Ke Jing, Tyler J M Jordan, Ning Li, Susan Burette, Baoqi Yang, M Peter Marinkovich, Luis A Diaz, Paul Googe, Nancy E Thomas, Suying Feng, Zhi Liu
Linear IgA bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering skin disease characterized by circulating and tissue-bound IgA autoantibodies that recognize epitopes within the hemidesmosomal protein BP180, including its NC16A domain. Histologically, LABD has long been defined by neutrophil infiltration and dermal-epidermal separation. However, the pathogenic roles of anti-NC16A IgA and neutrophils in LABD, as well as their interactions, have not been thoroughly studied. We show that passive transfer of patient-derived anti-NC16A IgA induce clinical and histologic LABD pathology in humanized NC16A mice that are reconstituted locally or systemically with human neutrophils...
July 10, 2023: Journal of Investigative Dermatology