Clinical, epidemiological, and therapeutic hallmarks of pyoderma gangrenosum: a case series of 35 patients.
inflammatory diseases
pyoderma gangrenosum
Journal
International journal of dermatology
ISSN: 1365-4632
Titre abrégé: Int J Dermatol
Pays: England
ID NLM: 0243704
Informations de publication
Date de publication:
25 Feb 2024
25 Feb 2024
Historique:
revised:
20
01
2024
received:
14
11
2023
accepted:
02
02
2024
medline:
25
2
2024
pubmed:
25
2
2024
entrez:
25
2
2024
Statut:
aheadofprint
Résumé
Over the past few decades, advances in medical research and diagnostic tools have shed light on some aspects of pyoderma gangrenosum (PG). Nevertheless, the multifactorial etiology, pathogenesis, and optimal management strategies for PG need to be further investigated. To address these knowledge gaps and contribute to a better understanding of this complex dermatological disorder, we collected epidemiological, clinical, and therapeutic aspects of a case series of PG patients occurring in our department over the past 10 years. We performed a single-centered, retrospective, observational study analyzing all cases with a diagnosis of PG observed at the Dermatology clinic of the Fondazione Policlinico A. Gemelli IRCCS Catholic University from January 1, 2013, to January 1, 2023. For each case, we retrieved demographic data, the presence of other skin and systemic conditions, and the histopathological and clinical characteristics of PG, such as clinical variant, number of lesions, disease localization, previous therapy, response to treatment, and occurrence of relapse. We included 35 patients, 22 females and 13 males with a mean age of 40.0 years. Twenty patients (57.1%) had multiple localizations of disease, and the most commonly involved site was the lower limbs (85.7%). The lesions were mainly associated with inflammatory bowel diseases (51.4%) and hidradenitis suppurativa (37.1%). Clinical resolution with complete re-epithelialization was achieved in 25 patients (71.4%) with an average time of 20.8 months. On average, patients who underwent therapy with biological drugs had better outcomes. PG is a severe, rare, and pleomorphic disease associated with a broad spectrum of conditions. Corticosteroids remain the primary first-line approach for severe forms, but using biological immunosuppressants is promising.
Sections du résumé
BACKGROUND
BACKGROUND
Over the past few decades, advances in medical research and diagnostic tools have shed light on some aspects of pyoderma gangrenosum (PG). Nevertheless, the multifactorial etiology, pathogenesis, and optimal management strategies for PG need to be further investigated. To address these knowledge gaps and contribute to a better understanding of this complex dermatological disorder, we collected epidemiological, clinical, and therapeutic aspects of a case series of PG patients occurring in our department over the past 10 years.
METHODS
METHODS
We performed a single-centered, retrospective, observational study analyzing all cases with a diagnosis of PG observed at the Dermatology clinic of the Fondazione Policlinico A. Gemelli IRCCS Catholic University from January 1, 2013, to January 1, 2023. For each case, we retrieved demographic data, the presence of other skin and systemic conditions, and the histopathological and clinical characteristics of PG, such as clinical variant, number of lesions, disease localization, previous therapy, response to treatment, and occurrence of relapse.
RESULTS
RESULTS
We included 35 patients, 22 females and 13 males with a mean age of 40.0 years. Twenty patients (57.1%) had multiple localizations of disease, and the most commonly involved site was the lower limbs (85.7%). The lesions were mainly associated with inflammatory bowel diseases (51.4%) and hidradenitis suppurativa (37.1%). Clinical resolution with complete re-epithelialization was achieved in 25 patients (71.4%) with an average time of 20.8 months. On average, patients who underwent therapy with biological drugs had better outcomes.
CONCLUSIONS
CONCLUSIONS
PG is a severe, rare, and pleomorphic disease associated with a broad spectrum of conditions. Corticosteroids remain the primary first-line approach for severe forms, but using biological immunosuppressants is promising.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© 2024 The Authors. International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology.
Références
Weiss EH, Ko CJ, Leung TH, Micheletti RG, Mostaghimi A, Ramachandran SM, et al. Neutrophilic dermatoses: a clinical update. Curr Dermatol Rep. 2022;11(2):89-102. https://doi.org/10.1007/s13671-022-00355-8
Marzano AV, Borghi A, Wallach D, Cugno M. A comprehensive review of neutrophilic diseases. Clin Rev Allergy Immunol. 2018;54:114-130.
