S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).
Journal
Journal of the European Academy of Dermatology and Venereology : JEADV
ISSN: 1468-3083
Titre abrégé: J Eur Acad Dermatol Venereol
Pays: England
ID NLM: 9216037
Informations de publication
Date de publication:
Jun 2023
Jun 2023
Historique:
received:
27
09
2022
accepted:
16
01
2023
medline:
15
5
2023
pubmed:
26
3
2023
entrez:
25
3
2023
Statut:
ppublish
Résumé
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. 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. 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. 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.
Sections du résumé
BACKGROUND
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
OBJECTIVE
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
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
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.
Types de publication
Guideline
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1118-1134Commentaires et corrections
Type : ErratumIn
Informations de copyright
© 2023 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.
Références
Anhalt GJ, Kim SC, Stanley JR, Korman NJ, Jabs DA, Kory M, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med. 1990;323:1729-35.
Anhalt GJ. Paraneoplastic pemphigus. Adv Dermatol. 1997;12:77-96; discussion 97.
Nguyen VT, Ndoye A, Bassler KD, Shultz LD, Shields MC, Ruben BS, et al. Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome: a reappraisal of paraneoplastic pemphigus. Arch Dermatol. 2001;137:193-206.
Schmidt E, Kasperkiewicz M, Joly P. Pemphigus. Lancet. 2019;394:882-94.
Maglie R, Genovese G, Solimani F, Guglielmo A, Pileri A, Portelli F, et al. Immune-mediated dermatoses in patients with haematological malignancies: a comprehensive review. Am J Clin Dermatol. 2020;21:833-54.
Kridin K, Schmidt E. Epidemiology of pemphigus. JID Innov. 2021;1:100004.
Hashimoto T, Amagai M, Watanabe K, Chorzelski TP, Bhogal BS, Black MM, et al. Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol. 1995;104:829-34.
Borradori L, Trueb RM, Jaunin F, Limat A, Favre B, Saurat JH. Autoantibodies from a patient with paraneoplastic pemphigus bind periplakin, a novel member of the plakin family. J Invest Dermatol. 1998;111:338-40.
Amagai M, Nishikawa T, Nousari HC, Anhalt GJ, Hashimoto T. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice. J Clin Invest. 1998;102:775-82.
Mahoney MG, Aho S, Uitto J, Stanley JR. The members of the plakin family of proteins recognized by paraneoplastic pemphigus antibodies include periplakin. J Invest Dermatol. 1998;111:308-13.
Oursler JR, Labib RS, Ariss-Abdo L, Burke T, O'Keefe EJ, Anhalt GJ. Human autoantibodies against desmoplakins in paraneoplastic pemphigus. J Clin Invest. 1992;89:1775-82.
Schepens I, Jaunin F, Begre N, Läderach U, Marcus K, Hashimoto T, et al. The protease inhibitor alpha-2-macroglobulin-like-1 is the p 170 antigen recognized by paraneoplastic pemphigus autoantibodies in human. PLoS One. 2010;5:e12250.
Probst C, Schlumberger W, Stocker W, Recke A, Schmidt E, Hashimoto T, et al. Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. Clin Chim Acta. 2009;410:13-8.
Kiyokawa C, Ruhrberg C, Nie Z, Karashima T, Mori O, Nishikawa T, et al. Envoplakin and periplakin are components of the paraneoplastic pemphigus antigen complex. J Invest Dermatol. 1998;111:1236-8.
Kim SC, Kwon YD, Lee IJ, Chang SN, Lee TG. cDNA cloning of the 210-kDa paraneoplastic pemphigus antigen reveals that envoplakin is a component of the antigen complex. J Invest Dermatol. 1997;109:365-9.
Proby C, Fujii Y, Owaribe K, Nishikawa T, Amagai M. Human autoantibodies against HD1/plectin in paraneoplastic pemphigus. J Invest Dermatol. 1999;112:153-6.
Tsuchisaka A, Numata S, Teye K, Natsuaki Y, Kawakami T, Takeda Y, et al. Epiplakin is a paraneoplastic pemphigus autoantigen and related to bronchiolitis obliterans in Japanese patients. J Invest Dermatol. 2016;136:399-408.
Ishii N, Teye K, Fukuda S, Uehara R, Hachiya T, Koga H, et al. Anti-desmocollin autoantibodies in nonclassical pemphigus. Br J Dermatol. 2015;173:59-68.
