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Linear IgA dermatosis (LAD) is a rare subepidermal autoimmune bullous disease (AIBD) defined by predominant or exclusive immune deposits of immunoglobulin A at the basement membrane zone of skin or mucous membranes. This disorder is a rare, clinically and immunologically heterogeneous disease occurring both in children and in adults. The aim of this project is to present the main clinical features of LAD, to propose a diagnostic algorithm and provide management guidelines based primarily on experts' opinion because of the lack of large methodologically sound clinical studies. These guidelines were initiated by the European Academy of Dermatology and Venereology (EADV) Task Force Autoimmune Bullous Diseases (AIBD). To achieve a broad consensus for these S2k consensus-based guidelines, a total of 29 experts from different countries, both European and non-European, including dermatologists, paediatric dermatologists and paediatricians were invited. All members of the guidelines committee agreed to develop consensus-based (S2k) guidelines. Prior to a first virtual consensus meeting, each of the invited authors elaborated a section of the present guidelines focusing on a selected topic, based on the relevant literature. All drafts were circulated among members of the writing group, and recommendations were discussed and voted during two hybrid consensus meetings. The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD. These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD. © 2024 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.

Citation

Frédéric Caux, Aikaterini Patsatsi, Meropi Karakioulaki, Emiliano Antiga, Eulalia Baselga, Luca Borradori, Marzia Caproni, Adela R Cardones, Nisha Suyien Chandran, Sören Dräger, Kossara Drenovska, Matthias Goebeler, Claudia Günther, Silke C Hofmann, Dimitrios Ioannides, Pascal Joly, Branka Marinović, Elena Biancamaria Mariotti, Angelo Valerio Marzano, Kimberly D Morel, Dedee F Murrell, Catherine Prost, Miklós Sárdy, Jane Setterfield, Dusan Skiljevic, Soner Uzun, Snejina Vassileva, Giovanna Zambruno, Enno Schmidt. S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology. Journal of the European Academy of Dermatology and Venereology : JEADV. 2024 Feb 29


PMID: 38421060

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