Paraneoplastic Pemphigus Differential Diagnoses

1. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med. 1990 Dec 20. 323(25):1729-35. [QxMD MEDLINE Link].

2. Anhalt GJ. Paraneoplastic pemphigus. J Investig Dermatol Symp Proc. 2004 Jan. 9(1):29-33. [QxMD MEDLINE Link].

3. Zimmermann J, Bahmer F, Rose C, Zillikens D, Schmidt E. Clinical and immunopathological spectrum of paraneoplastic pemphigus. J Dtsch Dermatol Ges. 2010 Aug. 8 (8):598-606. [QxMD MEDLINE Link].

4. Martel P, Loiseau P, Joly P, Busson M, Lepage V, Mouquet H, et al. Paraneoplastic pemphigus is associated with the DRB1*03 allele. J Autoimmun. 2003 Feb. 20 (1):91-5. [QxMD MEDLINE Link].

5. Liu Q, Bu DF, Li D, Zhu XJ. Genotyping of HLA-I and HLA-II alleles in Chinese patients with paraneoplastic pemphigus. Br J Dermatol. 2008 Mar. 158 (3):587-91. [QxMD MEDLINE Link].

6. Czernik A, Camilleri M, Pittelkow MR, Grando SA. Paraneoplastic autoimmune multiorgan syndrome: 20 years after. Int J Dermatol. 2011 Aug. 50 (8):905-14. [QxMD MEDLINE Link].

7. Qian SX, Li JY, Hong M, Xu W, Qiu HX. Nonhematological autoimmunity (glomerulosclerosis, paraneoplastic pemphigus and paraneoplastic neurological syndrome) in a patient with chronic lymphocytic leukemia: Diagnosis, prognosis and management. Leuk Res. 2009 Mar. 33 (3):500-5. [QxMD MEDLINE Link].

8. 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 Feb. 136 (2):399-408. [QxMD MEDLINE Link].

9. Taintor AR, Leiferman KM, Hashimoto T, Ishii N, Zone JJ, Hull CM. A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia. J Am Acad Dermatol. 2007 May. 56 (5 Suppl):S73-6. [QxMD MEDLINE Link].

10. 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 Jan. 56(1):153-9. [QxMD MEDLINE Link].

11. Lim JM, Kim JH, Hashimoto T, Kim SC. Lichenoid paraneoplastic pemphigus associated with follicular lymphoma without detectable autoantibodies. Clin Exp Dermatol. 2018 Jul. 43 (5):613-615. [QxMD MEDLINE Link].

12. Moreno-Artero E, Querol-Cisneros E, Rodríguez-Garijo N, Tomás-Velázquez A, Idoate MA, Ishii N, et al. Paraneoplastic pemphigus without detectable anti-plakin antibodies in a patient with non-Hodgkin lymphoma. Ann Hematol. 2018 Mar. 97 (3):543-544. [QxMD MEDLINE Link].

13. Bennett DD, Busick TL. Delayed detection of autoantibodies in paraneoplastic pemphigus. J Am Acad Dermatol. 2007 Dec. 57(6):1094-5. [QxMD MEDLINE Link].

14. 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 Jan 15. [QxMD MEDLINE Link].

15. Camisa C, Helm TN. Paraneoplastic pemphigus is a distinct neoplasia-induced autoimmune disease. Arch Dermatol. 1993 Jul. 129(7):883-6. [QxMD MEDLINE Link].

16. Yong AA, Tey HL. Paraneoplastic pemphigus. Australas J Dermatol. 2013 Nov. 54(4):241-50. [QxMD MEDLINE Link].

17. Proby C, Fujii Y, Owaribe K, Nishikawa T, Amagai M. Human autoantibodies against HD1/plectin in paraneoplastic pemphigus. J Invest Dermatol. 1999 Feb. 112(2):153-6. [QxMD MEDLINE Link].

18. Poot AM, Diercks GF, Kramer D, Schepens I, Klunder G, Hashimoto T. Laboratory diagnosis of paraneoplastic pemphigus. Br J Dermatol. 2013 Nov. 169(5):1016-24. [QxMD MEDLINE Link].

19. Leger S, Picard D, Ingen-Housz-Oro S, Arnault JP, Aubin F, Carsuzaa F. Prognostic factors of paraneoplastic pemphigus. Arch Dermatol. 2012 Oct. 148(10):1165-72. [QxMD MEDLINE Link].

