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Monoclonal gammopathy in the setting of Pyoderma gangrenosum

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Abstract

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.

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Data availability

No datasets were generated or analysed during the current study.

Code availability

Not applicable

Abbreviations

Ig:

Immunoglobulin

MDS:

Myelodysplastic syndromes

MG:

Monoclonal gammopathy

MGUS:

Monoclonal gammopathy of uncertain significance

PG:

Pyoderma gangrenosum

References

  1. Brunsting LA, Goeckerman WH, O’Leary PA (1930) Pyoderma (echthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol Syphilol 22(4):655–680

    Article  Google Scholar 

  2. Birnkrant MJ, Papadopoulos AJ, Schwartz RA, Lambert WC (2003) Pyoderma Gangrenosum, acne conglobata, and IgA gammopathy. Int J Dermatol 42(3):213–216

    Article  PubMed  Google Scholar 

  3. Montagnon CM, Fracica EA, Patel AA, Camilleri MJ, Murad MH, Dingli D et al (2020) Pyoderma Gangrenosum in hematologic malignancies: a systematic review. J Am Acad Dermatol 82(6):1346–1359

    Article  PubMed  Google Scholar 

  4. Hou YL, Lee CH (2022) Pyoderma Gangrenosum. JAMA Dermatol 158(2):202

    Article  PubMed  Google Scholar 

  5. Alavi A, French LE, Davis MD, Brassard A, Kirsner RS (2017) Pyoderma Gangrenosum: an update on pathophysiology, diagnosis and treatment. Am J Clin Dermatol 18(3):355–372

    Article  PubMed  Google Scholar 

  6. Alavi A, Sajic D, Cerci FB, Ghazarian D, Rosenbach M, Jorizzo J (2014) Neutrophilic dermatoses: an update. Am J Clin Dermatol 15(5):413–423

    Article  PubMed  Google Scholar 

  7. Katzmann J, Kyle RA, Lust J, Snyder M, Dispenzieri A (2013) Immunoglobulins and Laboratory Recognition of Monoclonal Proteins. In: Wiernik PH, Goldman JM, Dutcher JP, Kyle RA (eds) Neoplastic diseases of the blood. Springer New York, New York, NY, pp 565–588

    Chapter  Google Scholar 

  8. Mateos MV, Gonzalez-Calle V (2017) Smoldering multiple myeloma: who and when to treat. Clin Lymphoma Myeloma Leuk 17(11):716–722

    Article  PubMed  Google Scholar 

  9. Kaseb H, Annamaraju P, Babiker HM (2023) Monoclonal gammopathy of undetermined significance. StatPearls. Treasure Island (FL)

  10. Wadhera RK, Rajkumar SV (2010) Prevalence of monoclonal gammopathy of undetermined significance: a systematic review. Mayo Clin Proc 85(10):933–942

    Article  PubMed  PubMed Central  Google Scholar 

  11. Powell FC, Schroeter AL, Su WP, Perry HO (1983) Pyoderma gangrenosum and monoclonal gammopathy. Arch Dermatol 119(6):468–472

    Article  CAS  PubMed  Google Scholar 

  12. Daoud MS, Lust JA, Kyle RA, Pittelkow MR (1999) Monoclonal gammopathies and associated skin disorders. J Am Acad Dermatol 40(4):507–535 quiz 36 – 8

    Article  CAS  PubMed  Google Scholar 

  13. Chave TA, Hutchinson PE (2001) Pyoderma Gangrenosum, subcorneal pustular dermatosis, IgA paraproteinaemia and IgG antiepithelial antibodies. Br J Dermatol 145(5):852–854

    Article  CAS  PubMed  Google Scholar 

  14. Szalat R, Monsel G, Le Goff W, Battistella M, Bengouffa D, Schlageter MH et al (2015) The spectrum of neutrophilic dermatoses associated with monoclonal gammopathy: Association with IgA isotype and inflammatory profile. J Am Acad Dermatol 73(5):809–820

    Article  CAS  PubMed  Google Scholar 

  15. Lipsker D (2017) Monoclonal gammopathy of cutaneous significance: review of a relevant concept. J Eur Acad Dermatol Venereol 31(1):45–52

    Article  CAS  PubMed  Google Scholar 

  16. Claveau JS, Wetter DA, Kumar S (2022) Cutaneous manifestations of monoclonal gammopathy. Blood Cancer J 12(4):58

    Article  PubMed  PubMed Central  Google Scholar 

  17. Hoffman KP, Shearer S, Chung C, Kaffenberger JA, Kaffenberger BH (2020) Clinical and therapeutic overlap of pyoderma gangrenosum, cutaneous small vessel vasculitis, and immunoglobulin A. Int J Dermatol 59(8):e286–e8

    Article  PubMed  Google Scholar 

  18. Machan A, Azendour H, Frikh R, Hjira N, Boui M (2020) The dilemma of treating pyoderma gangrenosum associated with monoclonal gammopathy of undetermined significance. Dermatol Online J. 26(5)

