ALLERGIC MANIFESTATIONS OF PRIMARY IMMUNODEFICIENCY DISEASES AND ITS TREATMENT APPROACHES

Abstract

Allergic manifestations are one of the clinical signs of primary immunodeficiency diseases (PID). In this review, the common allergic manifestations of PID are summarized, and their main differential characteristics and treatment approaches are outlined. Allergic manifestations occur more often in patients with combined immunodeficiencies with or without associated or syndromic features. In patients with PID they usually are present in the 1st year of life, may be among the first symptoms of PID, and are commonly manifested by eczema and increased immunoglobulin (Ig)-G levels. Often the skin barrier function is not impaired in patients with eczema and PID, although some diseases (such as Comel-Netherton syndrome) do affect skin barrier function. There is usually no correlation between IgE levels and the severity of allergic skin manifestations. Allergic-like manifestations in PID patients include urticaria-like rash and angioedema. Urticaria-like rash is associated with autoinflammatory disorders, which are commonly accompanied by fever, and caused by a neutrophilic infiltrate in the dermis. Angioedema in hereditary angioedema patients is caused by high bradykinin production. Early differentiation of allergic manifestations in PID from atopic dermatitis and other atopic conditions is very difficult; however, it is very important because it influences on treatment methods. A multidisciplinary approach to the management of PID patients, with the involvement of immunologists, allergists, and formulation of appropriate treatment improve the prognosis and quality of life of the PID patients.