S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

Abstract

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.

Focus on pemphigus treatment publications: A bibliometric and visual analysis (1992–2022)

“…The treatment of PNP remains challenging. The treatment of the underlying malignancy is always recommended because it can result in PNP improvement [ 56 ]. Early detection and radical resection of tumors such as localized Castleman disease and benign thymoma can improve the condition of PNP or achieve complete remission within 1–2 years [ 56 , 57 ].…”

“…The treatment of the underlying malignancy is always recommended because it can result in PNP improvement [ 56 ]. Early detection and radical resection of tumors such as localized Castleman disease and benign thymoma can improve the condition of PNP or achieve complete remission within 1–2 years [ 56 , 57 ]. In terms of drug therapy, systemic corticosteroids (prednisolone) in combination with steroid-sparing agents (azathioprine, MMF, cyclosporine, etc.)…”

Progress in Dermatology and Venereology in 2023 as reflected in the Journal of the European Academy of Dermatology and Venereology

“…The European guidelines for paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome describe the clinical findings with severe chronic mucositis and polymorphic skin lesions and the diagnostic importance of anti‐envoplakin antibodies or antibodies to rat bladder epithelium 8 …”

“…The European guidelines for paraneoplastic pemphigus/ paraneoplastic autoimmune multiorgan syndrome describe the clinical findings with severe chronic mucositis and polymorphic skin lesions and the diagnostic importance of anti-envoplakin antibodies or antibodies to rat bladder epithelium. 8 From the international Degos Disease Registry, 96 patients with atrophic papulosis (Köhlmeier-Degos disease) were studied and differences between children and adults were investigated. In childhood, there were more male patients.…”

Corrigendum: S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

“…In the version of this article initially published, 1 Prof. Angelo Valerio Marzano affiliation was listed as “Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy”. His correct affiliations are the 3 Dermatology Unit, Fondazione IRCCS Ca′ Granda Ospedale Maggiore Policlinico, Milan, Italy and 56 Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.…”