坏疽性脓皮病系统合并症的研究进展

doi:10.35541/cjd.20240424

Abstract

Abstract: 【Abstract】 Pyoderma gangrenosum （PG） is an autoinflammatory disorder that belongs to neutrophilic dermatosis. It often presents as a rapidly developing painful cutaneous ulcer, which is characterized by disruption of the border and peripheral erythema. The exact etiology of PG remains unknown, but it is often associated with various immune-mediated disorders. PG is associated with a wide range of underlying disorders, with inflammatory bowel disease, hematological diseases and malignancies, autoinflammatory syndromes and inflammatory arthritis as the most common underlying disorders. This review summarizes PG-related systemic complications.

Key words: Pyoderma gangrenosum, Systemic comorbidities, Inflammatory bowel disease, Therapy, Pathogenesis, Biologic agents, JAK inhibitor

Xu Qiuyun, Ji Chao. Pyoderma gangrenosum-related systemic complications[J]. Chinese Journal of Dermatology, 2025, 58(4): 369-373.doi:10.35541/cjd.20240424

References ［62］

［1］	May MR, Rübben A, Lennertz A, et al. Dealing with corticosteroid and high⁃dose cyclosporine therapy in a pyoderma gangrenosum patient contracting a COVID⁃19 infection［J］. J Pers Med, 2022,12（2）:173. doi: 10.3390/jpm12020173.
［2］	Maverakis E, Marzano AV, Le ST, et al. Pyoderma gangrenosum［J］. Nat Rev Dis Primers, 2020,6（1）:81. doi: 10.1038/s41572⁃020⁃0213⁃x.
［3］	胡清洁, 周敏, 姚煦. 坏疽性脓皮病36例临床特点分析及PARACELSUS评分与Delphi标准的比较［J］. 中华皮肤科杂志, 2023,56（5）:410⁃414. doi: 10.35541/cjd.20220825.
［4］	Zhao H, Sengupta SK, Haddadin O, et al. Effects of comorbidities on delayed wound healing in patients with pyoderma gangrenosum［J］. J Eur Acad Dermatol Venereol, 2024. doi: 10.1111/jdv.20457.
［5］	Kridin K, Damiani G, Ludwig RJ, et al. Estimating the odds of ulcerative colitis⁃associated pyoderma gangrenosum: a population⁃based case⁃control study［J］. Dermatology, 2021,237（3）:323⁃329. doi: 10.1159/000512931.
［6］	Kridin K, Damiani G, Ludwig RJ, et al. Quantification of the relationship between pyoderma gangrenosum and Crohn's disease: a population⁃based case⁃control study［J］. Scand J Gastroenterol, 2020,55（7）:814⁃818. doi: 10.1080/00365521. 2020.1786849.
［7］	Ben Abdallah H, Fogh K, Vestergaard C, et al. Pyoderma gangrenosum and interleukin inhibitors: a semi⁃systematic review［J］. Dermatology, 2022,238（4）:785⁃792. doi: 10.1159/00051 9320.
［8］	Antonelli E, Bassotti G, Tramontana M, et al. Dermatological manifestations in inflammatory bowel diseases［J］. J Clin Med, 2021,10（2）:364. doi: 10.3390/jcm10020364.
［9］	Argüelles⁃Arias F, Castro⁃Laria L, Lobatón T, et al. Characteristics and treatment of pyoderma gangrenosum in inflammatory bowel disease［J］. Dig Dis Sci, 2013,58（10）:2949⁃2954. doi: 10.1007/s10620⁃013⁃2762⁃2.
［10］	van Loo G, Bertrand M. Death by TNF: a road to inflammation［J］. Nat Rev Immunol, 2023,23（5）:289⁃303. doi: 10.1038/s41577⁃022⁃00792⁃3.
［11］	Holmes Z, Nguyen J, Courtney A, et al. Treatment of severe, refractory pyoderma gangrenosum with infliximab, adalimumab and intravenous immunoglobulin［J］. Australas J Dermatol, 2023,64（3）:433⁃435. doi: 10.1111/ajd.14067.
［12］	Verstockt B, Salas A, Sands BE, et al. IL⁃12 and IL⁃23 pathway inhibition in inflammatory bowel disease［J］. Nat Rev Gastroenterol Hepatol, 2023,20（7）:433⁃446. doi: 10.1038/s41575⁃023⁃00768⁃1.