Numata S, Teye K, Tsuruta D, Sogame R, Ishii N, Koga H, et al. Anti-alpha-2-macroglobulin-like-1 autoantibodies are detected frequently and may be pathogenic in paraneoplastic pemphigus. J Invest Dermatol. 2013;133:1785-93.
Landegren N, Ishii N, Aranda-Guillen M, Gunnarsson HI, Sardh F, Hallgren Å, et al. A gene-centric approach to biomarker discovery identifies transglutaminase 1 as an epidermal autoantigen. Proc Natl Acad Sci USA. 2021;118:e2100687118.
Amber KT. Paraneoplastic autoimmune multi-organ syndrome is a distinct entity from traditional pemphigus subtypes. Nat Rev Dis Primers. 2018;4:18012.
Amber KT, Valdebran M, Grando SA. Paraneoplastic autoimmune multiorgan syndrome (PAMS): beyond the single phenotype of paraneoplastic pemphigus. Autoimmun Rev. 2018;17:1002-10.
Kaplan I, Hodak E, Ackerman L, Mimouni D, Anhalt GJ, Calderon S. Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol. 2004;40:553-62.
Ohzono A, Sogame R, Li X, Teye K, Tsuchisaka A, Numata S, et al. Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. Br J Dermatol. 2015;173:1447-52.
Marzano AV, Grammatica A, Cozzani E, Terracina M, Berti E. Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia. Br J Dermatol. 2001;145:127-31.
Lim JM, Lee SE, Seo J, Kim DY, Hashimoto T, Kim SC. Paraneoplastic pemphigus associated with a malignant thymoma: a case of persistent and refractory oral ulcerations following thymectomy. Ann Dermatol. 2017;29:219-22.
Choi Y, Nam KH, Lee JB, Lee JY, Ihm CW, Lee SE, et al. Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus. J Dermatol. 2012;39:973-81.
Wang J, Bu DF, Li T, Zheng R, Zhang BX, Chen XX, et al. Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. Br J Dermatol. 2005;153:558-64.
Wang M, Li F, Wang X, Wang X, Wang R, Yang Y, et al. Features and risk factors for paraneoplastic autoimmune multiorgan syndrome in 145 Chinese patients. Acta Derm Venereol. 2020;100:adv00312.
Mimouni D, Anhalt GJ, Lazarova Z, Aho S, Kazerounian S, Kouba DJ, et al. Paraneoplastic pemphigus in children and adolescents. Br J Dermatol. 2002;147:725-32.
Mignogna MD, Fortuna G, Leuci S, Adamo D, Ruoppo E. Metastatic prostate cancer presenting as paraneoplastic pemphigus: a favourable clinical response to combined androgen blockade and conventional immunosuppressive therapy. Br J Dermatol. 2009;160:468-70.
Lepekhova A, Olisova O, Teplyuk N, Zolotenkov D, Allenova A. A rare association of paraneoplastic pemphigus with gastric signet cell ring carcinoma. Australas J Dermatol. 2019;60:e168-9.
Cho JH, Kim NJ, Ko SM, Kim C, Ahn HK, Yun J, et al. A case report of paraneoplastic pemphigus associated with esophageal squamous cell carcinoma. Cancer Res Treat. 2013;45:70-3.
Su Z, Liu G, Liu J, Fang T, Zeng Y, Zhang H, et al. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma: report of a case and review of literature. Int J Clin Exp Pathol. 2015;8:11983-94.
Marzano AV, Vezzoli P, Mariotti F, Boneschi V, Caputo R, Berti E. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma and Castleman disease. Br J Dermatol. 2005;153:214-5.
Anhalt GJ. Paraneoplastic pemphigus: the role of tumours and drugs. Br J Dermatol. 2001;144:1102-4.
Bazarbachi A, Bachelez H, Dehen L, Delmer A, Zittoun R, Dubertret L. Lethal paraneoplastic pemphigus following treatment of chronic lymphocytic leukaemia with fludarabine. Ann Oncol. 1995;6:730-1.
Higo T, Miyagaki T, Nakamura F, Shinohara A, Asano H, Abe H, et al. Paraneoplastic pemphigus occurring after bendamustine and rituximab therapy for relapsed follicular lymphoma. Ann Hematol. 2015;94:683-5.
