Abstract
Pyoderma gangrenosum (PG) is a rare noninfectious inflammatory disease in the spectrum of the neutrophilic dermatoses. PG can be idiopathic or occurs in association with different factors. Underlying systemic diseases are found in up to 50% of the cases with PG. PG occurs most commonly on the pretibial region, but has been reported on other sites of the body as well, including breast, hand, trunk, head and neck, and peristomal skin. There are several PG subtypes: classical, bullous, superficial granulomatous, pustular, peristomal, and malignant. Biopsy is recommended to rule out clinical mimickers such as vasculitis, leg ulcers, and necrotizing fasciitis. Treatment of PG is selected according to severity and rate of progression. Topical treatment methods include wound care, local corticosteroids, and calcineurin inhibitors. Systemical corticosteroids are the cornerstone in the treatment scheme. Additionally, Cyclosporine, Azathioprine, intravenous immunoglobulin, Clofazemine, Thalidomide, Dapsone, and Colchicine can be used either alone or in combination with corticosteroids. Biologics use in PG is “off label” option, and there is still no consensus about the application of these drugs in the treatment of PG.
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Further Reading
Alberts JH, Sams HH, Miller JL, et al. Familial ulcerative pyoderma gangrenosum: a report of 2 kindred. Cutis. 2002;69:427–30.
Ashchyan HJ, Butler DC, Nelson CA, et al. The association of age with clinical presentation and comorbidities of pyoderma gangrenosum. JAMA Dermatol. 2019;154(4):409–13.
Binus AM, Qureshi AA, Li VW, et al. Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients. Br J Dermatol. 2011;165:1244–50.
Braun-Falco M, Kovnerystyy O, Lohse P, et al. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH)—a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2012;66:409–15.
Brocq L. Nouvelle contribution a l’etude du phagedenisme geometrique. Ann Dermatol Syphilogr (Paris). 1916;6:1–39.
Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma (ecthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol Syph. 1930;22:655–80.
Fletcher J, Alhusayen R, Alavi A. Recent advances in managing and understanding pyoderma gangrenosum. F1000Res. 2019;8:F1000 Faculty Rev-2092.
Graham JA, Hansen KK, Rabinowitz LG, et al. Pyoderma gangrenosum in infants and children. Pediatr Dermatol. 1994;11:10–7.
Haag CK, Ortega-loayza AG, Latour E, et al. Clinical factors influencing the response to intravenous immunoglobulin treatment in cases of treatment-resistant pyoderma gangrenosum. J Dermatolog Treat. 2019;0(0):1–4.
Jockenhöfer F, Wollina U, Salva K, et al. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019;180(3):615–20.
Langan SM, Groves RW, Card TR, et al. Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study. J Invest Dermatol. 2012;132:2166–70.
Levell NJ, Skellett AM, Chriba M. Beclomethasone inhaler used to treat pyoderma gangrenosum. Clin Exp Dermatol. 2010;35:337–8.
Madke B, Kar S, Yadav N. Newly described signs in dermatology. Indian Dermatol Online J. 2015;6:220–1.
Martinez-Rios C, Jariwala MP, Highmore K, et al. Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature. Pediatr Radiol. 2019 Jan;49(1):23–36.
Marzano AV, Fanoni D, Antiga E, et al. Expression of cytokines, chemokines and other effector molecules in two prototypic autoinflammatory skin diseases, pyoderma gangrenosum and Sweet’s syndrome. Clin Exp Immunol. 2014;178:48–56.
Maverakis E, Ma C, Shinkai K, et al. Diagnostic criteria of ulcerative pyoderma gangrenosum: a delphi consensus of international experts. JAMA Dermatol. 2018;154(4):461–6.
Mazokopakis EE, Kofteridis DP, Pateromihelaki AJ, et al. Improvement of ulcerative pyoderma gangrenosum with hyperbaric oxygen therapy. Dermatol Ther. 2011;24:134–6.
Oka M. Pyoderma gangrenosum and interleukin 8. Br J Dermatol. 2007;157:1279–81.
Reichrath J, Bens G, Bonowitz A, et al. Treatment recommendations for pyoderma gangrenosum: an evidence–based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005;53:273–83.
Su WPD, Davis MDP, Weenig RH, et al. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol. 2004;43:790–800.
Wollina U. Pyoderma gangrenosum—a review. Orphanet J Rare Dis. 2007;2:19.
Yamamoto T. Epidemiology of pyoderma gangrenosum in Japanese patients by questionnaire survey. J Dermatol. 2019;46(4):e145–6.
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Kazandjieva, J., Tsankov, N. (2023). Pyoderma Gangrenosum. In: Katsambas, A.D., Lotti, T.M., Dessinioti, C., D'Erme, A.M. (eds) European Handbook of Dermatological Treatments. Springer, Cham. https://doi.org/10.1007/978-3-031-15130-9_80
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