［13］	Fanizza J, D'Amico F, Lusetti F, et al. The role of IL⁃23 inhibitors in Crohn's disease［J］. J Clin Med, 2023,13（1）:224. doi: 10.3390/jcm13010224.
［14］	Westerdahl JS, Nusbaum KB, Chung CG, et al. Ustekinumab as adjuvant treatment for all pyoderma gangrenosum subtypes［J］. J Dermatolog Treat, 2022,33（4）:2386⁃2390. doi: 10.1080/0954 6634.2021.1937475.
［15］	Burgdorf B, Schlott S, Ivanov IH, et al. Successful treatment of a refractory pyoderma gangrenosum with risankizumab［J］. Int Wound J, 2020,17（4）:1086⁃1088. doi: 10.1111/iwj.13359.
［16］	Reese AM, Erickson K, Reed KB, et al. Modified dose of guselkumab for treatment of pyoderma gangrenosum［J］. JAAD Case Rep, 2022,21:38⁃42. doi: 10.1016/j.jdcr.2021.11.030.
［17］	Chen Y, Ye X, Escames G, et al. The NLRP3 inflammasome: contributions to inflammation⁃related diseases［J］. Cell Mol Biol Lett, 2023,28（1）:51. doi: 10.1186/s11658⁃023⁃00462⁃9.
［18］	Jatana S, Ponti AK, Johnson EE, et al. A novel murine model of pyoderma gangrenosum reveals that inflammatory skin⁃gut crosstalk is mediated by IL⁃1β⁃primed neutrophils［J］. Front Immunol, 2023,14:1148893. doi: 10.3389/fimmu.2023.1148893.
［19］	O'Connor C, Gallagher C, Hollywood A, et al. Anakinra for recalcitrant pyoderma gangrenosum［J］. Clin Exp Dermatol, 2021,46（8）:1558⁃1560. doi: 10.1111/ced.14809.
［20］	Ben Abdallah H, Fogh K, Vestergaard C, et al. Pyoderma gangrenosum and interleukin inhibitors: a semi⁃systematic review［J］. Dermatology, 2022,238（4）:785⁃792. doi: 10.1159/00051 9320.
［21］	McPhie ML, Kirchhof MG. Pyoderma gangrenosum treated with secukinumab: a case report［J］. SAGE Open Med Case Rep, 2020,8:2050313X20940430. doi: 10.1177/2050313X20940430.
［22］	Tee MW, Avarbock AB, Ungar J, et al. Rapid resolution of pyoderma gangrenosum with brodalumab therapy［J］. JAAD Case Rep, 2020,6（11）:1167⁃1169. doi: 10.1016/j.jdcr.2020.08.033.
［23］	Kao AS, King AD, Daveluy S. Successful treatment of cabozantinib⁃induced pyoderma gangrenosum with ixekizumab therapy: a case report［J］. Dermatol Ther, 2022,35（9）:e15716. doi: 10.1111/dth.15716.
［24］	Dissemond J, Marzano AV, Hampton PJ, et al. Pyoderma gangrenosum: treatment options［J］. Drugs, 2023,83（14）:1255⁃1267. doi: 10.1007/s40265⁃023⁃01931⁃3.
［25］	Deng Z, Wang S, Wu C, et al. IL⁃17 inhibitor⁃associated inflammatory bowel disease: a study based on literature and database analysis［J］. Front Pharmacol, 2023,14:1124628. doi: 10.3389/fphar.2023.1124628.
［26］	Salas A, Hernandez⁃Rocha C, Duijvestein M, et al. JAK⁃STAT pathway targeting for the treatment of inflammatory bowel disease［J］. Nat Rev Gastroenterol Hepatol, 2020,17（6）:323⁃337. doi: 10.1038/s41575⁃020⁃0273⁃0.
［27］	Herrera⁃deGuise C, Serra⁃Ruiz X, Lastiri E, et al. JAK inhibitors: a new dawn for oral therapies in inflammatory bowel diseases［J］. Front Med （Lausanne）, 2023,10:1089099. doi: 10. 3389/fmed.2023.1089099.
［28］	Castro L. JAK inhibitors: a novel, safe, and efficacious therapy for pyoderma gangrenosum［J］. Int J Dermatol, 2023,62（8）:1088⁃1093. doi: 10.1111/ijd.16676.
［29］	Kridin K, Ankary⁃Khaner M, Kridin M, et al. Hematological malignancy⁃associated pyoderma gangrenosum: evaluating the magnitude of the association［J］. Front Med （Lausanne）, 2024,11:1425454. doi: 10.3389/fmed.2024.1425454.
［30］	Ruocco E, Sangiuliano S, Gravina AG, et al. Pyoderma gangrenosum: an updated review［J］. J Eur Acad Dermatol Venereol, 2009,23（9）:1008⁃1017. doi: 10.1111/j.1468⁃3083. 2009.03199.x.