Preisz K, Horvath A, Sardy M, Somlai B, Harsing J, Amagai M, et al. Exacerbation of paraneoplastic pemphigus by cyclophosphamide treatment: detection of novel autoantigens and bronchial autoantibodies. Br J Dermatol. 2004;150:1018-24.
Lee MS, Kossard S, Ho KK, Barnetson RS, Ravich RB. Paraneoplastic pemphigus triggered by radiotherapy. Australas J Dermatol. 1995;36:206-10.
Ostezan LB, Fabre VC, Caughman SW, Swerlick RA, Korman NJ, Callen JP. Paraneoplastic pemphigus in the absence of a known neoplasm. J Am Acad Dermatol. 1995;33:312-5.
Verrini A, Cannata G, Cozzani E, Terracini M, Parodi A, Rebora A. A patient with immunological features of paraneoplastic pemphigus in the absence of a detectable malignancy. Acta Derm Venereol. 2002;82:382-4.
Park GT, Lee JH, Yun SJ, Lee SC, Lee JB. Paraneoplastic pemphigus without an underlying neoplasm. Br J Dermatol. 2007;156:563-6.
Kim JH, Kim SC. Paraneoplastic pemphigus: paraneoplastic autoimmune disease of the skin and mucosa. Front Immunol. 2019;10:1259.
Anhalt GJ. Paraneoplastic pemphigus. J Investig Dermatol Symp Proc. 2004;9:29-33.
Leger S, Picard D, Ingen-Housz-Oro S, Arnault JP, Aubin F, Carsuzaa F, et al. Prognostic factors of paraneoplastic pemphigus. Arch Dermatol. 2012;148:1165-72.
Ouedraogo E, Gottlieb J, de Masson A, Lepelletier C, Jachiet M, Salle de Chou C, et al. Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults. J Am Acad Dermatol. 2019;80:1544-9.
Fang Y, Zhao L, Yan F, Cui X, Xia Y, Duren A. A critical role of surgery in the treatment for paraneoplastic pemphigus caused by localized Castleman's disease. Med Oncol. 2010;27:907-11.
Chorzelski T, Hashimoto T, Maciejewska B, Amagai M, Anhalt GJ, Jablonska S. Paraneoplastic pemphigus associated with Castleman tumor, myasthenia gravis and bronchiolitis obliterans. J Am Acad Dermatol. 1999;41:393-400.
Zhang J, Qiao QL, Chen XX, Liu P, Qiu JX, Zhao H, et al. Improved outcomes after complete resection of underlying tumors for patients with paraneoplastic pemphigus: a single-center experience of 22 cases. J Cancer Res Clin Oncol. 2011;137:229-34.
Jing L, Shan Z, Yongchu H, Xixue C, Xuejun Z. Successful treatment of a paraneoplastic pemphigus in a teenager using plasmapheresis, corticosteroids and tumour resection. Clin Exp Dermatol. 2011;36:752-4.
Rivollier C, Vaillant L, Machet MC, Martin L, Jan V, Huttenberger B, et al. Paraneoplastic pemphigus: a pustular form during chronic lymphoid leukemia. Ann Dermatol Venereol. 2001;128:644-8.
van der Waal RI, Pas HH, Anhalt GJ, Schulten EA, Jonkman MF, Nieboer C. Paraneoplastic pemphigus as the presenting symptom of a lymphoma of the tongue. Oral Oncol. 1998;34:567-70.
Simmons BJ, Adams JA, Cho-Vega JH, Tosti A. Paraneoplastic pemphigus with cicatricial nail involvement. Cutis. 2020;106:E4-6.
Rashid H, Lamberts A, Diercks GFH, Pas HH, Meijer JM, Bolling MC, et al. Oral lesions in autoimmune bullous diseases: an overview of clinical characteristics and diagnostic algorithm. Am J Clin Dermatol. 2019;20:847-61.
Decaux J, Ferreira I, Van Eeckhout P, Dachelet C, Magremanne M. Buccal paraneoplastic pemphigus multi-resistant: case report and review of diagnostic and therapeutic strategies. J Stomatol Oral Maxillofac Surg. 2018;119:506-9.
Gissi DB, Bernardi A, D'Andrea M, Montebugnoli L. Paraneoplastic pemphigus presenting with a single oral lesion. BMJ Case Rep. 2013;2013:bcr2012007771.
Broussard KC, Leung TG, Moradi A, Thorne JE, Fine JD. Autoimmune bullous diseases with skin and eye involvement: cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica. Clin Dermatol. 2016;34:205-13.
