S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Frédéric Caux¹, Aikaterini Patsatsi², Meropi Karakioulaki³, Emiliano Antiga⁴, Eulalia Baselga⁵, Luca Borradori⁶, Marzia Caproni⁴, Adela R Cardones⁷, Nisha Suyien Chandran⁸, Sören Dräger⁹, Kossara Drenovska¹⁰, Matthias Goebeler¹¹, Claudia Günther¹², Silke C Hofmann¹³, Dimitrios Ioannides¹⁴, Pascal Joly¹⁵, Branka Marinović¹⁶, Elena Biancamaria Mariotti⁴, Angelo Valerio Marzano^{17

18}, Kimberly D Morel¹⁹, Dedee F Murrell²⁰, Catherine Prost¹, Miklós Sárdy^{21

22}, Jane Setterfield^{23

24}, Dusan Skiljevic²⁵, Soner Uzun²⁶, Snejina Vassileva¹⁰, Giovanna Zambruno²⁷, Enno Schmidt⁹

Affiliations

¹ Department of Dermatology and Referral Center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP and University Paris 13, Bobigny, France.
² Autoimmune Bullous Diseases Unit, 2nd Department of Dermatology, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece.
³ Department of Dermatology and Venereology, Medical Center, University Hospital Freiburg, Freiburg, Germany.
⁴ Department of Health Sciences, Section of Dermatology, Azienda USL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence, Italy.
⁵ Department of Dermatology, Hospital Sant Joan de Deu, Barcelona, Spain.
⁶ Department of Dermatology, University of Bern, Inselspital, Berne, Switzerland.
⁷ Division of Dermatology, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA.
⁸ Division of Dermatology, Department of Medicine, National University Hospital, Singapore, Singapore.
⁹ Department of Dermatology, Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
¹⁰ Department of Dermatology and Venereology, University Hospital "Alexandrovska", Medical University-Sofia, Sofia, Bulgaria.
¹¹ Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany.
¹² Department of Dermatology, University Hospital, Technical University Dresden, Dresden, Germany.
¹³ Department of Dermatology, Allergy and Dermatosurgery, Helios University Hospital Wuppertal, University Witten-Herdecke, Wuppertal, Germany.
¹⁴ 1st Department of Dermatology, Aristotle University School of Medicine, Hospital of Skin and Venereal Diseases, Thessaloniki, Greece.
¹⁵ Department of Dermatology, Rouen University Hospital, INSERM 1234, Normandie University, Rouen, France.
¹⁶ Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia.
¹⁷ Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
¹⁸ Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
¹⁹ Departments of Dermatology and Pediatrics at Columbia University Medical Center, Vagelos College of Physicians and Surgeons, New York, New York, USA.
²⁰ Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia.
²¹ Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
²² Department of Dermatology and Allergy, University Hospital, LMU, Munich, Germany.
²³ St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
²⁴ Centre for Host-Microbiome Interactions, King's College London Faculty of Dentistry, Oral & Craniofacial Sciences, London, UK.
²⁵ Department of Dermatovenereology, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
²⁶ Department of Dermatology, Akdeniz University Faculty of Medicine, Antalya, Turkey.
²⁷ Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

PMID: 38421060
DOI: 10.1111/jdv.19880

Free article

Practice Guideline

S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Frédéric Caux et al. J Eur Acad Dermatol Venereol. 2024 Jun.

Free article

. 2024 Jun;38(6):1006-1023.

doi: 10.1111/jdv.19880. Epub 2024 Feb 29.