［31］	Montagnon CM, Fracica EA, Patel AA, et al. Pyoderma gangrenosum in hematologic malignancies: a systematic review［J］. J Am Acad Dermatol, 2020,82（6）:1346⁃1359. doi: 10.1016/j.jaad.2019.09.032.
［32］	Martins M, Barbosa J, Eusébio SP, et al. From the deep: overlap of neutrophilic dermatoses disorders associated with acute myeloid leukemia［J］. Cureus, 2023,15（1）:e33456. doi: 10.7759/cureus.33456.
［33］	Szalat R, Monsel G, Le Goff W, et al. The spectrum of neutrophilic dermatoses associated with monoclonal gammopathy: association with IgA isotype and inflammatory profile［J］. J Am Acad Dermatol, 2015,73（5）:809⁃820. doi: 10.1016/j.jaad.2015. 07.031.
［34］	Maglie R, Genovese G, Solimani F, et al. Immune⁃mediated dermatoses in patients with haematological malignancies: a comprehensive review［J］. Am J Clin Dermatol, 2020,21（6）:833⁃854. doi: 10.1007/s40257⁃020⁃00553⁃9.
［35］	Parrondo RD, Iqbal M, Von Roemeling R, et al. IRAK⁃4 inhibition: emavusertib for the treatment of lymphoid and myeloid malignancies［J］. Front Immunol, 2023,14:1239082. doi: 10.3389/fimmu.2023.1239082.
［36］	Ferrarini I, Rigo A, Zamò A, et al. Classical Hodgkin lymphoma cells may promote an IL⁃17⁃enriched microenvironment［J］. Leuk Lymphoma, 2019,60（14）:3395⁃3405. doi: 10.1080/1042 8194.2019.1636983.
［37］	Wang F, Li Y, Yang Z, et al. Targeting IL⁃17A enhances imatinib efficacy in Philadelphia chromosome⁃positive B⁃cell acute lymphoblastic leukemia［J］. Nat Commun, 2024,15（1）:203. doi: 10.1038/s41467⁃023⁃44270⁃3.
［38］	Yu S, Jiang J. Immune infiltration⁃related genes regulate the progression of AML by invading the bone marrow microenvironment［J］. Front Immunol, 2024,15:1409945. doi: 10.3389/fimmu.2024.1409945.
［39］	Blicharz L, Czuwara J, Rudnicka L, et al. Autoinflammatory keratinization diseases⁃the concept, pathophysiology, and clinical implications［J］. Clin Rev Allergy Immunol, 2023,65（3）:377⁃402. doi: 10.1007/s12016⁃023⁃08971⁃3.
［40］	Wouters F, Bogie J, Wullaert A, et al. Recent insights in pyrin inflammasome activation: identifying potential novel therapeutic approaches in pyrin⁃associated autoinflammatory syndromes［J］. J Clin Immunol, 2023,44（1）:8. doi: 10.1007/s10875⁃023⁃01621⁃5.
［41］	D'Onghia M, Malvaso D, Galluccio G, et al. Evidence on hidradenitis suppurativa as an autoinflammatory skin disease［J］. J Clin Med, 2024,13（17）:5211. doi: 10.3390/jcm13175211.
［42］	Yamanaka K. New treatment of pyoderma gangrenosum and hidradenitis suppurativa: a review［J］. J Dermatol, 2024,51（2）:172⁃179. doi: 10.1111/1346⁃8138.17031.
［43］	Brandão L, Moura RR, Marzano AV, et al. Variant enrichment analysis to explore pathways functionality in complex autoinflammatory skin disorders through whole exome sequencing analysis［J］. Int J Mol Sci, 2022,23（4）:2278. doi: 10.3390/ijms23042278.
［44］	Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH syndromes: pathophysiology, presentation and treatment［J］. Am J Clin Dermatol, 2017,18（4）:555⁃562. doi: 10.1007/s40257⁃017⁃0265⁃1.
［45］	Chokoeva AA, Cardoso JC, Wollina U, et al. Pyoderma gangrenosum⁃a novel approach?［J］. Wien Med Wochenschr, 2017,167（3⁃4）:58⁃65. doi: 10.1007/s10354⁃016⁃0472⁃z.
［46］	Xu A, Strunk A, Garg A, et al. Prevalence of inflammatory bowel disease in patients with pyoderma gangrenosum: a population⁃based analysis［J］. J Am Acad Dermatol, 2022,86（6）:1351⁃1352. doi: 10.1016/j.jaad.2021.05.006.
