BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

1st Department of Dermatology Venereology Aristotle University School of Medicine Thessaloniki Greece

Autoimmune Blistering Dermatoses Section Department of Dermatology Poznan University of Medical Sciences Poznań Poland

Autoimmune Bullous Diseases Unit 2nd Dermatology Department Aristotle University School of Medicine Thessaloniki Greece

Azrieli Faculty of Medicine Bar Ilan University Safed Israel

Centre for Host Microbiome Interactions Faculty of Dentistry Oral and Craniofacial Sciences King's College London London UK

Clinic of Dermatovenereology University Clinical Center of Serbia Belgrade Serbia

Clinical Immunodermatology St John's Institute of Dermatology Guy's Hospital London UK

Department Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland

Department of Dermatology Allergy and Dermatosurgery Helios University Hospital University Witten Herdecke Wuppertal Germany

Department of Dermatology and Allergology Philipps University Marburg Germany

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Bobigny France

Department of Dermatology and Venereology Akdeniz University Faculty of Medicine Antalya Turkey

Department of Dermatology and Venereology Medical Faculty Medical University Sofia Bulgaria

Department of Dermatology and Venerology Aarhus University Hospital Aarhus Denmark

Department of Dermatology Autoimmune Bullous Diseases Research Center Razi Hospital University of Medical Sciences Tehran Iran

Department of Dermatology Expertise Center for Blistering Disease University Medical Center Groningen University of Groningen Groningen The Netherlands

Department of Dermatology Groupe Hospitalier Paris Seine Saint Denis AP HP Bobigny France

Department of Dermatology Hospital Clínic of Barcelona Universitat de Barcelona Barcelona Spain

Department of Dermatology Immunodermatology and Venereology Medical University of Warsaw Warsaw Poland

Department of Dermatology Inselspital Bern University Hospital University of Bern Bern Switzerland

Department of Dermatology Klinik Landstraße Vienna Austria

Department of Dermatology Koç University School of Medicine Istanbul Turkey

Department of Dermatology Osaka Metroplitan University Graduate School of Medicine Osaka Japan

Department of Dermatology Peking University 1st Hospital Beijing China

Department of Dermatology PGIMER Chandigarh India

Department of Dermatology Rouen University Hospital and INSERM U1234 Centre de Référence des Maladies Bulleuses Autoimmunes Normandie University Rouen France

Department of Dermatology University Hospital Carl Gustav Carus Technical University Dresden Dresden Germany

Department of Dermatology University of California Davis Sacramento California USA

Department of Dermatology University of Lübeck Lübeck Germany

Department of Dermatology University of Münster Münster Germany

Department of Dermatology Venereology and Allergology Innsbruck Medical University Innsbruck Austria

Department of Dermatology Venereology and Allergology University Hospital Würzburg Würzburg Germany

Department of Dermatology Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh India

Department of Dermatology Xijing Hospital 4th Military Medical University Xi'an China

Department of Dermatovenereology Faculty of Medicine University of Belgrade Belgrade Serbia

Department of Dermatovenereology Masaryk University St Anna Hospital Brno Czech Republic

Department of Haematology University Hospital of Aarhus Aarhus Denmark

Department of Medicine NUS Yong Loo Lin School of Medicine Singapore Singapore

Department of Pathophysiology and Transplantation Università degli Studi di Milano Milan Italy

Dermatology Unit Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico Milan Italy

DermoLab Department of Surgical Medical Dental and Morphological Sciences University of Modena and Reggio Emilia Modena Italy

Division of Dermatology Department of Medicine National University Hospital Singapore Singapore

Division of Dermatology Tel Aviv Sourasky Medical Center Tel Aviv Israel

Faculty of Medicine and Dentistry Danube Private University Krems Austria

Genodermatosis Unit Genetics and Rare Diseases Research Division Bambino Gesù Children's Hospital IRCCS Rome Italy

Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany

National Clinical Research Center for Skin and Immune Diseases Beijing China

National Skin Centre Singapore Singapore

PEDEGO Research Unit Department of Dermatology Medical Research Center Oulu Oulu University Hospital and University of Oulu Oulu Finland

Rare Diseases Unit Azienda USL Toscana Centro European Reference Network Skin Member Florence Italy

Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel

Section of Dermatology Department of Clinical and Experimental Medicine University of Parma Parma Italy

Section of Dermatology Department of Health Sciences University of Florence Florence Italy

Unit of Dermatology and Skin Research Laboratory Baruch Padeh Medical Center Poriya Israel

University Hospital Center Zagreb School of Medicine University of Zagreb Zagreb Croatia

Yong Loo Lin School of Medicine Lee Kong Chian School of Medicine Duke NUS Singapore Singapore

Erratum v

J Eur Acad Dermatol Venereol. 2023 Nov;37(11):2378-2379. doi: 10.1111/jdv.19489. PubMed

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