Incontinentia pigmenti (Bloch-Sulzberger syndrome) in neonates

Jūratė Buinauskienė, Evelina Buinauskaitė¹, Skaidra Valiukevičienė²

Clinic of Neonatology, ¹Faculty of Medicine, ²Clinic of Skin and Venereal Diseases, Kaunas University of Medicine, Lithuania

Key words: incontinentia pigmenti, female, neonatal period, skin alterations, eosinophilia.

Summary. A female newborn presented with emerging skin lesions, systemic eosinophilia, and eosinophilic reaction in the skin, liver, lungs, spleen, lymphatic nodes, porencephalia, convulsions, and disorders of thermoregulation. In addition to that, respiratory and heart failure, as well as brain edema were progressing. The suspected diagnosis of incontinentia pigmenti (Bloch-Sulzberger syndrome) was confirmed postmortem by skin biopsy.

Correspondence to J. Buinauskienė, Clinic of Neonatology, Kaunas University of Medicine Hospital, Eivenių 2, 50009 Kaunas, Lithuania. E-mail: jbuinauskiene@yahoo.com

Received 24 February 2005, accepted 10 May 2005