A Case of Mild Type of Incontinentia Pigmenti

Abstract

A female infant who was born after a full-term spontaneous normal delivery, developed at birth several vesicles and erosions on her left leg, both arms and on the left side of her abdomen. Some of them were linearly arranged. The peripheral eosinophil fraction was 12%. Histologically, intraepidermal vesicles and spongiosis with marked infiltration of eosinophils were observed. From these findings, incontinentia pigmenti was diagnosed. There was no family history of this disorder. She also had a nasolacrimal obstruction, but this spontaneously resolved within three months after birth. We defined mild type incontinentia pigmenti as cases with unilateral eruptions on any of the face, trunk, arms and legs. We summarized the patients with incontinentia pigmenti reported in Japan, classifying then into mild-type and common-type, and carefully evaluated the clinical characteristics of both types while also referring to the literature.