Pyoderma Gangrenosum: Retrospective Evaluation of Clinical Features and Treatment Responses in 23 Cases

Pyoderma Gangrenosum: Retrospective Evaluation of Clinical Features and Treatment Responses in 23 Cases

Pyoderma gangrenosum (PG) is a rare inflammatory disease in the spectrum of neutrophilic diseases characterized by rapidly progressive, painful and large ulcers. In this study, we aimed to investigate the relationship between the clinical characteristics and treatment responses of patients diagnosed with PG, treated and followed up in our clinic. Between 2018 and 2023, 23 patients diagnosed with PG in our clinic were included in the study. Medical file records were retrospectively analyzed and various demographic and clinical characteristics, comorbid systemic diseases, treatment protocols and treatment responses of the patients were recorded. Twelve (52.2%) of the patients were female and 11 (47.8%) were male, with a mean age of 42.4±12.9 years. The median disease duration was 52 months (range: 10-540 months). Systemic diseases accompanying PG were present in 14 patients (60.9%). Systemic steroids were the most common treatment (43.5%). Other treatment approaches included adalimumab, infliximab, cyclosporine, oral doxycycline, topical tacrolimus, topical corticosteroids and intralesional steroids. Remission was achieved in 14 patients (60.9%), relapse was observed in 5 patients (21.74%), and no remission/relapse information was available for 4 patients (17.4%) who lost follow-up. The median duration of remission was 23 months (range: 3-96 months). Although systemic steroids are most commonly used in the treatment of pyoderma gangrenosum, it should be kept in mind that there are various treatment options ranging from systemic and topical anti-inflammatory therapies to biologic agents according to close follow-up of the patients and additional systemic diseases.

___

  • [1]Langan, S.M., Groves, R.W., Card, T.R., Gulliford, M.C. (2012). Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. J Invest Dermatol. 132(9):2166-2170. DOI: 10.1038/jid.2012.130.
  • [2] Braun–Falco, M., Kovnerystyy, O., Lohse, P., Ruzicka, T. (2012). Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH) – a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 66(3):409-415. DOI: 10.1016/j.jaad.2010.12.025.
  • [3]Ortega-Loayza, A.G., Nugent, W.H., Lucero, O.M., Washington, S.L., Nunley, J.R., Walsh, S.W. (2018). Dysregulation of inflammatory gene expression in lesional and nonlesional skin of patients with pyoderma gangrenosum. Br J Dermatol. 178(1):e35–e36. DOI: 10.1111/bjd.15837.
  • [4]Ortega-Loayza, A.G., Friedman, M.A., Reese, A.M., Liu, Y., Greiling, T.M., Cassidy, P.B., … rosenbaum, J.T. (2022). Molecular and cellular characterization of pyoderma gangrenosum: implications for the use of gene expression. J Investig Dermatol. 142(4):1217-1220. DOI: 10.1016/j.jid.2021.08.431.
  • [5]Weenig, R.H., Davis, M.D., Dahl, P.R., Su, W.P.D. (2002). Skin ulcers misdiagnosed as pyoderma gangrenosum. N Engl J Med. 347:1412–1418. DOI: 10.1056/NEJMoa013383.
  • [6]Chevrant-Breton, J., Logeais, B., Pibouin, M. (1989). Pyoderma gangrenosum. Ann Dermatol Venereol. 116: 577–589.
  • [7]Ormerod, A.D., Thomas, K.S., Craig, F.E., Mitchell, E., Greenlaw, M., Norrie, J….Willams, H.C. (2015). Comparison of the two most commonly used treatments for pyoderma gangrenosum: results of the STOP GAP randomised controlled trial. BMJ. 350:h2958. DOI: 10.1136/bmj.h2958.
  • [8]Ben Abdallah, H., Fogh, K., Bech, R. (2019). Pyoderma gangrenosum and tumour necrosis factor alpha inhibitors: a semi-systematic review. Int Wound J. 16(2):511–521. DOI: 10.1111/iwj.13067.
