Severe atopic dermatitis associated with hypogammaglobulinemia
Severe atopic dermatitis associated with hypogammaglobulinemia
Severe forms of atopic dermatitis in children represent complex chronic conditions which are often challenging and difficult to treat. The ethiopathogenesis leading to the development of atopic dermatitis in childhood is complex, with a significant share of dysregulation of immune functions. In some of these children, hypogammaglobulinemia is also present in the context of impaired immune dysregulation. Hypogammaglobulinaemia is usually severe, however, it is mostly transient, characterized as transient hypogammaglobulinaemia in infancy, with a tendency to gradual improvement. Publications describing hypogammaglobulinemia associated with atopic dermatitis are available, but surprisingly, there are almost no published studies using immunoglobulin replacement therapy to modulate immune functions (https://pubmed.ncbi.nlm.nih.gov/?term=atopic%20dermatitis%20and%20hypogammaglobulinemia&sort=date ). In our pilot study, we demonstrated that regular immunoglobulin substitution ameliorates atopic dermatitis in a subset of children, as evidenced by a clinical improvement, reduction of the clinical severity score and a reduction in the need for local corticoid use. The success of immunoglobulin substitution had, in some cases, prevented the use of immunosuppressive therapy otherwise indicated in the most severe forms of the disease. Administration of immunoglobulins is currently available via subcutaneous route in the form of home therapy, which is well tolerated by young children and appreciated by patients’ families.
WG Immunodeficiencies (Basic & Clinical Immunology Section)
Chair: Anna Sediva
Secretary: Marketa Bloomfield