Pyoderma gangrenosum: examination and treatment experience
- Authors: Teplyuk N.P.1, Grabovskaya O.V.1, Kusraeva D.T.1, Varshavsky V.A.1
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Affiliations:
- I.M. Sechenov First Moscow State Medical University (Sechenov University)
- Issue: Vol 25, No 1 (2022)
- Pages: 61-72
- Section: DERMATOLOGY
- URL: https://journals.rcsi.science/1560-9588/article/view/105685
- DOI: https://doi.org/10.17816/dv105685
- ID: 105685
Cite item
Abstract
BACKGROUND: Pyoderma gangrenosum is a rare inflammatory skin disease that currently belongs to the group of neutrophilic dermatoses.
AIMS: Development of epidemiological, clinical, laboratory characteristics of patients with pyoderma gangrenosum, as well as the treatment of this disease.
MATERIALS AND METHODS: The study revealed 30 patients, 16 (53%) were women, the average age at the time of diagnosis was 59±16.3 years. The most common localization of rashes was the lower leg 20 patients (67%), lesions of the skin of the face, genitals, and breast rarely noted, in one case, respectively. In 14 patients (47%), two or more ulcers were detected at the same time (max. 9 ulcers). The pathergy phenomenon was positive in 23 patients (77%) of 30. Ulcerative pyoderma gangrenosum occurred in 25 (83%), and extracutaneous pyoderma gangrenosum with lung involvement was identified in one patient. The most common associated disease was rheumatoid arthritis in 6 patients (20%), there were also rare associated diseases, such as hepatitis C two cases, multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, non-Hodgkin’s lymphoma by one observation. Histological examination revealed neutrophilic infiltration of the dermis in 30 patients (100%), and the presence of leukocytoclastic vasculitis in 16 (53%). Complete scarring during the treatment was observed in 22 patients (73%). Relapses during the observation period were observed in 12 patients (40%), the number of deaths was two cases.
CONCLUSIONS: One of the largest case series of pyoderma gangrenosum to date is presented. During the examination of patients, it was found that neutrophilic infiltration of the dermis is a characteristic sign of the disease. Rare comorbidities (multiple endocrine neoplasia syndrome type 1, autoimmune hepatitis, hepatitis C, non-Hodgkin’s lymphoma) have been found. A sufficiently large percentage of relapses indicates the need for further research to develop an additional examination method for the purpose of modern diagnostics and justification for the timely prescription of targeted therapy.
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##article.viewOnOriginalSite##About the authors
Nataliа P. Teplyuk
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: teplyukn@gmail.com
ORCID iD: 0000-0002-5800-4800
SPIN-code: 8013-3256
MD, Dr. Sci. (Med.), Professor
Russian Federation, MoscowOlga V. Grabovskaya
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: olgadoctor2013@yandex.ru
ORCID iD: 0000-0002-5259-7481
SPIN-code: 1843-1090
MD, Cand. Sci. (Med.), Associate Professor
Russian Federation, MoscowDiana T. Kusraeva
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Author for correspondence.
Email: kysra1992@mail.ru
ORCID iD: 0000-0002-5633-7986
SPIN-code: 1478-3501
Graduate Student
Russian Federation, MoscowVladimir A. Varshavsky
I.M. Sechenov First Moscow State Medical University (Sechenov University)
Email: vavarsh@gmail.com
ORCID iD: 0000-0002-5855-3092
MD, Dr. Sci. (Med.), Proffessor
Russian Federation, MoscowReferences
- Hobbs MM, Ortega-Loayza AG. Pyoderma gangrenosum: from historical perspectives to emerging investigations. Int Wound J. 2020;17(5):1255–1265. doi: 10.1111/iwj.13389
- Alavi A, French LE, Davis MD, et al. Pyoderma gangrenosum: an update on pathophysiology, diagnosis and treatment. Am J Clin Dermatol. 2017;18(3):355–372. doi: 10.1007/s40257-017-0251-7
- Ormerod AD. Epidemiology, comorbidities and mortality of pyoderma gangrenosum: new insights. Br J Dermatol. 2021;185(6):1089–1090. doi: 10.1111/bjd.20713
- Ben Abdallah H, Bech R, Fogh K, et al. Comorbidities, mortality and survival in patients with pyoderma gangrenosum: a Danish nationwide registry-nested case-control study. Br J Dermatol. 2021;185(6):1169–1175. doi: 10.1111/bjd.20474
- Maverakis E, Marzano AV, Le ST, et al. Pyoderma gangrenosum. Nat Rev Dis Primers. 2020;6(1):81. doi: 10.1038/s41572-020-0213-x
- Marzano AV, Damiani G, Ceccherini I, et al. Autoinflammation in pyoderma gangrenosum and its syndromic form (pyoderma gangrenosum, acne and suppurative hidradenitis). Br J Dermatol. 2017;176(6):1588–1598. doi: 10.1111/bjd.15226
- Alonso-León T, Hernández-Ramírez HH, Fonte-Avalos V, et al. The great imitator with no diagnostic test: pyoderma gangrenosum. Int Wound J. 2020;17(6):1774–1782. doi: 10.1111/iwj.13466
- Ehrl DC, Heidekrueger PI, Broer PN. Pyoderma gangrenosum after breast surgery: a systematic review. J Plast Reconstr Aesthet Surg. 2018;71(7):1023–1032. doi: 10.1016/j.bjps.2018.03.013
- Goldust M, Hagstrom EL, Rathod D, Ortega-Loayza AG. Diagnosis and novel clinical treatment strategies for pyoderma gangrenosum. Expert Rev Clin Pharmacol. 2020;13(2):157–161. doi: 10.1080/17512433.2020.1709825
- Barbe M, Batra A, Golding S, et al. Pyoderma gangrenosum: a literature review. Clin Podiatr Med Surg. 2021;38(4):577–588. doi: 10.1016/j.cpm.2021.06.002
- Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients: PG-patient characteristics, comorbidities and therapy. Brit J Dermatol. 2011;165(6):1244–1250. doi: 10.1111/j.1365-2133.2011.10565.x
- Hasselmann DO, Bens G, Tilgen W, Reichrath J. Pyoderma gangrenosum: clinical presentation and outcome in 18 cases and review of the literature. JDDG. 2007;5(7):560–564. doi: 10.1111/j.1610-0387.2007.0328.x
- Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985;55(217):173–186.
