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. 2019 Jun 4;10:1259. doi: 10.3389/fimmu.2019.01259

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Copyright © 2019 Kim and Kim.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Schematic representation of a membrane-associated adhesive junction in the epidermis and autoantigens in PNP. Keratinocytes in the epidermis are connected via desmosomes. Desmosomal cadherins, desmoglein (Dsg) and desmocollin (Dsc), are transmembrane proteins that form hetero- or homodimers in the intercellular area. At the cytoplasmic side of the desmosome, plakophilin (Pkp) and plakoglobin (Pg) bind to intracellular domains of desmosomal cadherins. Desmoplakin (Dpk) interacts with Pkp, Pg, and keratin filaments. Envoplakin, periplakin, and epiplakin serve to link keratin filaments and the plasma membrane. Desmosomal components known to act as autoantigens in PNP are envoplakin, periplakin, epiplakin, Dpk, Dsg, and Dsc. Hemidesmosomes anchor the epidermis to the dermis. Plectin and BP230, which connect keratin filaments, bind to α6β4 integrin and BP180, which are transmembrane proteins in hemidesmosomes. α6β4 integrin binds to laminin 332, which interacts with type VII collagen in the dermis. Autoantibodies against BP230, BP180, and plectin can be observed in PNP.