Incontinentia pigmenti is an X_linked dominant genodermat | Dermatology

Incontinentia pigmenti is an X_linked dominant genodermatosis which presents in the newborn period with vesicles in a Blaschkoid distribution. A biopsy would show eosinophilic spongiosis with dyskeratotic keratinocytes and pigment incontinence. A gene defect in NEMO has been identified as the cause for the constellation of fingings