After that, clinically similar patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) [14–16] and IPEX-like syndrome [17, 18], hyper IgE syndrome (HIES) [19, 20], zeta-chain-associated protein kinase 70 (ZAP70) deficiency [21], and common variable immunodeficiency (CVID) [22] were reported to suffer from pemphigoid blistering conditions, including BP, pemphigoid nodularis, and mucous membrane pemphigoid. Here, ZAP70 is linked to polyendocrinopathy.