［47］	Binus AM, Qureshi AA, Li VW, et al. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients［J］. Br J Dermatol, 2011,165（6）:1244⁃1250. doi: 10.1111/j.1365⁃2133.2011.10565.x.
［48］	Kridin K, Cohen AD, Amber KT. Underlying systemic diseases in pyoderma gangrenosum: a systematic review and meta⁃analysis［J］. Am J Clin Dermatol, 2018,19（4）:479⁃487. doi: 10.1007/s40257⁃018⁃0356⁃7.
［49］	Kridin K, Damiani G, Cohen AD. Rheumatoid arthritis and pyoderma gangrenosum: a population⁃based case⁃control study［J］. Clin Rheumatol, 2021,40（2）:521⁃528. doi: 10.1007/s10067⁃020⁃05253⁃7.
［50］	Cugno M, Gualtierotti R, Meroni PL, et al. Inflammatory joint disorders and neutrophilic dermatoses: a comprehensive review［J］. Clin Rev Allergy Immunol, 2018,54（2）:269⁃281. doi: 10. 1007/s12016⁃017⁃8629⁃0.
［51］	Weber B, Liao KP. Evidence for biologic drug modifying anti⁃rheumatoid drugs and association with cardiovascular disease risk mitigation in inflammatory arthritis［J］. Rheum Dis Clin North Am, 2023,49（1）:165⁃178. doi: 10.1016/j.rdc.2022.08.005.
［52］	Maalouf D, Battistella M, Bouaziz JD. Neutrophilic dermatosis: disease mechanism and treatment［J］. Curr Opin Hematol, 2015,22（1）:23⁃29. doi: 10.1097/MOH.0000000000000100.
［53］	Sawka E, Zhou A, Latour E, et al. Inflammatory arthritis⁃associated pyoderma gangrenosum: a systematic review［J］. Clin Rheumatol, 2021,40（10）:3963⁃3969. doi: 10.1007/s10067⁃021⁃05768⁃7.
［54］	Xing F, Chiu KH, Yang J, et al. Pyoderma gangrenosum with pulmonary involvement: a pulmonary special report and literature review［J］. Expert Rev Respir Med, 2022,16（2）:149⁃159. doi: 10.1080/17476348.2022.2027756.
［55］	Shavit E, Dagan O, Cohen AD, et al. Risk of cardiovascular diseases in pyoderma gangrenosum: a population⁃based study［J］. J Eur Acad Dermatol Venereol, 2023,37（2）:e197⁃e199. doi: 10.1111/jdv.18527.
［56］	Gillard M, Anuset D, Maillard H, et al. Comorbidities of pyoderma gangrenosum: a retrospective multicentric analysis of 126 patients［J］. Br J Dermatol, 2018,179（1）:218⁃219. doi: 10. 1111/bjd.16463.
［57］	Zaino ML, Schadt CR, Callen JP, et al. Pyoderma gangrenosum: diagnostic criteria, subtypes, systemic associations, and workup［J］. Dermatol Clin, 2024,42（2）:157⁃170. doi: 10.1016/j.det. 2023.08.003.
［58］	Kridin K, Solomon A, Britva RL, et al. Chronic renal comorbidities in pyoderma gangrenosum: a retrospective cohort study［J］. Immunol Res, 2021,69（3）:249⁃254. doi: 10.1007/s12026⁃021⁃09187⁃3.
［59］	Al Ghazal P, Herberger K, Schaller J, et al. Associated factors and comorbidities in patients with pyoderma gangrenosum in Germany: a retrospective multicentric analysis in 259 patients［J］. Orphanet J Rare Dis, 2013,8:136. doi: 10.1186/1750⁃1172⁃8⁃136.
［60］	Shah M, Sachdeva M, Gefri A, et al. Paraneoplastic pyoderma gangrenosum in solid organ malignancy: a literature review［J］. Int J Dermatol, 2020,59（2）:154⁃158. doi: 10.1111/ijd.14637.
［61］	Kridin K, Laufer Britva R, Tzur Bitan D, et al. Is pyoderma gangrenosum associated with solid malignancies? Insights from a population⁃based cohort study［J］. Australas J Dermatol, 2021,62（3）:336⁃341. doi: 10.1111/ajd.13631.
［62］	Ravi M, Trinidad J, Spaccarelli N, et al. The impact of comorbidity identification on outcomes in patients with pyoderma gangrenosum: a retrospective cohort study of previously hospitalized patients［J］. J Am Acad Dermatol, 2022,87（1）:194⁃197. doi: 10.1016/j.jaad.2021.07.032.