20. Hertzberg MS, Schifter M, Sullivan J, Stapleton K. Paraneoplastic pemphigus in two patients with B-cell non-Hodgkin's lymphoma: significant responses to cyclophosphamide and prednisolone. Am J Hematol. 2000 Feb. 63(2):105-6. [QxMD MEDLINE Link].

21. Martinez De Pablo MI, Iranzo P, Mascaro JM, Llambrich A, Baradad M, Herrero C. Paraneoplastic pemphigus associated with non-Hodgkin B-cell lymphoma and good response to prednisone. Acta Derm Venereol. 2005. 85(3):233-5. [QxMD MEDLINE Link].

22. Tilakaratne W, Dissanayake M. Paraneoplastic pemphigus: a case report and review of literature. Oral Dis. 2005 Sep. 11(5):326-9. [QxMD MEDLINE Link].

23. Hung IJ, Lin JJ, Yang CP, Hsueh C. Paraneoplastic syndrome and intrathoracic Castleman disease. Pediatr Blood Cancer. 2006 Oct 15. 47(5):616-20. [QxMD MEDLINE Link].

24. 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 Jul. 153(1):214-5. [QxMD MEDLINE Link].

25. Wang J, Zhu X, Li R, et al. Paraneoplastic pemphigus associated with Castleman tumor: a commonly reported subtype of paraneoplastic pemphigus in China. Arch Dermatol. 2005 Oct. 141(10):1285-93. [QxMD MEDLINE Link].

26. Koch LH, Layton CJ, Pilichowska M, Stadecker MJ, Barak O. Paraneoplastic Pemphigus and Castleman's Disease in the Setting of Herpes Simplex Virus Infection. Pediatr Dermatol. 2011 Oct 20. [QxMD MEDLINE Link].

27. Perera GK, Devereux S, Mufti G, Salisbury J, Creamer D. PNP with Waldenström's macroglobulinaemia. Clin Exp Dermatol. 2005 Jan. 30(1):27-9. [QxMD MEDLINE Link].

28. Wang J, Bu DF, Li T, et al. Autoantibody production from a thymoma and a follicular dendritic cell sarcoma associated with paraneoplastic pemphigus. Br J Dermatol. 2005 Sep. 153(3):558-64. [QxMD MEDLINE Link].

29. Anhalt GJ. Paraneoplastic pemphigus: the role of tumours and drugs. Br J Dermatol. 2001 Jun. 144 (6):1102-4. [QxMD MEDLINE Link].

30. Tull T, Papineni P, Cook C, Mee J, Bower M, Morar N. Paraneoplastic pemphigus in patient infected with the human immunodeficiency virus. Clin Exp Dermatol. 2014 Jan. 39(1):83-5. [QxMD MEDLINE Link].

31. Eccersley LR, Hoffbrand AV, Rustin MH, McNamara CJ. Paraneoplastic pemphigus associated with systemic mastocytosis. Am J Hematol. 2009 May 27. [QxMD MEDLINE Link].

32. Kennedy NA, Dawe S. Atypical paraneoplastic pemphigus secondary to endometrial carcinoma with no mucosal involvement. Clin Exp Dermatol. 2009 Jul. 34(5):e130-3. [QxMD MEDLINE Link].

33. Kakurai M, Demitsu T, Iida E, et al. Coexistence of paraneoplastic pemphigus and bullous pemphigoid. J Eur Acad Dermatol Venereol. 2009 Aug. 23(8):962-4. [QxMD MEDLINE Link].

34. Ishii N, Hashimoto T. A case of paraneoplastic pemphigus who shifted to pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2008 Mar. 22(3):374-5. [QxMD MEDLINE Link].

35. 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 May. 44(5):366-71. [QxMD MEDLINE Link].

36. Zhu X, Zhang B. Paraneoplastic pemphigus. J Dermatol. 2007 Aug. 34(8):503-11. [QxMD MEDLINE Link].

37. Laforest C, Huilgol SC, Casson R, Selva D, Leibovitch I. Autoimmune bullous diseases: ocular manifestations and management. Drugs. 2005. 65(13):1767-79. [QxMD MEDLINE Link].

38. 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 Apr. 172 (4):968-75. [QxMD MEDLINE Link].

39. Paolino G, Didona D, Magliulo G, Iannella G, Didona B, Mercuri SR, et al. Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy. Int J Mol Sci. 2017 Nov 26. 18 (12):[QxMD MEDLINE Link].