  • [9]Mlika, R.B., Riahi, I., Fenniche, S., Mokni, M., Dhaoui, M.R., Dess, N., …Mokhtar, I. (2022). Pyoderma gangrenosum: a report of 21 cases. Int J Dermatol. 41:65-8. DOI: 10.1046/j.1365-4362.2002.01329.x.
  • [10]Ehling, A., Karrer, S., Klebl, F., Schäffler, A., Müller-Ladner, U. (2004). Therapeutic management of pyoderma gangrenosum. Arthritis Rheum. 50:3076-3084. DOI: 10.1002/art.20559.
  • [11]von den Driesch, P. (1997). Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 137:1000-1005.
  • [12]Güngör, E., Karakayali, G., Alli, N., Artüz, F., Lenk, N. (1999). Penile pyoderma gangrenosum. J Eur Acad Dermatol Venereol. 12:59-62.
  • [13]Farrell, A.M., Black, M.M., Bracka, A., Bunker, C.B. (1998). Pyoderma gangrenosum of the penis. Br J Dermatol. 138:337-340. DOI: 10.1046/j.1365-2133.1998.02087.x.
  • [14]Brown, T.S., Marshall, G.S., Callen, J.P. (2000). Cavitating pulmonary infiltrate in an adolescent with pyoderma gangrenosum: A rarely recognized extracutaneous manifestation of a neutrophilic dermatosis. J Am Acad Dermatol. 43:108-112. DOI: 10.1067/mjd.2000.103627.
  • [15]Richetta, A.G., Maiani, E., Carboni, V, Carlomagno, V., Cimillo, M., Mattozzi, C., Calvieri, Z. (2007). Pyoderma gangrenosum: case series. Clin Ter. 158:325-329.
  • [16]Powell, F.C., Su, W.P., Perry, H.O. (1996). Pyoderma gangrenosum: classification and management. J Am Acad Dermatol. 34:395-409;410-412. DOI: 10.1016/s0190-9622(96)90428-4.
  • [17]Ahmadi, S., Powell, F.C. (2005). Pyoderma gangrenosum: uncommon presentations. Clin Dermatol. 23:612-620. DOI: 10.1016/j.clindermatol.2005.01.014.
  • [18]Kim, T.H., Oh, S.Y., Myung, S.C. (2009). Pyoderma gangrenosum of the penis. J Korean Med Sci. 24:1200-1202. DOI: 10.3346/jkms.2009.24.6.1200.
  • [19]Reichrath, J., Bens, G., Bonowitz, A., Tilgen, W. (2005). Treatment recommendations for pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 53:273-283. DOI: 10.1016/j.jaad.2004.10.006.
  • [20]Ah-Weng, A., Langtry, J.A., Velangi, S., Evans, C.D., Douglas, W.S. (2005). Pyoderma gangrenosum associated with hidradenitis suppurativa. Clin Exp Dermatol. 30:669-671. DOI: 10.1111/j.1365-2230.2005.01897.x.
  • [21]Haim, S., Friedman-Birnbaum, R. (1976). Pyoderma gangrenosum in immunosuppressed patients. Dermatologica. 153:44-48. DOI: 10.1159/000251106.
  • [22]Hasselmann, D.O., Bens, G., Tilgen, W., Reichrath, J. (2007). Pyoderma gangrenosum: clinical presentation and outcome in 18 cases and review of the literature. J Dtsch Dermatol Ges. 5:560-564. DOI: 10.1111/j.1610-0387.2007.0328.x.
  • [23]Weedon, D. (2010). The vasculopathic reaction pattern: Pyoderma gangrenosum. Weedon's skin pathology. (3.th ed.), China, Churchill livingstone elsevier196-244.
  • [24]Waldman, M.A., Callen, J.P. (2005). Pyoderma gangrenosum Preceding the Diagnosis of Systemic Lupus erythematosus. Dermatology. 10:64-67. DOI: 10.1159/000081488.
  • [25]Rustin, M.H., Gilkes, J.J., Robinson, T.W. (1990). Pyoderma gangrenosum associated with Behçet's disease: treatment with thalidomide. J Am Acad Dermatol. 23:941-944. DOI: 10.1016/s0190-9622(08)80705-0.
