A Case of Incontinentia Pigmenti in a Male Infant

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A male infant aged 0, gestational age 39 weeks and 6 days, was delivered by Cesarean section (birth weight 2752g). After birth, he had vesicles and erythema with scales and crusts that were mainly arranged on the basis of Blaschko lines of the right upper and lower extremities, and the tinted erythema was present in a small area of the left side. Histological examination of an erythema sample from his right thigh showed numerous eosinophils and neutrophils in a spongiotic epidermis and perivascular infiltration of eosinophils and lymphocytes in the upper dermis. He was diagnosed as incontinentia pigmenti based on the characteristic features and pathological findings. All vesicles and erythema improved within 5 weeks, leaving only hyperpigmentation. He had a normal 46, XY karyotype. Results of MRI brain scans and ophthalmic examination were normal. Male cases of incontinentia pigmenti are rare, so we have reviewed the characteristic features of this skin condition.
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