Abstract :
Incontinentia pigmenti (IP) is an X linked dominant genodermatosis that affects skin, eyes, CNS, teeth and skeletal system. Skin manifestations are most common and occur in four quite distinctive phases. Here, we present a female neonate with vesiculobullous lesions in the limbs and trunk which healed with hyperpigmentation. Skin biopsy revealed massive eosinophilia in the intraepidermal region. Also, eosinophil-filled intraepidermal vesicles were noted. The dermis showed spongiotic inflammation and infiltrates of lymphocytes in superficial dermis which confirmed the diagnosis of IP.
Keyword :
Incontinentia pigmenti, vesiculobullous lesion, Genodermatosis.