Inborn Errors of Immunity

Eczematous rash and elevated serum IgE are common findings in the general population. However, eczema may be a presenting clinical manifestation of an inborn error of immunity (IEI) such as the hyper-IgE syndromes, Wiskott-Aldrich syndrome, immune polyendocrinopathy X-linked syndrome, and Omenn syndrome. The list of IEIs associated with eczema has been expanding in recent years. These defects often present with additional non-dermatologic features which should increase suspicion for an underlying immune defect. These features may include recurrent or severe infections, skeletal defects, impaired growth, autoimmunity, and other findings. The clinician treating severe eczema should be aware of associations between skin findings and immunodeficiency diseases as prompt evaluation may lead to early detection and treatment of serious or life-threatening immunologic defects in patients with IEI presenting as eczema.