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.Histology revealed typical eosinophilic infiltrate and spongiosis in the biopsy sample.The mother had hypopigmented lesions compatible with the same diagnosis, later confirmed in both cases by genetic testing.Although rare, incontinentia pigmenti should be considered in a child presenting vesicular and verrucous cutaneous lesions.Timely diagnosis is a must for establishing adequate follow-up.