A Case of Hyper-IgE Syndrome Presenting as a Vesiculopustular Eruption in an Infant / 대한피부과학회지

ABSTRACT

The hyper-IgE syndrome is characterized by marked elevated levels of IgE, recurrent cutaneous and systemic staphylococcal infections, peripheral eosinophilia and defective neutrophil chemotaxis. The characteristic cutaneous findings of hyper-IgE syndrome have been reported as an eczematous or atopic dermatitis-like eruption with multiple skin abscesses, whereas the initial presentation of hyper-IgE syndrome in infancy as a papulopustular or vesicular eruption. We report a case of hyper-IgE syndrome featuring a vesiculopustular eruption in infancy. A 15-month-old baby presented with recurrent vesiculopustules on the face and hands. The biopsy specimen revealed eosinophilic spongiotic dermatitis with an eosinophil-predominant perivascular lymphocytic infiltration. These findings might lead to an earlier recognition of hyper-IgE syndrome and an institution of appropriate treatment.