Regulatory T-cell deficiency and autoimmune skin disease: Beyond the scurfy mouse and immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome  Takashi Hashimoto, MD, Hayato Takahashi, MD, PhD, Shimon Sakaguchi, MD, PhD  Journal of Allergy and Clinical Immunology  Volume 142, Issue 6, Pages 1754-1756 (December 2018) DOI: 10.1016/j.jaci.2018.08.028 Copyright © 2018 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Illustrations for cellular and humoral immunity-related findings in the study by Muramatsu et al.8 A, Schematic presentations of how Treg cell dysfunction leads to abnormal skin conditions in scurfy mice or patients with IPEX syndrome, as well as a possible disease process in the development of blister formation in patients with BP. ?, Issues that should be confirmed in future studies. B, Schematic presentations of protein structures of human BP180 and BP230 and differences in immune reactivity. Beneath the schematic structures of both BP180 and BP230, positive domains are shown for (1) pathogenicity in passive transfer mouse disease models, (2) autoantibodies in patients with BP, (3) autoantibodies in scurfy mouse sera, and (4) autoantibodies in sera from patients with IPEX syndrome. Shaded rectangles indicate positive domains. Positive rates for both scurfy mice and patients with IPEX syndrome in this study are shown within rectangles. Abs, Antibodies; C15 domain, 15th collagenous domain of BP180; DPP4, dipeptidyl peptidase 4; LABD, linear IgA bullous dermatosis; MMP, mucous membrane pemphigoid; NC16A domain, noncollagenous 16A domain of BP180 (corresponding to NC14A domain in murine BP180; Tfh, follicular helper T. Journal of Allergy and Clinical Immunology 2018 142, 1754-1756DOI: (10.1016/j.jaci.2018.08.028) Copyright © 2018 American Academy of Allergy, Asthma & Immunology Terms and Conditions