Hyper Immunoglobulin E Syndrome (HIES): Report of a New Case Treated with Omalizumab and Dexametasone

Abstract 4934

The classical triad of eczema, recurrent skin and lung infections and high serum IgE, other connective tissue, cardiac and brain abnormalities are the accepted diagnostic criteria for HIES. The treatment of HIES is not established due to the scarce number of published cases, however is limited to skin local measures and supportive care. Herein we inform a severe case with response to Omalizumab, a monoclonal anti IgE antibody in combination with Dexametasone.

A 36 year old male with family history of asthma and atopic dermatitis (AD). Since child, the patient had allergies and AD. At 8 years he presented asthma and recurrent upper airway infections at 2 to 3 times per year and 7 dental pieces removal. In the last 3 years he noted important increase of AD with very intense and disabling pruritus without response to treatment and progression to generalized erithrodermia, nodes in legs and arms, and skin trasudate, axilar and inguinal lymph nodes (6×4 cm). Laboratory positive test were eosinophils 6%, serum IgE 29,280 IU/mL. Skin biopsy showed psoriasis-like dermatitis, micro abscesses and dermatopathic lymphadenopathy. We started treatment with trimetoprim/Sulfamethoxazole 80/400 mg twice a day, oral Dexametasone 20 mg weekly and Omalizumab 200 mg subcutaneously every two weeks. After 15 days of treatment there was a rapid improvement of skin derangement and IgE levels dropped to 2000 IU/mL after 16 weeks of treatment. Patient continues on treatment with no side effects and pruritus disappears.

Omalizumab is a monoclonal antibody against IgE recently introduced to the treatment of HIES in a few patients reported since 2008 with excellent response, along with intensive care of skin lesions, prompt antibiotic and antimycotic treatment for infections are the mainstay of HIES management.