Top

Published in:

01-12-2017 | Original Paper

Oral Pyoderma Gangrenosum: Diagnosis, Treatment and Challenges: A Systematic Review

Authors: Caroline Bissonnette, Adel Kauzman, Gisele N. Mainville

Published in: Head and Neck Pathology | Issue 4/2017

Abstract

Pyoderma gangrenosum (PG) is a distinctive ulcerative skin disorder of unknown etiology, associated with an underlying systemic disease in up to 70% of cases. The condition is characterized by the appearance of one or more necrotic ulcers with a ragged undermined violaceous border and surrounding erythema. Lesions are often initiated by minor trauma. The condition can affect any anatomical site, however the head and neck are rarely involved. Although the oral cavity is subject to recurrent minor trauma through everyday activities such as mastication and oral hygiene, as well as during dental treatment, oral lesions appear to be extremely rare. In an effort to provide a detailed explanation of the oral manifestations of PG, a systematic search was conducted using medical databases. A total of 20 cases of PG with oral involvement were reported in the English and French literature. The objectives of this article are to present the pertinent diagnostic criteria and to discuss the differential diagnosis and therapeutic modalities.

Powell FC, Schroeter AL, Su WP, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med. 1985;55(217):173–86.PubMed

Su WP, Davis MD, Weenig RH, Powell FC, Perry HO. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria. Int J Dermatol. 2004;43(11):790–800. doi:10.1111/j.1365-4632.2004.02128.x.CrossRefPubMed

von den Driesch P. Pyoderma gangrenosum: a report of 44 cases with follow-up. Br J Dermatol. 1997;137(6):1000–5.CrossRef

Kiran RP, O’Brien-Ermlich B, Achkar JP, Fazio VW, Delaney CP. Management of peristomal Pyoderma gangrenosum. Dis Colon Rectum. 2005;48(7):1397–403. doi:10.1007/s10350-004-0944-x.CrossRefPubMed

Setterfield JF, Shirlaw PJ, Challacombe SJ, Black MM. Pyoderma gangrenosum associated with severe oropharyngeal involvement and IgA paraproteinaemia. Br J Dermatol. 2001;144(2):393–6.CrossRefPubMed

Lazarus GS, Goldsmith LA, Rocklin RE, Pinals RS, de Buisseret JP, David JR. Pyoderma gangrenosum, altered delayed hypersensitivity and polyarthritis. Arch Dermatol. 1972;105(1):46–51.CrossRefPubMed

Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: classification and management. J Am Acad Dermatol. 1996;34(3):395–409 (quiz 10–2).CrossRefPubMed

Braswell SF, Kostopoulos TC, Ortega-Loayza AG. Pathophysiology of Pyoderma gangrenosum (PG): an updated review. J Am Acad Dermatol. 2015;73(4):691–8. doi:10.1016/j.jaad.2015.06.021.CrossRefPubMed

Bhat RM. Pyoderma gangrenosum: An update. Indian Dermatol Online J. 2012;3(1):7–13. doi:10.4103/2229-5178.93482.CrossRefPubMedPubMedCentral

10.

Yco MS, Warnock GR, Cruickshank JC, Burnett JR. Pyoderma gangrenosum involving the head and neck. Laryngoscope. 1988;98(7):765–8. doi:10.1288/00005537-198807000-00016.CrossRefPubMed

11.

Al Ghazal P, Dissemond J. Multilocular Pyoderma gangrenosum after uterus resection. Chirurg. 2012;83(3):254–7.CrossRefPubMed

12.

Basu MK, Asquith P. Oral manifestations of inflammatory bowel disease. Clin Gastroenterol. 1980;9(2):307–21.PubMed

13.

Drinda S, Oelzner P, Codina Canet C, Kaatz M, Wolf G, Hein G. Fatal outcome of Pyoderma gangrenosum with multiple organ involvement and partially responding to Infliximab. Cent Eur J Med. 2006;1(3):306–12. doi:10.2478/s11536-006-0024-9.

14.

Margoles JS, Wenger J. Stomal ulceration associated with Pyoderma gangrenosum and chronic ulcerative colitis. Report of two cases. Gastroenterology. 1961;41:594–8.PubMed

15.

Tsuboi H. Case of Pyoderma gangrenosum showing oral and genital ulcers, misdiagnosed as Behcet’s disease at first medical examination. J Dermatol. 2008;35(5):289–92. doi:10.1111/j.1346-8138.2008.00468.x.CrossRefPubMed

16.