Tam PM, Cheng LL, Young AL, Lam PT. Paraneoplastic pemphigus: an uncommon cause of chronic cicatrising conjunctivitis. BMJ Case Rep. 2009;2009:bcr1220081306.
Beele H, Claerhout I, Kestelyn P, Dierckxens L, Naeyaert JM, De Laey JJ. Bilateral corneal melting in a patient with paraneoplastic pemphigus. Dermatology. 2001;202:147-50.
Piscopo R, Romano M, Maria AD, Vinciguerra R, Vinciguerra P. Ocular onset of paraneoplastic pemphigus presenting as hyperemic conjunctivitis and massive bilateral eyelid ulceration: a case report and literature review. Ocul Immunol Inflamm. 2018;26:265-8.
Meyers SJ, Varley GA, Meisler DM, Camisa C, Wander AH. Conjunctival involvement in paraneoplastic pemphigus. Am J Ophthalmol. 1992;114:621-4.
Han SP, Fu LS, Chen LJ. Masked pemphigus among pediatric patients with Castleman's disease. Int J Rheum Dis. 2019;22:121-31.
Takahashi M, Shimatsu Y, Kazama T, Kimura K, Otsuka T, Hashimoto T. Paraneoplastic pemphigus associated with bronchiolitis obliterans. Chest. 2000;117:603-7.
Nousari HC, Deterding R, Wojtczack H, Aho S, Uitto J, Hashimoto T, et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Engl J Med. 1999;340:1406-10.
Maldonado F, Pittelkow MR, Ryu JH. Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome. Respirology. 2009;14:129-33.
Sano Y, Kikuchi Y, Morita S, Oka T, Saito K, Takeda N, et al. Paraneoplastic pemphigus and fatal bronchiolitis obliterans associated with Castleman disease: report of an autopsy case. Pathol Int. 2021;71:170-2.
Chorzelski TP, Hashimoto T, Amagai M, Sysa-Jedrzejowska A, Choczaj A, Nousari HC, et al. Paraneoplastic pemphigus with cutaneous and serological features of pemphigus foliaceus. Br J Dermatol. 1999;141:357-9.
Kanaoka M, Matsukura S, Ishikawa H, Matsuura M, Ishii N, Hashimoto T, et al. Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and appearance of anti-BP180 antibodies in the late stage of the disease. J Dermatol. 2014;41:628-30.
Hata T, Nishimoto S, Nagao K, Takahashi H, Yoshida K, Ohyama M, et al. Ectopic expression of epidermal antigens renders the lung a target organ in paraneoplastic pemphigus. J Immunol. 2013;191:83-90.
Miida H, Kazama T, Inomata N, Takizawa H, Iwafuchi M, Ito M, et al. Severe gastrointestinal involvement in paraneoplastic pemphigus. Eur J Dermatol. 2006;16:420-2.
Odani K, Itoh A, Yanagita S, Kaneko Y, Tachibana M, Hashimoto T, et al. Paraneoplastic pemphigus involving the respiratory and gastrointestinal mucosae. Case Rep Pathol. 2020;2020:7350759.
Frew JW, Murrell DF. Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. Dermatol Clin. 2011;29:419-25, viii.
Wang R, Li J, Wang M, Hao H, Chen X, Li R, et al. Prevalence of myasthenia gravis and associated autoantibodies in paraneoplastic pemphigus and their correlations with symptoms and prognosis. Br J Dermatol. 2015;172:968-75.
Winkler DT, Strnad P, Meier ML, Roten S, Went P, Lyrer P, et al. Myasthenia gravis, paraneoplastic pemphigus and thymoma, a rare triade. J Neurol. 2007;254:1601-3.
Horn TD, Anhalt GJ. Histologic features of paraneoplastic pemphigus. Arch Dermatol. 1992;128:1091-5.
Joly P, Richard C, Gilbert D, Courville P, Chosidow O, Roujeau JC, et al. Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol. 2000;43:619-26.
Rybojad M, Leblanc T, Flageul B, Bernard P, Morel P, Schaison G, et al. Paraneoplastic pemphigus in a child with a T-cell lymphoblastic lymphoma. Br J Dermatol. 1993;128:418-22.
Fournet M, Roblot P, Levillain P, Guillet G, Machet L, Misery L. Paraneoplastic pemphigus: retrospective study of a case series. Ann Dermatol Venereol. 2018;145:564-71.
