Primary Immunodeficiency and Related Diseases

Abstract

In Allergology International ( AI ) Vol. 61 No. 2, we have 6 Review Articles, 11 Original Articles, and 1 Letter-to-the-Editor report. AI is publishing theme issues that include reviews in important fields of current research activity written by eminent experts. The theme of this issue is “ Primary Immunodeficiency and Related Diseases”. Cutaneous manifestations are displayed as both concomitant or more rarely main symptoms in immunological disorders such as immunedeficiency disease, autoimmune disease, or autoinflammatory disease. Patients with immunedeficiency often have cutaneous abnormalities ( eczema, telangiectasia, molluscum contagiosum, warts, etc.) and chronic eczema is a common problem, including atopic dermatitis-like skin lesion. On the other hand, autoinflammatory disease, which is categorized as a group of inflammatory disorders that are non-infectious, non-allergic, nonautoimmune and non-immuno-deficient, also displays distinctive cutaneous manifestations. 1 Tadashi Ariga discusses about Wiskott-Aldrich syndrome (WAS), including the renewed clinical and basic researches for WAS. WAS is characterized clinically with the triad; immunodeficiency, bleeding tendency with microthrombocytopenia and severe eczema. Topical tacrolimus would be effective in some WAS patients with severe eczema. The pathogenesis of eczema in WAS remains unknown; however, the similar basis for atopic dermatitis could be considered, because high level of IgE and inbalanced cytokine production patterns (Th1 < Th2) are generally observed. 2 Minegishi and Saito discusses comprehensively reviewed hyper-IgE syndrome (HIES). HIES is a complex primary immunodeficiency, characterized by atopic dermatitis-like skin lesion associated with extremely high serum IgE levels and susceptibility to infections with extracellular bacteria and fungi. Yoshiyuki Minegishi is an excellent pioneer for clinical and basic researches of HIES and identified that most of the patients with HIES have dominant negative mutations in STAT3. 3