Severe atopic dermatitis in patient with common variable immunodeficiency: case report

Common Variable Immunodeficiency (CVID) is a rare inborn error of humoral immunity that is characterized by reduced or absent antibody production and recurrent infections. CVID is also associated with a higher risk of autoimmune and lymphoproliferative disorders. Currently, there is no curative treatment and only immunoglobulin replacement therapy is available to treat CVID patients. Atopy is not a common manifestation of CVID. Case report: The patient, a 28-year-old female, had suffered from numerous pulmonary infections, severe atopic dermatitis, and boils since the age of 4. At the age of 16, her immunologic tests revealed a severe reduction of the IgG, IgM, IgE levels with a complete absence of the IgA, and she was diagnosed with CVID. After starting the immunoglobulin replacement therapy, her clinical condition improved. However, atopic dermatitis persisted and worsened over the next 10 years. Despite receiving treatment for atopic dermatitis, she was hospitalized 2-3 times a year with exacerbations and secondary bacterial infections. In 2019 she was involved in a six months clinical trial and received a treatment with anti-thymic stromal lymphopoietin monoclonal antibody Tezepelumab. Within two months her clinical condition significantly improved, no new exacerbations occurred during the study period. Before the treatment her EASI score was 54, after – 12, SCORAD before 46% and after 21%, and IGA before 4 and after 2. After the study, she continued to experience occasional flares of atopic dermatitis and gradual worsening of skin condition. In CVID patients the presence of atopic disorders has not been completely understood due to low levels of total and specific IgE. In the case of recurrent infections and severe atopic dermatitis, an immunodeficiency should be included in the differential diagnosis. In patients with CVID and frequent exacerbations of atopic dermatitis, biological medications could be a promising pathogenetic treatment.