Incontinentia pigmenti. Recurrence of inflammatory lesions in a young adult.

Abstract

Rarely, pediatric disorders recur later in life. The case of a young girl presenting a relapse of the inflammatory stage of incontinentia pigmenti is reported. The dermatitis was preceded by febrile pharingytis and was distributed along the hypopigmented streaks of stage IV. The role of the NEMO gene in the NF-kB pathway has allowed our undestanding of these inflammatory recurrences.