Cutaneous manifestations of hyper-IgE syndrome in infants and children

Chamlin, Sarah L.; McCalmont, Timothy H.; Cunningham, Bari B.; Esterly, Nancy B.; Lai, Chan-Ho; Mallory, Susan Bayliss; Mancini, Anthony J.; Tamburro, Joan; Frieden, Ilona J.

doi:10.1067/mpd.2002.127503

« Previous Next »The Journal of Pediatrics
Volume 141, Issue 4 , Pages 572-575, October 2002

Cutaneous manifestations of hyper-IgE syndrome in infants and children☆

Departments of Dermatology, Pathology, and Pediatrics, University of California, San Francisco; the Division of Dermatology, Children's Hospital and Health Center, San Diego, California; the Division of Dermatology, Children's Memorial Hospital, Chicago, Illinois; the Division of Dermatology, Washington University, St Louis, Missouri; and the Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee

Received 18 February 2002; received in revised form 6 May 2002; accepted 13 June 2002.

Abstract

We describe 8 children with hyper-IgE syndrome who had papulopustular eruption on the face and scalp in the first year of life. Seven of the 8 patients had persistent peripheral eosinophilia and 3 had leukocytosis noted before diagnosis. Skin biopsy specimens in 6 patients revealed spongiosis and perivascular dermatitis and/or folliculitis with a predominance of eosinophils. Two patients had bone fractures and osteopenia. Recurrent pneumonia occurred in 6 children and pneumatoceles in 5. The diagnosis of hyper-IgE syndrome was made an average of 18 months after the onset of the initial papulopustular eruption. These findings may lead to earlier recognition of the disease and institution of appropriate treatment. (J Pediatr 2002;141:572-5)

Abbreviations: EPF , Eosinophilic pustular folliculitis