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Clin Cosmet Investig Dermatol. 2015 May 28;8:285-93. doi: 10.2147/CCID.S61202. eCollection 2015.

Pyoderma gangrenosum: challenges and solutions.

Clinical, cosmetic and investigational dermatology

Ana Gameiro, Neide Pereira, José Carlos Cardoso, Margarida Gonçalo

Affiliations

  1. Dermatology Department, Coimbra University Hospital, Coimbra, Portugal.
  2. Dermatology Department, Centro Hospitalar Cova da Beira, Covilhã, Portugal.

PMID: 26060412 PMCID: PMC4454198 DOI: 10.2147/CCID.S61202

Abstract

Pyoderma gangrenosum (PG) is a rare disease, but commonly related to important morbidity. PG was first assumed to be infectious, but is now considered an inflammatory neutrophilic disease, often associated with autoimmunity, and with chronic inflammatory and neoplastic diseases. Currently, many aspects of the underlying pathophysiology are not well understood, and etiology still remains unknown. PG presents as painful, single or multiple lesions, with several clinical variants, in different locations, with a non specific histology, which makes the diagnosis challenging and often delayed. In the classic ulcerative variant, characterized by ulcers with inflammatory undermined borders, a broad differential diagnosis of malignancy, infection, and vasculitis needs to be considered, making PG a diagnosis of exclusion. Moreover, there are no definitively accepted diagnostic criteria. Treatment is also challenging since, due to its rarity, clinical trials are difficult to perform, and consequently, there is no "gold standard" therapy. Patients frequently require aggressive immunosuppression, often in multidrug regimens that are not standardized. We reviewed the clinical challenges of PG in order to find helpful clues to improve diagnostic accuracy and the treatment options, namely topical care, systemic drugs, and the new emerging therapies that may reduce morbidity.

Keywords: biologics; neutrophilic dermatosis; pyoderma gangrenosum; treatment

References

  1. Int J Dermatol. 2004 Nov;43(11):790-800 - PubMed
  2. J Eur Acad Dermatol Venereol. 2015 Nov;29(11):2243-7 - PubMed
  3. Br J Dermatol. 2015 Aug;173(2):610-2 - PubMed
  4. Int J Immunopathol Pharmacol. 2011 Apr-Jun;24(2):451-60 - PubMed
  5. J Am Acad Dermatol. 2001 Jan;44(1):61-6 - PubMed
  6. Eur Rev Med Pharmacol Sci. 2009 Mar;13 Suppl 1:15-21 - PubMed
  7. J Dtsch Dermatol Ges. 2013 May;11(5):447-9 - PubMed
  8. Gastroenterol Rep (Oxf). 2013 Jul;1(1):1-8 - PubMed
  9. J Oncol Pharm Pract. 2015 Dec;21(6):471-3 - PubMed
  10. Proc R Soc Med. 1960 Apr;53:296-7 - PubMed
  11. J Wound Care. 2009 Dec;18(12 ):521-6 - PubMed
  12. Dermatol Online J. 2014 Jun 15;20(6):null - PubMed
  13. Case Rep Dermatol. 2014 Oct 23;6(3):239-44 - PubMed
  14. An Bras Dermatol. 2012 Jul-Aug;87(4):637-9 - PubMed
  15. Adv Wound Care (New Rochelle). 2012 Oct;1(5):194-199 - PubMed
  16. Int J Dermatol. 2013 Aug;52(8):938-45 - PubMed
  17. Clin Exp Dermatol. 2014 Aug;39(6):750-1 - PubMed
  18. MedGenMed. 2001 Jun 27;3(3):6 - PubMed
  19. Dermatol Res Pract. 2014;2014:461467 - PubMed
  20. J Immunol. 2005 Dec 1;175(11):7678-86 - PubMed
  21. Clin Exp Immunol. 2010 Oct;162(1):100-7 - PubMed
  22. Arch Dermatol. 2008 Jun;144(6):755 - PubMed
  23. J Am Acad Dermatol. 2013 May;68(5):834-53 - PubMed
  24. Clin Exp Immunol. 2014 Oct;178(1):48-56 - PubMed
  25. Am J Clin Dermatol. 2014 Oct;15(5):413-23 - PubMed
  26. Br J Dermatol. 2015 Jun;172(6):1487-97 - PubMed
  27. Ostomy Wound Manage. 2014 Jun;60(6):50-4 - PubMed
  28. Br J Dermatol. 2000 Oct;143(4):728-32 - PubMed
  29. Trends Immunol. 2013 Apr;34(4):174-81 - PubMed
  30. Br J Dermatol. 1997 Dec;137(6):1000-5 - PubMed
  31. J Am Acad Dermatol. 2005 Aug;53(2):273-83 - PubMed
  32. J Clin Rheumatol. 2012 Dec;18(8):413-5 - PubMed
  33. J Dermatolog Treat. 2015 Apr;26(2):171-2 - PubMed
  34. Trials. 2012 Apr 28;13:51 - PubMed
  35. Eur J Dermatol. 2012 Sep-Oct;22(5):711-2 - PubMed
  36. Gut. 2006 Apr;55(4):505-9 - PubMed
  37. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):1008-17 - PubMed
  38. J Eur Acad Dermatol Venereol. 2015 Jun;29(6):1245-7 - PubMed
  39. Int Wound J. 2016 Oct;13(5):1041-2 - PubMed
  40. Dermatol Clin. 2013 Jul;31(3):405-25 - PubMed
  41. Medicine (Baltimore). 2000 Jan;79(1):37-46 - PubMed
  42. Arch Dermatol. 2011 Oct;147(10):1203-5 - PubMed
  43. Br J Dermatol. 2015 Jul;173(1):275-8 - PubMed
  44. J Am Acad Dermatol. 2014 Nov;71(5):e225-6 - PubMed
  45. Acta Derm Venereol. 2015 May;95(5):525-31 - PubMed
  46. Clin Exp Dermatol. 2014 Oct;39(7):785-90 - PubMed
  47. Curr Clin Pharmacol. 2012 Nov;7(4):271-5 - PubMed
  48. Proc R Soc Med. 1956 Apr;49(4):234-5 - PubMed
  49. Hautarzt. 2012 Jul;63(7):577-83 - PubMed
  50. J Invest Dermatol. 2012 Sep;132(9):2166-70 - PubMed
  51. Arch Dermatol. 2012 May;148(5):655; author reply 656 - PubMed
  52. J Eur Acad Dermatol Venereol. 2011 Apr;25(4):485-8 - PubMed
  53. J Med Case Rep. 2014 Jun 25;8:226 - PubMed
  54. Autoimmun Rev. 2012 Nov;12(1):22-30 - PubMed
  55. J Dermatol Case Rep. 2013 Jun 30;7(2):64-8 - PubMed
  56. J Eur Acad Dermatol Venereol. 2016 Jan;30(1):188-9 - PubMed
  57. Orphanet J Rare Dis. 2007 Apr 15;2:19 - PubMed

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