  19. Kyle RA, Rajkumar SV (2006) Monoclonal gammopathy of undetermined significance. Br J Haematol 134(6):573–589

    Article  CAS  PubMed  Google Scholar 

  20. Weinhold N, Johnson DC, Rawstron AC, Forsti A, Doughty C, Vijayakrishnan J et al (2014) Inherited genetic susceptibility to monoclonal gammopathy of unknown significance. Blood 123(16):2513–2517 quiz 93

    Article  CAS  PubMed  Google Scholar 

  21. Ma L, Xu S, Qu J, Hou J, Wang Y, Wen L et al (2019) Monoclonal gammopathy of undetermined significance in Chinese population: a prospective epidemiological study. Hematol Oncol 37(1):75–79

    Article  CAS  PubMed  Google Scholar 

  22. Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Offord JR et al (2006) Prevalence of monoclonal gammopathy of undetermined significance. N Engl J Med 354(13):1362–1369

    Article  CAS  PubMed  Google Scholar 

  23. Lecluse Y, Comby E, Mariotte D, Tual S, Le Mauff B, Lebailly P et al (2016) Prevalence of monoclonal gammopathy of undetermined significance (MGUS) among farmers involved in open field farming and/or cattle breading in France. Leuk Lymphoma 57(7):1727–1730

    Article  PubMed  Google Scholar 

  24. Cabrera Q, Macro M, Hebert B, Cornet E, Collignon A, Troussard X (2014) Epidemiology of Monoclonal Gammopathy of undetermined significance (MGUS): the experience from the specialized registry of hematologic malignancies of Basse-Normandie (France). Cancer Epidemiol 38(4):354–356

    Article  PubMed  Google Scholar 

  25. Iwanaga M, Tagawa M, Tsukasaki K, Kamihira S, Tomonaga M (2007) Prevalence of monoclonal gammopathy of undetermined significance: study of 52,802 persons in Nagasaki City, Japan. Mayo Clin Proc 82(12):1474–1479

    Article  PubMed  Google Scholar 

  26. Kyle RA, Rajkumar SV (2005) Monoclonal gammopathies of undetermined significance. Best Pract Res Clin Haematol 18(4):689–707

    Article  CAS  PubMed  Google Scholar 

  27. Anagnostopoulos A, Evangelopoulou A, Sotou D, Gika D, Mitsibounas D, Dimopoulos MA (2002) Incidence and evolution of monoclonal gammopathy of undetermined significance (MGUS) in Greece. Ann Hematol 81(7):357–361

    Article  CAS  PubMed  Google Scholar 

  28. Cohen HJ, Crawford J, Rao MK, Pieper CF, Currie MS (1998) Racial differences in the prevalence of monoclonal gammopathy in a community-based sample of the elderly. Am J Med 104(5):439–444

    Article  CAS  PubMed  Google Scholar 

  29. Romanska-Gocka K, Ciescinska C, Zegarska B, Schwartz RA, Ciescinski J, Olszewska-Slonina D et al (2015) Pyoderma gangrenosum with monoclonal IgA gammopathy and pulmonary tuberculosis. Illustrative case and review. Postepy Dermatol Alergol 32(2):137–141

    Article  PubMed  PubMed Central  Google Scholar 

  30. Velasco-Tamariz V, Carreno-Tarragona G, Tous-Romero F, Gil-de la Cruz E, Martin-Clavero E, Rivera-Diaz R (2017) Dramatic resolution of disseminated pyoderma gangrenosum associated with monoclonal gammopathy after therapy with bortezomib and dexamethasone. Int Wound J 14(6):1382–1384

    Article  PubMed  PubMed Central  Google Scholar 

  31. Chang CM, Hwang WL, Hsieh ZY, Wang RC, Teng CL (2010) Monoclonal gammopathy of undetermined significance related pyoderma gangrenosum successfully treated with autologous peripheral blood stem cell transplantation. Ann Hematol 89(8):823–824

    Article  PubMed  Google Scholar 

  32. Binus AM, Qureshi AA, Li VW, Winterfield LS (2011) Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol 165(6):1244–1250

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

The Scientific Publications staff at Mayo Clinic provided editorial consultation, proofreading, and administrative and clerical support.

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Authors

Contributions

V.S. data extraction, review the data, draft the paperV. G. data extraction, review the data, draft the paper S. K. review of the paper D. A. W. review of the paperM. D. P. D. review of the paperA. T. statisticsJ. D. review the paperA. A. study design, review the data, major contribution in the manuscript, review the final draft.

Corresponding author

Correspondence to Afsaneh Alavi.

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Saeidi, V., Garimella, V., Shaji, K. et al. Monoclonal gammopathy in the setting of Pyoderma gangrenosum. Arch Dermatol Res 316, 268 (2024). https://doi.org/10.1007/s00403-024-03098-7

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  • DOI: https://doi.org/10.1007/s00403-024-03098-7

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