Authors

Affiliations

¹ Department of Dermatology and Referral Center for Autoimmune Bullous Diseases, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP and University Paris 13, Bobigny, France.
² Autoimmune Bullous Diseases Unit, 2nd Department of Dermatology, Aristotle University School of Medicine, Papageorgiou General Hospital, Thessaloniki, Greece.
³ Department of Dermatology and Venereology, Medical Center, University Hospital Freiburg, Freiburg, Germany.
⁴ Department of Health Sciences, Section of Dermatology, Azienda USL Toscana Centro, Rare Diseases Unit, European Reference Network-Skin Member, University of Florence, Florence, Italy.
⁵ Department of Dermatology, Hospital Sant Joan de Deu, Barcelona, Spain.
⁶ Department of Dermatology, University of Bern, Inselspital, Berne, Switzerland.
⁷ Division of Dermatology, Department of Internal Medicine, University of Kansas Medical Center, Kansas City, Kansas, USA.
⁸ Division of Dermatology, Department of Medicine, National University Hospital, Singapore, Singapore.
⁹ Department of Dermatology, Lübeck Institute of Experimental Dermatology (LIED), University of Lübeck, Lübeck, Germany.
¹⁰ Department of Dermatology and Venereology, University Hospital "Alexandrovska", Medical University-Sofia, Sofia, Bulgaria.
¹¹ Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Würzburg, Germany.
¹² Department of Dermatology, University Hospital, Technical University Dresden, Dresden, Germany.
¹³ Department of Dermatology, Allergy and Dermatosurgery, Helios University Hospital Wuppertal, University Witten-Herdecke, Wuppertal, Germany.
¹⁴ 1st Department of Dermatology, Aristotle University School of Medicine, Hospital of Skin and Venereal Diseases, Thessaloniki, Greece.
¹⁵ Department of Dermatology, Rouen University Hospital, INSERM 1234, Normandie University, Rouen, France.
¹⁶ Department of Dermatology and Venereology, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia.
¹⁷ Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
¹⁸ Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
¹⁹ Departments of Dermatology and Pediatrics at Columbia University Medical Center, Vagelos College of Physicians and Surgeons, New York, New York, USA.
²⁰ Department of Dermatology, St George Hospital, University of New South Wales, Sydney, New South Wales, Australia.
²¹ Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Budapest, Hungary.
²² Department of Dermatology and Allergy, University Hospital, LMU, Munich, Germany.
²³ St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
²⁴ Centre for Host-Microbiome Interactions, King's College London Faculty of Dentistry, Oral & Craniofacial Sciences, London, UK.
²⁵ Department of Dermatovenereology, Faculty of Medicine, University of Belgrade, Belgrade, Serbia.
²⁶ Department of Dermatology, Akdeniz University Faculty of Medicine, Antalya, Turkey.
²⁷ Genodermatosis Unit, Translational Pediatrics and Clinical Genetics Research Division, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

PMID: 38421060
DOI: 10.1111/jdv.19880

Abstract

Introduction: 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.

Methods: 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.

Results: The guidelines summarizes evidence-based and expert opinion-based recommendations (S2 level) on the diagnosis and treatment of LAD.

Conclusion: These guidelines will support dermatologists to improve their knowledge on the diagnosis and management of LAD.

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References

REFERENCES

1. Jablonska S, Chorzelski TP, Beutner EH, Maciejowska E, Rzesa G. Dermatitis herpetiformis and bullous pemphigoid. Intermediate and mixed forms. Arch Dermatol. 1976;112(1):45–48.
1. Chorzelski TP, Jablonska S. IgA linear dermatosis of childhood (chronic bullous disease of childhood). Br J Dermatol. 1979;101(5):535–542.
1. Venning VA. Linear IgA disease: clinical presentation, diagnosis, and pathogenesis. Immunol Allergy Clin North Am. 2012;32(2):245–253, vi.
1. Wojnarowska F, Marsden RA, Bhogal B, Black MM. Chronic bullous disease of childhood, childhood cicatricial pemphigoid, and linear IgA disease of adults. A comparative study demonstrating clinical and immunopathologic overlap. J Am Acad Dermatol. 1988;19(5 Pt 1):792–805.
1. Giraud L, Welfringer‐Morin A, Boccara O, Frassati‐Biaggi A, Leclerc‐Mercier S, Grootenboer‐Mignot S, et al. Neonatal and self‐healing linear immunoglobulin A dermatosis. J Eur Acad Dermatol Venereol. 2020;34(2):e86–e87.

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S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Affiliations

S2k guidelines on diagnosis and treatment of linear IgA dermatosis initiated by the European Academy of Dermatology and Venereology

Authors

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Miscellaneous