Targeting B cells has become a treatment approach of increasing interest in patients with a number of different autoimmune syndromes. In these entities, rituximab, a chimeric antibody against CD20 that depletes peripheral blood B lymphocytes but not plasma cells, has been shown to be effective and safe.1 However, in spite of its use in the treatment of lymphoma, to our knowledge no experience of the effect of rituximab on the course of hyper-IgE syndrome (HIE) has been described thus far.2 Although neutrophils from affected patients have fairly consistently demonstrated chemotactic defects, the pathogenesis of HIE is not fully understood. Assuming that IgE might play a role in the pathogenesis of HIE, and therefore that the reduction of total IgE levels would be beneficial, we used rituximab as a direct treatment of HIE in a patient presenting with mostly atopic manifestations.
Trendelenburg M, Schifferli JA. Rituximab in a Patient With Hyper-IgE Syndrome. Arch Dermatol. 2007;143(6):799-816. doi:10.1001/archderm.143.6.807