A challenging case of recalcitrant Hyper-IgE syndrome successfully treated with omalizumab

Hyper-IgE syndrome (HIES) is a rare group of primary immunodeficiency diseases characterized by elevated IgE levels, eosinophilia, recurrent pyoderma, ear infection, eczematous dermatitis, and pulmonary infection. The treatment depends on the clinical presentation of the disease. The eczematous skin lesions usually respond to a topical steroid, calcineurin inhibitors, and sometimes, based on severity, systemic drugs are given. The secondary infections are usually treated with oral and topical antibiotics. We are describing a case of HIES who presented with severe recalcitrant itchy oozy eczematous skin lesions which did not respond to conventional systemic drugs and were later on controlled with injection omalizumab. In addition, we have discussed the HIES in detail in the current article.