  • [26]Wollina, U. (2002). Clinical management of pyoderma gangrenosum. Am J Clin Dermatol. 3:149-158. DOI: 10.2165/00128071-200203030-00002.
  • [27]Wollina, U. (2007). Pyoderma gangrenosum-a review. Orphanet J Rare Dis. 2:19. DOI: 10.1186/1750-1172-2-19.
  • [28]Trémezaygues, L., Schmaltz, R., Vogt, T., Reichrath, J. (2010). Management of pyoderma gangrenosum. An update on clinical features, diagnosis and therapy. Hautarzt. 61:345-53;354-355. DOI: 10.1007/s00105-009-1909-8.
  • [29]Miller, J., Yentzer, B.A., Clark, A., Jorizzo, J.L., Feldman, S.R. (2010). Pyoderma gangrenosum: a review and update on new therapies. J Am Acad Dermatol. 62:646-54. DOI: 10.1016/j.jaad.2009.05.030.
  • [30]Duarte, A.F., Nogueira, A., Lisboa, C., Azeved,o F. (2209). Pyoderma gangrenosum-clinical, laboratory and therapeutic approaches. Review of 28 cases. Dermatol Online J. 15:3.
  • [31]Coutinho, A.E., Chapman, K.E. (2011). The anti-inflammatory and immunosuppressive effects of glucocorticoids, recent developments and mechanistic insights. Mol Cell Endocrinol. 335(1):2–13. DOI: 10.1016/j.mce.2010.04.005.
  • [32]Kolios, A.G.A., Gubeli, A., Meier, B., Maul, J.T., Kündig, T., Nilsson, J….Cozzio, A. (2017). Clinical disease patterns in a regional Swiss cohort of 34 pyoderma gangrenosum patients. Dermatology. 233(4):268–276. DOI: 10.1159/000481432.
  • [33]Maronese, C.A., Pimental, M.A., Li, M.M., Genovese, G., Ortega-Laoyza, A.G., Marzano, A.V. (2022). Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments. Am J Clin Drmatol. 23(5):615-634. DOI: 10.1007/s40257-022-00699-8.
  • [34]Henehan, M., Montuno, M., De Benedetto, A. (2017). Doxycycline as an anti-inflammatory agent: updates in dermatology. J Eur Acad Dermatol Venereol. 31(11):1800-1808. DOI: 10.1111/jdv.14345.
  • [35]Anuset, D., Reguiai, Z., Perceau, G., Colomb, M., Durlach, A., Bernard, P. (2016). Caractéristiques cliniques et traitement du pyoderma gangrenosum dans la Marne. Ann Dermatol Venereol. 143(2):108-117. DOI: 10.1016/j.annder.2015.10.593.
  • [36]Kontos, A.P., Kerr, H.A., Fivenson, D.P., Remishofsky, C., Jacobsen, G. (2006). An open-label study of topical tacrolimus ointment 0.1% under occlusion ort he treatment of pyoderma gangrenosum. Int J Dermatol. 45(11):1383-1385. DOI: 10.1111/j.1365-4632.2006.03133.x.
  • [37]Lyon, C.C., Stapleton, M., Smith, A.J., Mendelsohn, S., Beck, M.H., Griffiths, C.E. (2001). Topical tacrolimus in the management of peristomal pyoderma gangrenosum. J Dermatolog Treat. 12(1):13-17. DOI: 10.1080/095466301750163518.
  • [38]Maronese,C.A., Pimente,l M.A., Li, M.M., Genovese, G., Ortega-Loayza, A.G., Marzano, A.V. (2022). Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments. Am J Clin Dermatol. 23(5):615-634. DOI: 10.1007/s40257-022-00699-8.
  • [39]Brooklyn, T.N., Dunnill, M.G., Shetty, A., Bowden, J.J., Williams, J.D.L., Griffiths, C.E.M….Probert, C.S. (2006). Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 55(4):505–509. DOI: 10.1136/gut.2005.074815.
  • [40]Yamamoto, T. (2021). An update on adalimumab for pyoderma gangrenosum. Drugs Today (Barc). 57(9):535–42. DOI: 10.1358/dot.2021.57.9.3293619.