[1]	Wan Yongjun, Yang Haijing, Yan Qiao, Chen Mei, Wang Fengyuan, Su Qianya, Dong Zhengbang, Wang Fei. Clinical and prognostic analysis of nine cases of immune checkpoint inhibitor-related Stevens-Johnson syndrome/toxic epidermal necrolysis [J]. Chinese Journal of Dermatology, 2025, 58(4): 347-351.
[2]	Group on Pediatric Dermatology, China Dermatologist Association, Committee on Psoriasis, Chinese Society of Dermatology. Expert consensus on the diagnosis and treatment of pediatric pustular psoriasis in China （2025 version） [J]. Chinese Journal of Dermatology, 2025, 58(4): 297-306.
[3]	Li Ziyu, Lu Yan. Paying attention to severe skin diseases after COVID-19 [J]. Chinese Journal of Dermatology, 2025, 58(4): 378-383.
[4]	Lyu Mingyue, Man Xiaoyong. Application of cell therapy in systemic lupus erythematosus [J]. Chinese Journal of Dermatology, 2025, 58(4): 374-377.
[5]	Ye Hui, Deng Shilin, Liang Jingyao, Zhang Xibao. Correlations between the control of atopic dermatitis recurrence and tissue-resident memory T cells [J]. Chinese Journal of Dermatology, 2025, 0(3): 20240192-e20240192.
[6]	Li Xiangqian, Zhang Jianzhong, Zhou Cheng. Advances in the clinical research on systemic small-molecule drugs and biologics for the treatment of alopecia areata [J]. Chinese Journal of Dermatology, 2025, 0(3): 20240247-e20240247.
[7]	Chen Qitao, Li Yuqian, Shao Guanghui, Zhu Jing, Zhu Qilin, Li Zhongming, Du Xufeng, Fan Weixin. Erosive pustular dermatosis of the scalp [J]. Chinese Journal of Dermatology, 2025, 58(3): 272-275.
[8]	Li Xiuzhen, Xu Xiulian. Effects of interleukin-23 inhibitors on psoriasis comorbidities [J]. Chinese Journal of Dermatology, 2025, 58(3): 268-272.
[9]	Jiang Jiayi, Wang Daguang. Pathogenesis of nail damages secondary to systemic diseases [J]. Chinese Journal of Dermatology, 2025, 58(3): 282-285.
[10]	Chinese Association of Rehabilitation Dermatology, Chinese Society of Dermatology, Photomedicine Therapeutic Equipment Group, Committee on Skin Disease and Cosmetic Dermatology, China Association of Medical Equipment. Clinical application of red, blue, and yellow light therapy in dermatology: an expert consensus statement （2025 edition） [J]. Chinese Journal of Dermatology, 2025, 58(3): 209-215.
[11]	Dermatology Professional Committee of Chinese Research Hospital Society, China Dermatologist Association. Guideline on phototherapy for vitiligo （2025 edition） [J]. Chinese Journal of Dermatology, 2025, 58(3): 197-208.
[12]	Xu Zhuohong, Hu Yu, Gu Heng. Role of mesenchymal stem cell-derived exosomes in skin diseases [J]. Chinese Journal of Dermatology, 2025, 0(3): 20220881-e20220881.
[13]	Committee on Skin Disease and Cosmetic Dermatology, China Association of Medical Equipment. Expert consensus on the treatment of dermatoses with targeted drugs （2025 version） [J]. Chinese Journal of Dermatology, 2025, 58(2): 99-125.
[14]	Li Meirong, Tang Xuhua, Wu Rong, Liu Wentao, Feng Peiying. Four cases of skin infections caused by rare Neoscytalidium dimidiatum in China [J]. Chinese Journal of Dermatology, 2025, 0(2): 20230579-e20230579.
[15]	Zhou Miaoni, Sheng Anqi, Fu Lifang, Jin Rong, Xu Wen, Wei Xiaodong, Xu Ai′e . Efficacy and safety of tea polyphenol antioxidant gel combined with narrow-band ultraviolet B in the treatment of vitiligo: a single-center randomized controlled trial [J]. Chinese Journal of Dermatology, 2025, 0(2): 20240421-e20240421.

Pyoderma gangrenosum-related systemic complications

PDF

Knowledge

Abstract

Cite this article

share this article

References ［62］

Related Articles 15

Metrics

Comments

Recommended 10