Cummins DL, Mimouni D, Tzu J, Owens N, Anhalt GJ, Meyerle JH. Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. J Am Acad Dermatol. 2007;56:153-9.
Poot AM, Siland J, Jonkman MF, Pas HH, Diercks GF. Direct and indirect immunofluorescence staining patterns in the diagnosis of paraneoplastic pemphigus. Br J Dermatol. 2016;174:912-5.
Kwatra SG, Boozalis E, Pasieka H, Anhalt GJ. Decreased recognition of paraneoplastic pemphigus in patients previously treated with anti-CD 20 monoclonal antibodies. Br J Dermatol. 2019;180:1238-9.
Wang X, Chen T, Zhao J, Peng Y, Chen X, Tu P, et al. Extremities of the N-terminus of envoplakin and C-terminus of its linker subdomain are major epitopes of paraneoplastic pemphigus. J Dermatol Sci. 2016;84:24-9.
Helou J, Allbritton J, Anhalt GJ. Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol. 1995;32:441-7.
Poot AM, Diercks GF, Kramer D, Schepens I, Klunder G, Hashimoto T, et al. Laboratory diagnosis of paraneoplastic pemphigus. Br J Dermatol. 2013;169:1016-24.
Brandt O, Rafei D, Podstawa E, Niedermeier A, Jonkman MF, Terra JB, et al. Differential IgG recognition of desmoglein 3 by paraneoplastic pemphigus and pemphigus vulgaris sera. J Invest Dermatol. 2012;132:1738-41.
Futei Y, Amagai M, Hashimoto T, Nishikawa T. Conformational epitope mapping and IgG subclass distribution of desmoglein 3 in paraneoplastic pemphigus. J Am Acad Dermatol. 2003;49:1023-8.
Ohyama M, Amagai M, Hashimoto T, Nousari HC, Anhalt GJ, Nishikawa T. Clinical phenotype and anti-desmoglein autoantibody profile in paraneoplastic pemphigus. J Am Acad Dermatol. 2001;44:593-8.
Tsuchisaka A, Kawano H, Yasukochi A, Teye K, Ishii N, Koga H, et al. Immunological and statistical studies of anti-BP180 antibodies in paraneoplastic pemphigus. J Invest Dermatol. 2014;134:2283-7.
Bazzini C, Begre N, Favre B, Hashimoto T, Hertl M, Schlapbach C, et al. Detection of autoantibodies against alpha-2-macroglobulin-like 1 in paraneoplastic pemphigus sera utilizing novel green fluorescent protein-based immunoassays. J Dermatol Sci. 2020;98:173-8.
Hashimoto T, Amagai M, Ning W, Nishikawa T, Karashima T, Mori O, et al. Novel non-radioisotope immunoprecipitation studies indicate involvement of pemphigus vulgaris antigen in paraneoplastic pemphigus. J Dermatol Sci. 1998;17:132-9.
Witte M, Zillikens D, Schmidt E. Diagnosis of autoimmune blistering diseases. Front Med (Lausanne). 2018;5:296.
van Beek N, Zillikens D, Schmidt E. Diagnosis of autoimmune bullous diseases. J Dtsch Dermatol Ges. 2018;16:1077-91.
Camisa C, Helm TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol. 1993;129:883-6.
Billet SE, Grando SA, Pittelkow MR. Paraneoplastic autoimmune multiorgan syndrome: review of the literature and support for a cytotoxic role in pathogenesis. Autoimmunity. 2006;39:617-30.
Karlhofer FM, Hashimoto T, Slupetzky K, Kiss M, Liu Y, Amagai M, et al. 230-kDa and 190-kDa proteins in addition to desmoglein 1 as immunological targets in a subset of pemphigus foliaceus with a combined cell-surface and basement membrane zone immune staining pattern. Exp Dermatol. 2003;12:646-54.
Cozzani E, Dal Bello MG, Mastrogiacomo A, Drosera M, Parodi A. Antidesmoplakin antibodies in pemphigus vulgaris. Br J Dermatol. 2006;154:624-8.
Cozzani E, Di Zenzo G, Calabresi V, Caproni M, Schena D, Quaglino P, et al. Anti-desmoplakin antibodies in erythema multiforme and Stevens-Johnson syndrome sera: pathogenic or epiphenomenon? Eur J Dermatol. 2011;21:32-6.