Goulden V, Bond L, Highet AS. Pyoderma gangrenosum associated with paroxysmal nocturnal haemoglobinuria. Clin Exp Dermatol. 1994;19(3):271–3.CrossRefPubMed

17.

Hernandez-Martin A, Arias-Palomo D, Hermida G, Gutierrez-Ortega MC, Ramirez-Herrera M, Rodriguez-Vegas M, et al. Oral Pyoderma gangrenosum. Br J Dermatol. 2003;149(3):663–4.CrossRefPubMed

18.

Park HJ, Han BG, Kim YC, Cinn YW. Recalcitrant oral Pyoderma gangrenosum in a child responsive to cyclosporine. J Dermatol. 2003;30(8):612–6.CrossRefPubMed

19.

Buckley C, Bayoumi AH, Sarkany I. Pyoderma gangrenosum with severe pharyngeal ulceration. J R Soc Med. 1990;83(9):590–1.PubMedPubMedCentral

20.

Poiraud C, Gagey-Caron V, Barbarot S, Durant C, Ayari S, Stalder JF. Cutaneous, mucosal and systemic Pyoderma gangrenosum. Ann Dermatol Venereol. 2010;137(3):212–5. doi:10.1016/j.annder.2010.01.007.CrossRefPubMed

21.

Curi MM, Cardoso CL, Koga DH, Zardetto C, Araújo SR. Pyoderma gangrenosum affecting the mouth. Open J Stomatol. 2013;3(2):4.CrossRef

22.

Verma S, Field S, Murphy G. Photoletter to the editor: oral ulceration in Pyoderma gangrenosum. J Dermatol Case Rep. 2011;5(2):34–5. doi:10.3315/jdcr.2011.1070.CrossRefPubMedPubMedCentral

23.

Chariatte N, Lysitsa S, Lombardi T, Samson J. Pyoderma gangrenosum (2ème partie): manifestations buccales et présentation d’un cas. Med Buccale Chir Buccale. 2011;17(3):225–35.CrossRef

24.

Paramkusam G, Meduri V, Gangeshetty N. Pyoderma gangrenosum with oral involvement: case report and review of the literature. Int J Oral Sci. 2010;2(2):111–6. doi:10.4248/ijos10032.CrossRefPubMedPubMedCentral

25.

Bertram-Callens A, Machet L, Vaillant L, Camenen I, Lorette G. Buccal and ocular localizations of Pyoderma gangrenosum in Vaquez’s disease. Ann Dermatol Venereol. 1991;118(9):611–4.PubMed

26.

Kennedy KS, Prendergast ML, Sooy CD. Pyoderma gangrenosum of the oral cavity, nose, and larynx. Otolaryngol Head Neck Surg. 1987;97(5):487–90.CrossRefPubMed

27.

Yusuf H, Ead RD. Pyoderma gangrenosum with involvement of the tongue. Br J Oral Maxillofac Surg. 1985;23(4):247–50.CrossRefPubMed

28.

Zampeli VA, Lippert U, Nikolakis G, Makrantonaki E, Tzellos TG, Krause U, et al. Disseminated refractory pyoderma gangraenosum during an ulcerative colitis flare. Treatment with infliximab. J Dermatol Case Rep. 2015;9(3):62–6. doi:10.3315/jdcr.2015.1206.CrossRefPubMedPubMedCentral

29.

Al Attas KM, Ahsan MK, Buraik M, Gamal AM, Hannani HY. Fatal pyoderma gangrenosum in a patient with inflammatory bowel disease and hypogonadotropic hypogonadism: case report. J Saudi Soc Dermatol Dermatol Surg. 2013;17(2):4.CrossRef

30.

Isomura I, Miyawaki S, Morita A. Pyoderma gangrenosum associated with nasal septal perforation, oropharyngeal ulcers and IgA paraproteinemia. J Dermatol. 2005;32(3):193–8.CrossRefPubMed

31.

Kaomongkolgit R, Subbalekha K, Sawangarun W, Thongprasom K. Pyoderma gangrenosum-like oral ulcerations in an elderly patient. Gerodontology. 2015;32(4):309–13. doi:10.1111/ger.12158.CrossRefPubMed

32.

Siddiqui S, Fiorillo M, Tismenetsky M, Spinnell M. Isolated oral Pyoderma gangrenosum following proctocolectomy. Am J Gastroenterol. 2012;107:S517–S8.

33.

Callen JP, Taylor WB. Pyoderma gangrenosum: a literature review. Cutis. 1978;21(1):61–4.PubMed

34.

Philpott JA Jr, Goltz RW, Park RK. Pyoderma gangrenosum, rheumatoid arthritis, and diabetes mellitus. Arch Dermatol. 1966;94(6):732–8.CrossRefPubMed

35.