Maronese CA, Pimentel MA, Li MM, Genovese G, Ortega-Loayza AG, Marzano AV. Pyoderma gangrenosum: an updated literature review on established and emerging pharmacological treatments. Am J Clin Dermatol. 2022;23(5):615-634. https://doi.org/10.1007/s40257-022-00699-8
Ashchyan HJ, Nelson CA, Stephen S, James WD, Micheletti RG, Rosenbach M. Neutrophilic dermatoses: pyoderma gangrenosum and other bowel- and arthritis-associated neutrophilic dermatoses. J Am Acad Dermatol. 2018;79(6):1009-1022. https://doi.org/10.1016/j.jaad.2017.11.063
Langan SM, Groves RW, Card TR, Gulliford MC. Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. J Invest Dermatol. 2012;132(9):2166-2170. https://doi.org/10.1038/jid.2012.130
Marzano AV, Borghi A, Meroni PL, Cugno M. Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation. Br J Dermatol. 2016;175(5):882-891. https://doi.org/10.1111/bjd.14691
Saraceno R, Babino G, Chiricozzi A, Zangrilli A, Chimenti S. PsAPASH: a new syndrome associated with hidradenitis suppurativa with response to tumor necrosis factor inhibition. J Am Acad Dermatol. 2015;72(1):e42-e44. https://doi.org/10.1016/j.jaad.2014.10.002
Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH syndromes: pathophysiology, presentation and treatment. Am J Clin Dermatol. 2017;18(4):555-562. https://doi.org/10.1007/s40257-017-0265-1
Ma M, Wu X, Cao Y, Liu Y, Liu S, Li C. Synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome associated with pyoderma gangrenosum: report of two patients. Int J Dermatol. 2023;62(3):e186-e189. https://doi.org/10.1111/ijd.16492
Marzano AV, Ortega-Loayza AG, Heath M, Morse D, Genovese G, Cugno M. Mechanisms of inflammation in neutrophil-mediated skin diseases. Front Immunol. 2019;10:1059. https://doi.org/10.3389/fimmu.2019.01059
Marzano AV, Damiani G, Genovese G, Gattorno M. A dermatologic perspective on autoinflammatory diseases. Clin Exp Rheumatol. 2018;36 Suppl 110(1):32-38.
Moura RR, Brandão L, Moltrasio C, Agrelli A, Tricarico PM, Maronese CA, et al. Different molecular pathways are disrupted in Pyoderma gangrenosum patients and are associated with the severity of the disease. Sci Rep. 2023;13(1):4919. https://doi.org/10.1038/s41598-023-31914-z
Patel F, Fitzmaurice S, Duong C, He Y, Fergus J, Raychaudhuri SP, et al. Effective strategies for the management of pyoderma gangrenosum: a comprehensive review. Acta Derm Venereol. 2015;95(5):525-531. https://doi.org/10.2340/00015555-2008
Garcovich S, De Simone C, Berti E, Marzano AV. Drug management of neutrophilic dermatoses. Expert Rev Clin Pharmacol. 2017;10(10):1119-1128. https://doi.org/10.1080/17512433.2017.1356719
Jockenhöfer F, Wollina U, Salva KA, Benson S, Dissemond J. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019;180:615-620. https://doi.org/10.1111/bjd.16401
Jockenhöfer F, Klode J, Kröger K, Roesch A, Al Ghazal P, Dissemond J. Patients with pyoderma gangrenosum - analyses of the German DRG data from 2012. Int Wound J. 2016;13(5):951-956. https://doi.org/10.1111/iwj.12463
Bernstein CN, Blanchard JF, Rawsthorne P, Yu N. The prevalence of extraintestinal diseases in inflammatory bowel disease: a population-based study. Am J Gastroenterol. 2001;96(4):1116-1122. https://doi.org/10.1111/j.1572-0241.2001.03756.x
Ampuero J, Rojas-Feria M, Castro-Fernández M, Cano C, Romero-Gómez M. Predictive factors for erythema nodosum and pyoderma gangrenosum in inflammatory bowel disease. J Gastroenterol Hepatol. 2014;29(2):291-295. https://doi.org/10.1111/jgh.12352
Moulton VR. Sex hormones in acquired immunity and autoimmune disease. Front Immunol. 2018;9:2279. https://doi.org/10.3389/fimmu.2018.02279
Kronzer VL, Bridges SL Jr, Davis JM 3rd. Why women have more autoimmune diseases than men: an evolutionary perspective. Evol Appl. 2020;14(3):629-633. https://doi.org/10.1111/eva.13167
Binus AM, Qureshi AA, Li VW, Winterfield LS. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011;165(6):1244-1250. https://doi.org/10.1111/j.1365-2133.2011.10565.x
Riyaz N, Mary V, Sasidharanpillai S, Roshin RA, Snigdha O, Latheef EN, et al. Pyoderma gangrenosum: a clinico-epidemiological study. Indian J Dermatol Venereol Leprol. 2017;83(1):33-39. https://doi.org/10.4103/0378-6323.188654
Brooklyn TN, Dunnill MG, Shetty A, Bowden JJ, Williams JD, Griffiths CE, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomized, double blind, placebo-controlled trial. Gut. 2006;55(4):505-509. https://doi.org/10.1136/gut.2005.074815
Mooij JE, van Rappard DC, Mekkes JR. Six patients with pyoderma gangrenosum successfully treated with infliximab. Int J Dermatol. 2013;52(11):1418-1420. https://doi.org/10.1111/j.1365-4632.2011.05201.x
De la Morena F, Martin L, Gisbert JP, Fernandez Herrera J, Goiriz R. Refractory and infected pyoderma gangrenosum in a patient with ulcerative colitis: response to infliximab. Inflamm Bowel Dis. 2007;13:509-510.