Streifel AM, Wessman LL, Schultz BJ, Miller D, Pearson DR. Refractory mucositis associated with underlying follicular dendritic cell sarcoma of the thymus: paraneoplastic pemphigus versus malignancy-exacerbated pemphigus vulgaris. JAAD Case Rep. 2019;5:933-6.
Joly P, Horvath B, Patsatsi A, Uzun S, Bech R, Beissert S, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2020;34:1900-13.
Foedinger D, Sterniczky B, Elbe A, Anhalt G, Wolff K, Rappersberger K. Autoantibodies against desmoplakin I and II define a subset of patients with erythema multiforme major. J Invest Dermatol. 1996;106:1012-6.
Egan CA, Lazarova Z, Darling TN, Yee C, Cote T, Yancey KB. Anti-epiligrin cicatricial pemphigoid and relative risk for cancer. Lancet. 2001;357:1850-1.
Goletz S, Probst C, Komorowski L, Schlumberger W, Fechner K, van Beek N, et al. A sensitive and specific assay for the serological diagnosis of antilaminin 332 mucous membrane pemphigoid. Br J Dermatol. 2019;180:149-56.
Rashid H, Meijer JM, Bolling MC, Diercks GFH, Pas HH, Horvath B. Insights into clinical and diagnostic findings as well as treatment responses in patients with mucous membrane pemphigoid: a retrospective cohort study. J Am Acad Dermatol. 2021;87:48-55.
Schmidt E, Rashid H, Marzano AV, Lamberts A, di Zenzo G, Diercks GFH, et al. European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology-part II. J Eur Acad Dermatol Venereol. 2021;35:1926-48.
Du G, Patzelt S, van Beek N, Schmidt E. Mucous membrane pemphigoid. Autoimmun Rev. 2022;21:103036.
Chiorean R, Danescu S, Virtic O, Mustafa MB, Baican A, Lischka A, et al. Molecular diagnosis of anti-laminin 332 (epiligrin) mucous membrane pemphigoid. Orphanet J Rare Dis. 2018;13:111.
Yamada H, Nobeyama Y, Matsuo K, Ishiji T, Takeuchi T, Fukuda S, et al. A case of paraneoplastic pemphigus associated with triple malignancies in combination with antilaminin-332 mucous membrane pemphigoid. Br J Dermatol. 2012;166:230-1.
Okahashi K, Oiso N, Ishii N, Uchida S, Yanagihara S, Sano A, et al. Paraneoplastic pemphigus presenting lichen planus-like lesions. J Dermatol. 2019;46:e140-2.
Coelho S, Reis JP, Tellechea O, Figueiredo A, Black M. Paraneoplastic pemphigus with clinical features of lichen planus associated with low-grade B cell lymphoma. Int J Dermatol. 2005;44:366-71.
Bowen GM, Peters NT, Fivenson DP, Su LD, Nousari HC, Anhalt GJ, et al. Lichenoid dermatitis in paraneoplastic pemphigus: a pathogenic trigger of epitope spreading? Arch Dermatol. 2000;136:652-6.
Tey HL, Tang MB. A case of paraneoplastic pemphigus associated with Castleman's disease presenting as erosive lichen planus. Clin Exp Dermatol. 2009;34:e754-6.
Yokokura H, Demitsu T, Kakurai M, Umemoto N, Azuma R, Yamada T, et al. Paraneoplastic pemphigus mimicking erosive mucosal lichen planus associated with primary hepatocellular carcinoma. J Dermatol. 2006;33:842-5.
Passeron T, Bahadoran P, Lacour JP, Perrin C, Gilbert D, Benzaken S, et al. Paraneoplastic pemphigus presenting as erosive lichen planus. Br J Dermatol. 1999;140:552-3.
Wang M, Wang X, Chen T, Zhao J, Peng Y, Chen X, et al. Erosive oral lichen planus as a sign of paraneoplastic pemphigus. J Dermatol. 2016;43:983.
Kelly S, Schifter M, Fulcher DA, Lin MW. Paraneoplastic pemphigus: two cases of intra-abdominal malignancy presenting solely as treatment refractory oral ulceration. J Dermatol. 2015;42:300-4.
Chou S, Zhao C, Hwang SJE, Fernandez-Penas P. PD-1 inhibitor-associated lichenoid inflammation with incidental suprabasilar acantholysis or vesiculation-report of 4 cases. J Cutan Pathol. 2017;44:851-6.