- Ashchyan HJ, Butler DC, Nelson CA, et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol. 2018;154(4):409. doi: 10.1001/jamadermatol.2017.5978
- Kridin K, Cohen AD, Amber KT. Underlying systemic diseases in pyoderma gangrenosum: a systematic review and meta-analysis. Am J Clin Dermatol. 2018;19(4):479–487. doi: 10.1007/s40257-018-0356-7
- Rodríguez-Zúñiga MJ, Heath MS, Gontijo JR, Ortega-Loayza AG. Pyoderma gangrenosum: a review with special emphasis on Latin America literature. An Bras Dermatol. 2019;94(6):729–743. doi: 10.1016/j.abd.2019.06.001
- Sawka E, Zhou A, Latour E, et al. Inflammatory arthritis-associated pyoderma gangrenosum: a systematic review. Clin Rheumatol. 2021;40(10):3963–3969. doi: 10.1007/s10067-021-05768-7
- Beber AA, Knob CF, Shons KR, et al. Pioderma gangrenoso associado à artrite reumatoide: descrição de caso. Revista Brasileira de Reumatologia. 2014;54(4):322–325. doi: 10.1016/j.rbr.2013.02.004
- Xing F, Chiu KH, Yang J, et al. Pyoderma gangrenosum with pulmonary involvement: a pulmonary special report and literature review. Expert Rev Respir Med. 2022;16(2):149–159. doi: 10.1080/17476348.2022.2027756
- Wang JY, French LE, Shear NH, et al. Drug-Induced pyoderma gangrenosum: a review. Am J Clin Dermatol. 2018;19(1):67–77. doi: 10.1007/s40257-017-0308-7
- Billings SD. Common and critical inflammatory dermatoses every pathologist should know. Mod Pathol. 2019;33(Suppl 1): 107–117. doi: 10.1038/s41379-019-0400-z
- Figueras-Nart I, Mascaró JM, Solanich X, Hernández-Rodríguez J. Dermatologic and dermatopathologic features of monogenic autoinflammatory diseases. Front Immunol. 2019;10:2448. doi: 10.3389/fimmu.2019.02448
- Haag CK, Nutan F, Cyrus JW, et al. Pyoderma gangrenosum misdiagnosis resulting in amputation: a review. J Trauma Acute Care Surg. 2019;86(2):307–313. doi: 10.1097/TA.0000000000002096
- Olisova OY, Snarskaya E, Smirnova L, et al. Dermatitis artefacta: self-inflicted genital injury. IMCRJ. 2019;12:71–73. doi: 10.2147/IMCRJ.S192522
- Capron M, Antiga E, Volpi W, et al. The Treg/Th17 cell ratio is reduced in the skin lesions of patients with pyoderma gangrenosum. Br J Dermatol. 2015;173(1):275–278. doi: 10.1111/bjd.13670
- Yamada A, Wang J, Komaki Y, et al. Systematic review with meta-analysis: risk of new onset IBD with the use of anti-interleukin-17 agents. Aliment Pharmacol Ther. 2019;50(4):373–385. doi: 10.1111/apt.15397
- Olisova OY, Nikuradze VO. Efficacy of secukinumab in the treatment of patients with severe psoriasis. Effective Pharmacotherapy. 2020;16(9):6–10. (In Russ). doi: 10.33978/2307-3586-2020-16-9-6-10
- Petty AJ, Whitley MJ, Balaban A, et al. Pyoderma gangrenosum induced by secukinumab in a patient with psoriasis successfully treated with ustekinumab. JAAD Case Reports. 2020;6(8):731–733. doi: 10.1016/j.jdcr.2020.06.011
- Vilches SF, Vera-Kellet C. Pyoderma gangrenosum: classic and emerging therapies. Med Clín (English Edition). 2017;149(6):256–260. doi: 10.1016/j.medcle.2017.08.007