Kouklakis G, Moschos J, Leontiadis GI, Kadis S, Mpoumponaris A, Molyvas E, et al. Infliximab for treatment of pyoderma gangrenosum associated with clinically inactive Crohn's disease. A case report. Rom J Gastroenterol. 2005;14:401-403.
Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985;55(217):173-186.
von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137(6):1000-1005.
Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP. Pyoderma gangrenosum: a comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore). 2000;79:37-46.
Mlika RB, Riahi I, Fenniche S, Mokni M, Raouf Dhaoui M, Dess N, et al. Pyoderma gangrenosum: a report of 21 cases. Int J Dermatol. 2002;41:65-68.
Vidal D, Puig L, Gilaberte M, Alomar A. Review of 26 cases of classical pyoderma gangrenosum: clinical and therapeutic features. J Dermatolog Treat. 2004;15:146-152.
Marzano AV, Trevisan V, Lazzari R, Crosti C. Pyoderma gangrenosum: study of 21 patients and proposal of a “clinicotherapeutic” classification. J Dermatolog Treat. 2011;22:254-260.
Hasselmann DO, Bens G, Tilgen W, Reichrath J. Pyoderma gangrenosum: clinical presentation and outcome in 18 cases and review of the literature. J Dtsch Dermatol Ges. 2007;5:560-564.
Al Ghazal P, Körber A, Klode J, Dissemond J. Investigation of new co-factors in 49 patients with pyoderma gangrenosum. Dtsch Dermatol Ges. 2012;10:251-257.
Al Ghazal P, Herberger K, Schaller J, Strölin A, Hoff N-P, Goerge T, et al. Associated factors and comorbidities in patients with pyoderma gangrenosum in Germany: a retrospective multicentric analysis in 259 patients. Orphanet J Rare Dis. 2013;8:136.
Saracino A, Kelly R, Liew D, Chong A. Pyoderma gangrenosum requiring inpatient management: a report of 26 cases with follow up. Australas J Dermatol. 2011;52:218-221.
Pereira N, Brites MM, Goncalo M, Tellechea O, Figueiredo A. Pyoderma gangrenosum: a review of 24 cases observed over 10 years. Int J Dermatol. 2013;52:938-945.
Ye MJ, Ye JM. Pyoderma gangrenosum: a review of clinical features and outcomes of 23 cases requiring inpatient management. Dermatol Res Pract. 2014;2014:461467.
Cabalag MS, Wasiak J, Lim SW, Raiola FB. Inpatient management of pyoderma gangrenosum: treatments, outcomes, and clinical implications. Ann Plast Surg. 2015;74:354-360.
Adışen E, Erduran F, Gürer MA. Pyoderma gangrenosum: a report of 27 patients. Int J Low Extrem Wounds. 2016;15(2):148-154. https://doi.org/10.1177/1534734616639172
Jockenhofer F, Herberger K, Schaller J, Hohaus KC, StoffelsWeindorf M, Ghazal PA, et al. Tricenter analysis of cofactors and comorbidity in patients with pyoderma gangrenosum. J Dtsch Dermatol Ges. 2016;14:1023-1030.
Inoue S, Furuta JI, Fujisawa Y, Onizawa S, Ito S, Sakiyama M, et al. Pyoderma gangrenosum and underlying diseases in Japanese patients: a regional long-term study. J Dermatol. 2017;44:1281-1284.
Vacas AS, Torre AC, Bollea-Garlatti ML, Warley F, Galimberti RL. Pyoderma gangrenosum: clinical characteristics, associated diseases, and responses to treatment in a retrospective cohort study of 31 patients. Int J Dermatol. 2017;56:386-391.
Kikuchi N, Yamamoto T. Clinical analysis of pyoderma gangrenosum: 41 Japanese cases over 15 years. Eur J Dermatol. 2018;28(2):230-232. https://doi.org/10.1684/ejd.2017.3204
Ashchyan HJ, Butler DC, Nelson CA, Noe MH, Tsiaras WG, Lockwood SJ, et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol. 2018;154(4):409-413. https://doi.org/10.1001/jamadermatol.2017.5978
Schøsler L, Fogh K, Bech R. Pyoderma gangrenosum: a retrospective study of clinical charac-teristics, comorbidities, response to treatment and mortality related to prednisone dose. Acta Derm Venereol. 2021;101(4):adv00431. https://doi.org/10.2340/00015555-3776
Zhao H, Mostaghimi A, Sisley JM, Ortega-Loayza AG. Multilesional pyoderma gangrenosum is associated with longer healing times and higher rates of underlying comorbidities: a single-center prospective registry study. JAAD Int. 2023;12:133-135. https://doi.org/10.1016/j.jdin.2023.05.009