DeFilippis EM, Feldman SR, Huang WW. The genetics of Pyoderma gangrenosum and implications for treatment: a systematic review. Br J Dermatol. 2015;172(6):1487–97. doi:10.1111/bjd.13493.CrossRefPubMed

36.

Tolkachjov SN, Fahy AS, Wetter DA, Brough KR, Bridges AG, Davis MD, et al. Postoperative Pyoderma gangrenosum (PG): the Mayo Clinic experience of 20 years from 1994 through 2014. J Am Acad Dermatol. 2015;73(4):615–22. doi:10.1016/j.jaad.2015.06.054.CrossRefPubMed

37.

Braun-Falco M, Kovnerystyy O, Lohse P, Ruzicka T. Pyoderma gangrenosum, acne, and suppurative hidradenitis (PASH): a new autoinflammatory syndrome distinct from PAPA syndrome. J Am Acad Dermatol. 2012;66(3):409–15. doi:10.1016/j.jaad.2010.12.025.CrossRefPubMed

38.

Hong JB, Su YN, Chiu HC. Pyogenic arthritis, Pyoderma gangrenosum, and acne syndrome (PAPA syndrome): report of a sporadic case without an identifiable mutation in the CD2BP1 gene. J Am Acad Dermatol. 2009;61(3):533–5. doi:10.1016/j.jaad.2008.11.017.CrossRefPubMed

39.

Calderon-Castrat X, Bancalari-Diaz D, Roman-Curto C, Romo-Melgar A, Amoros-Cerdan D, L AA-M, et al. PSTPIP1 gene mutation in a Pyoderma gangrenosum, acne and suppurative hidradenitis (PASH) syndrome. Br J Dermatol. 2015. doi:10.1111/bjd.14383.

40.

Marzano AV, Trevisan V, Gattorno M, Ceccherini I, De Simone C, Crosti C. Pyogenic arthritis, Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH): a new autoinflammatory syndrome associated with a novel mutation of the PSTPIP1 gene. JAMA Dermatol. 2013;149(6):762–4. doi:10.1001/jamadermatol.2013.2907.CrossRefPubMed

41.

Neville BW, Damm DD, Allen CM, Chi AC. Oral and maxillofacial pathology. 4th ed. St. Louis: W. B. Saunders; 2016.

42.

Hirshberg A, Amariglio N, Akrish S, Yahalom R, Rosenbaum H, Okon E, et al. Traumatic ulcerative granuloma with stromal eosinophilia: a reactive lesion of the oral mucosa. Am J Clin Pathol. 2006;126(4):522–9. doi:10.1309/afha406gbt0n2y64.CrossRefPubMed

43.

Sirois DA, Miller AS, Harwick RD, Vonderheid EC. Oral manifestations of cutaneous T cell lymphoma. A report of eight cases. Oral Surg Oral Med Oral Pathol. 1993;75(6):700–5.CrossRefPubMed

44.

Comfere NI, Macaron NC, Gibson LE. Cutaneous manifestations of Wegener’s granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol. 2007;34(10):739–47. doi:10.1111/j.1600-0560.2006.00699.x.CrossRefPubMed

45.

Wang WC, Chen JY, Chen YK, Lin LM. Tuberculosis of the head and neck: a review of 20 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009;107(3):381–6. doi:10.1016/j.tripleo.2008.11.002.CrossRefPubMed

46.

Wallach D, Vignon-Pennamen MD. Pyoderma gangrenosum and Sweet syndrome: the prototypic neutrophilic dermatoses. Br J Dermatol. 2015. doi:10.1111/bjd.13955.PubMed

47.

Amouri M, Masmoudi A, Ammar M, Boudaya S, Khabir A, Boudawara T, et al. Sweet’s syndrome: a retrospective study of 90 cases from a tertiary care center. Int J Dermatol. 2016. doi:10.1111/ijd.13232.PubMed

48.

The International Criteria for Behcet’s Disease (ICBD). A collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338–47. doi:10.1111/jdv.12107.CrossRef

49.

Gibson LE, Daoud MS, Muller SA, Perry HO. Malignant pyodermas revisited. Mayo Clin Proc. 1997;72(8):734–6. doi:10.1016/s0025-6196(11)63593-3.CrossRefPubMed

50.

Reichrath J, Bens G, Bonowitz A, Tilgen W. Treatment recommendations for Pyoderma gangrenosum: an evidence-based review of the literature based on more than 350 patients. J Am Acad Dermatol. 2005;53(2):273–83. doi:10.1016/j.jaad.2004.10.006.CrossRefPubMed

51.

