Incontinentia pigmenti - eine seltene Differenzialdiagnose bei Neugeborenen

A newborn girl of Afghan parents was referred to the childrensʼ hospital on the day of birth with the suspected diagnosis of a congenital herpes infection. The otherwise healthy child had scaly and vesicular lesions. The parents were cousins and had 3 healthy daughters and 2 healthy sons. Over the next few days, papules and linear hyperpigmentation developed in the newborn. Histologically, an eosinophilic spongiosis was seen. Based on the clinical and histological findings, we diagnosed an incontinentia pigmenti. When re-interviewed, the mother reported about 2 abortions with male fetuses. The physical examination of the whole family revealed striate, hypopigmented patches and plaques on the lower legs of the mother, a typical finding of the later stage of incontinentia pigmenti.