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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Abbreviations
- Ig:
-
Immunoglobulin
- MDS:
-
Myelodysplastic syndromes
- MG:
-
Monoclonal gammopathy
- MGUS:
-
Monoclonal gammopathy of uncertain significance
- PG:
-
Pyoderma gangrenosum
References
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
Birnkrant MJ, Papadopoulos AJ, Schwartz RA, Lambert WC (2003) Pyoderma Gangrenosum, acne conglobata, and IgA gammopathy. Int J Dermatol 42(3):213–216
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
Hou YL, Lee CH (2022) Pyoderma Gangrenosum. JAMA Dermatol 158(2):202
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
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
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
Mateos MV, Gonzalez-Calle V (2017) Smoldering multiple myeloma: who and when to treat. Clin Lymphoma Myeloma Leuk 17(11):716–722
Kaseb H, Annamaraju P, Babiker HM (2023) Monoclonal gammopathy of undetermined significance. StatPearls. Treasure Island (FL)
Wadhera RK, Rajkumar SV (2010) Prevalence of monoclonal gammopathy of undetermined significance: a systematic review. Mayo Clin Proc 85(10):933–942
Powell FC, Schroeter AL, Su WP, Perry HO (1983) Pyoderma gangrenosum and monoclonal gammopathy. Arch Dermatol 119(6):468–472
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
Chave TA, Hutchinson PE (2001) Pyoderma Gangrenosum, subcorneal pustular dermatosis, IgA paraproteinaemia and IgG antiepithelial antibodies. Br J Dermatol 145(5):852–854
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
Lipsker D (2017) Monoclonal gammopathy of cutaneous significance: review of a relevant concept. J Eur Acad Dermatol Venereol 31(1):45–52
Claveau JS, Wetter DA, Kumar S (2022) Cutaneous manifestations of monoclonal gammopathy. Blood Cancer J 12(4):58
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
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)
Kyle RA, Rajkumar SV (2006) Monoclonal gammopathy of undetermined significance. Br J Haematol 134(6):573–589
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
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
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
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
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
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
Kyle RA, Rajkumar SV (2005) Monoclonal gammopathies of undetermined significance. Best Pract Res Clin Haematol 18(4):689–707
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
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
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
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
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
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
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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