Dupilumab therapy in a 12-year-old boy with Kimura disease, allergic rhinitis, and IgG3 deficiency

ABSTRACT

Kimura disease is characterized by painless subcutaneous masses, usually in the head and neck area as well as serum/tissue eosinophilia and elevated serum IgE levels. Various treatment methods, such as surgical excision, corticosteroids, and radiotherapy, have been suggested, but none of these are considered standard therapy due to high recurrence rates. Dupilumab is a human monoclonal antibody that binds to the alpha subunit of IL-4 receptor for IL-4 and IL-13 and inhibits T helper 2 (Th2) response, which plays an important role in the pathogenesis of allergic diseases, such as atopic dermatitis, asthma, and nasal polyps. Kimura disease has also been shown to increase CD4+Th2 cells and Th2 cytokines. We report a case of a 12-year-old boy who had subcutaneous neck and orbital masses of Kimura disease concurrent with allergic rhinitis, decreased lung function, and IgG subclass deficiency. The masses did not improve with omalizumab or immunomodulatory agents, but resolved with dupilumab. Considering potential side effects of steroids or immunosuppressant agents, dupilumab may be an option for initial treatment with Kimura diseases in children.