Zuo KJ, Fung E, Tredget EE, Lin AN. A systematic review of post-surgical Pyoderma gangrenosum: identification of risk factors and proposed management strategy. J Plast Reconstr Aesthet Surg. 2015;68(3):295–303. doi:10.1016/j.bjps.2014.12.036.CrossRefPubMed

52.

Marzano AV, Alberti-Violetti S, Crippa R, Angiero F, Tadini G, Crosti C. Pyoderma gangrenosum with severe cutaneous and oral involvement. Eur J Dermatol. 2013;23(2):257–8. doi:10.1684/ejd.2013.1968.PubMed

53.

Snyder RA. Pyoderma gangrenosum involving the head and neck. Arch Dermatol. 1986;122(3):295–302.CrossRefPubMed

54.

Baloch BK, Baloch SK, Kumar S, Mansoor F, Jawad A. Orogenital ulcers of Pyoderma gangrenosum resembling sexually transmitted disease. J Coll Physicians Surg Pak. 2014;24 Suppl 3:S207–8.PubMed

55.

Ormerod AD, Thomas KS, Craig FE, Mitchell E, Greenlaw N, Norrie J, et al. Comparison of the two most commonly used treatments for Pyoderma gangrenosum: results of the STOP GAP randomised controlled trial. Bmj. 2015;350:h2958. doi:10.1136/bmj.h2958.CrossRefPubMedPubMedCentral

56.

Vavricka SR, Schoepfer A, Scharl M, Lakatos PL, Navarini A, Rogler G. Extraintestinal manifestations of inflammatory bowel disease. Inflamm Bowel Dis. 2015;21(8):1982–92. doi:10.1097/mib.0000000000000392.CrossRefPubMedPubMedCentral

57.

Brooklyn TN, Dunnill MG, Shetty A, Bowden JJ, Williams JD, Griffiths CE, et al. Infliximab for the treatment of Pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 2006;55(4):505–9. doi:10.1136/gut.2005.074815.CrossRefPubMedPubMedCentral

58.

Agarwal A, Andrews JM. Systematic review: IBD-associated Pyoderma gangrenosum in the biologic era, the response to therapy. Aliment Pharmacol Ther. 2013;38(6):563–72. doi:10.1111/apt.12431.CrossRefPubMed

59.

Ambooken B, Khader A, Muhammed K, Rajan U, Snigdha O. Malignant Pyoderma gangrenosum eroding the parotid gland successfully treated with dexamethasone pulse therapy. Int J Dermatol. 2014;53(12):1536–8. doi:10.1111/ijd.12519.CrossRefPubMed

60.

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. doi:10.1155/2014/461467.PubMedPubMedCentral

61.

Miller J, Yentzer BA, Clark A, Jorizzo JL, Feldman SR. Pyoderma gangrenosum: a review and update on new therapies. J Am Acad Dermatol. 2010;62(4):646–54. doi:10.1016/j.jaad.2009.05.030.CrossRefPubMed

62.

Brunsting LA, Goeckerman WH, O’Leary PA. Pyoderma (echthyma) gangrenosum: clinical and experimental observations in five cases occurring in adults. Arch Dermatol Syphilol. 1930;22(4):655–80. doi:10.1001/archderm.1930.01440160053009.CrossRef

Title: Oral Pyoderma Gangrenosum: Diagnosis, Treatment and Challenges: A Systematic Review
Authors: Caroline Bissonnette
Adel Kauzman
Gisele N. Mainville
Publication date: 01-12-2017
Publisher: Springer US
Published in: Head and Neck Pathology / Issue 4/2017
Electronic ISSN: 1936-0568
DOI: https://doi.org/10.1007/s12105-017-0804-3

At a glance: The STEP trials

Springer Medicine

Oral Pyoderma Gangrenosum: Diagnosis, Treatment and Challenges: A Systematic Review

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

Please log in to get access to this content

Other articles of this Issue 4/2017

Hyalinizing Clear Cell Carcinoma of the Bronchus

Genetic Characterization of Adenoid Cystic Carcinoma of the Minor Salivary Glands: A Potential Familial Occurrence in First-Degree Relatives

NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review

Pseudomyogenic Hemangioendothelioma: A Vascular Tumor Previously Undescribed in the Oral Cavity

A Combined Epithelial Odontogenic Tumor? A 7-Year Follow-Up Case

Intraoral Salivary Duct Cyst: Clinical and Histopathologic Features of 177 Cases