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. 2022 Jul 22;13:953546. doi: 10.3389/fimmu.2022.953546

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Copyright © 2022 Schauer, Rafei-Shamsabadi, Mai, Mai, Izumi, Meiss and Kiritsi

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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Clinical presentation and BP progression of patient #1. Upper left picture (Nov 2017): eroded, haemorrhagic crusts on erythematous ground, which changed to urticarial plaques in June 2018 (upper right picture), when BP180 NC16A ELISA became positive. Direct immunofluorescence (lower left picture) with linear IgM n-serrated deposition at DEJZ of patient #1. Serological course of patient #1: initial positivity of BP230, followed by FLBP180 and BP180 NC16A in the course of time. Serological remission after therapy with rituximab in February 2019.