breasts, eyes and nervous system. We present a case of a newborn who presented with a vesicular rash in the first week of life.