Malphettes M, Gerard L, Galicier L, Boutboul D, Asli B, Szalat R, et al. Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. Clin Infect Dis. 2015;61:e13-9.
Hayanga AJ, Lee TM, Pannucci CJ, Knipp BS, Olsen SH, Wang SC, et al. Paraneoplastic pemphigus in a burn intensive care unit: case report and review of the literature. J Burn Care Res. 2010;31:826-9.
Kokubu H, Nishikawa J, Kato T, Mukaisho KI, Hayashi D, Tateishi C, et al. Paraneoplastic pemphigus mimicking toxic epidermal necrolysis associated with follicular lymphoma: possible pathological role of CD8 T cells. Acta Derm Venereol. 2020;100:adv00204.
Oiso N, Yanagihara S, Tateishi C, Ishii N, Hashimoto T, Tsuruta D, et al. Case of antiplakin dermatosis. JAMA Dermatol. 2021;157:602-3.
Mahler V, Antoni C, Anhalt GJ, Koch HJ, Peters KP, Manger B, et al. Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and systemic lupus erythematosus in a patient with non-Hodgkin's lymphoma. Dermatology. 1998;197:78-83.
Reich K, Brinck U, Letschert M, Blaschke V, Dames K, Braess J, et al. Graft-versus-host disease-like immunophenotype and apoptotic keratinocyte death in paraneoplastic pemphigus. Br J Dermatol. 1999;141:739-46.
Maruta CW, Miyamoto D, Aoki V, Carvalho RGR, Cunha BM, Santi CG. Paraneoplastic pemphigus: a clinical, laboratorial, and therapeutic overview. An Bras Dermatol. 2019;94:388-98.
Didona D, Fania L, Didona B, Eming R, Hertl M, Di Zenzo G. Paraneoplastic dermatoses: a brief general review and an extensive analysis of paraneoplastic pemphigus and paraneoplastic dermatomyositis. Int J Mol Sci. 2020;21:2178.
Grando SA. New approaches to the treatment of pemphigus. J Investig Dermatol Symp Proc. 2004;9:84-91.
Paolino G, Didona D, Magliulo G, Iannella G, Didona B, Mercuri S, et al. Paraneoplastic pemphigus: insight into the autoimmune pathogenesis, clinical features and therapy. Int J Mol Sci. 2017;18:2532.
Riera-Monroig J, Iranzo P, Ishii N, Hashimoto T, Mascaro JM Jr. Persistence of antienvoplakin and antiperiplakin antibodies in a patient with paraneoplastic pemphigus 20 years after remission. Br J Dermatol. 2020;182:797-8.
Joly P, Sin C. Pemphigus: a review. Ann Dermatol Venereol. 2011;138:182-200.
Borradori L, Lombardi T, Samson J, Girardet C, Saurat JH, Hugli A. Anti-CD20 monoclonal antibody (rituximab) for refractory erosive stomatitis secondary to CD20+ follicular lymphoma-associated paraneoplastic pemphigus. Arch Dermatol. 2001;137:269-72.
Barnadas M, Roe E, Brunet S, Garcia P, Bergua P, Pimentel L, et al. Therapy of paraneoplastic pemphigus with rituximab: a case report and review of literature. J Eur Acad Dermatol Venereol. 2006;20:69-74.
Frew JW, Murrell DF. Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome). Dermatol Clin. 2011;29:607-12.
Bech R, Baumgartner-Nielsen J, Peterslund NA, Steiniche T, Deleuran M, d'Amore F. Alemtuzumab is effective against severe chronic lymphocytic leukaemia-associated paraneoplastic pemphigus. Br J Dermatol. 2013;169:469-72.
Anhalt G. Paraneoplastic pemphigus: diagnosis. Clinical associations and management. International Pemphigus Pemphigoid Foundation Fifteenth Annual Patient/Doctor Meeting; Boston (MA), May 18-20, 2012.
Wang J, Zhang Y, Pan M. Thalidomide as a potential adjuvant treatment for paraneoplastic pemphigus: a single-center experience. Dermatol Ther. 2020;33:e14353.
Chen W, Zhao L, Guo L, Zhao L, Niu H, Lian H, et al. Clinical and pathological features of bronchiolitis obliterans requiring lung transplantation in paraneoplastic pemphigus associated with Castleman disease. Clin Respir J. 2022;16:173-81.