40. 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 Oct. 44 (10):851-856. [QxMD MEDLINE Link].

41. Chen WS, Tetzlaff MT, Diwan H, Jahan-Tigh R, Diab A, Nelson K, et al. Suprabasal acantholytic dermatologic toxicities associated checkpoint inhibitor therapy: A spectrum of immune reactions from paraneoplastic pemphigus-like to Grover-like lesions. J Cutan Pathol. 2018 Oct. 45 (10):764-773. [QxMD MEDLINE Link].

42. Helou J, Allbritton J, Anhalt GJ. Accuracy of indirect immunofluorescence testing in the diagnosis of paraneoplastic pemphigus. J Am Acad Dermatol. 1995 Mar. 32(3):441-7. [QxMD MEDLINE Link].

43. Huang Y, Li J, Zhu X. Detection of anti-envoplakin and anti-periplakin autoantibodies by ELISA in patients with paraneoplastic pemphigus. Arch Dermatol Res. 2009 Nov. 301(10):703-9. [QxMD MEDLINE Link].

44. Mouquet H, Drenovska K, Lartigue A, et al. Detection and characterization of anti-envoplakin linker autoantibodies in paraneoplastic pemphigus using specific bead-based assay. Clin Immunol. 2008 Nov. 129(2):304-12. [QxMD MEDLINE Link].

45. Probst C, Schlumberger W, Stocker W, et al. Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. Clin Chim Acta. 2009 Sep 6. [QxMD MEDLINE Link].

46. Barnadas MA, Curell R, Alomar A, Gelpi C. Paraneoplastic pemphigus with negative direct immunofluorescence in epidermis or mucosa but positive findings in adnexal structures. J Cutan Pathol. 2009 Jan. 36(1):34-8. [QxMD MEDLINE Link].

47. Khudhur AS, Di Zenzo G, Carrozzo M. Oral lichenoid tissue reactions: diagnosis and classification. Expert Rev Mol Diagn. 2014 Mar. 14 (2):169-84. [QxMD MEDLINE Link].

48. Frew JW, Murrell DF. Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome). Dermatol Clin. 2011 Oct. 29(4):607-12. [QxMD MEDLINE Link].

49. Barnadas M, Roe E, Brunet S, et al. Therapy of paraneoplastic pemphigus with Rituximab: a case report and review of literature. J Eur Acad Dermatol Venereol. 2006 Jan. 20(1):69-74. [QxMD MEDLINE Link].

50. Peterson JD, Chan LS. Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007. Ther Clin Risk Manag. 2009 Feb. 5(1):1-7. [QxMD MEDLINE Link]. [Full Text].

51. Hohwy T, Bang K, Steiniche T, Peterslund NA, d'Amore F. Alemtuzumab-induced remission of both severe paraneoplastic pemphigus and leukaemic bone marrow infiltration in a case of treatment-resistant B-cell chronic lymphocytic leukaemia. Eur J Haematol. 2004 Sep. 73 (3):206-9. [QxMD MEDLINE Link].

52. Frew JW, Murrell DF. Paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome): clinical presentations and pathogenesis. Dermatol Clin. 2011 Jul. 29(3):419-25, viii. [QxMD MEDLINE Link].

Lynne J Goldberg, MD Professor, Departments of Dermatology and Pathology, Boston University School of Medicine

Lynne J Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, International Society of Dermatopathology, Massachusetts Academy of Dermatology, New England Dermatological Society, North American Hair Research Society, Phi Beta Kappa, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Nauman Nisar, MD Dermatopathologist, Mercy Medical Center, Sioux City, IA

Nauman Nisar, MD is a member of the following medical societies: College of American Pathologists

Disclosure: Nothing to disclose.

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Genentech as a consultant for a potential study on a drug that has yet to be approved for any use<br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts; I served on a safety monitoring committee for Principia Biopharma (ended 9-2021) for: Stocks held in various trust accounts: Allergen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen; Gilead.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Ponciano D Cruz, Jr, MD Professor and Vice-Chair, Paul R Bergstresser Chair, Department of Dermatology, University of Texas Southwestern Medical Center

Ponciano D Cruz, Jr, MD is a member of the following medical societies: Texas Medical Association

Disclosure: Received consulting fee from RCTS for independent contractor; Received honoraria from Mary Kay Cosmetics for consulting; Received grant/research funds from Galderma for principal investigator.

• Print this section
• Print the entire contents of
• Print the entire contents of article