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Pyoderma Gangrenosum Tanı ve Tedavisi

Yıl 2025, Cilt: 8 Sayı: 2, 85 - 89, 28.08.2025

Öz

Pyoderma gangrenosum (PG) nadir bir nötrofilik dermatozdur. PG kutanöz lezyonları eritemli papül, püstül, nodül veya bül olarak başlayıp hızlı
genişleyen, ağrılı, eritemli altı yenik viyolose kenarlı ülserlere ilerler. Lezyonlar en sık alt ekstremitede yerleşir, paterji pozitifliği karakteristiktir.
Hastalığın seyri değişkendir, lezyonlar kendiliğinden gerileyebileceği gibi kronik, tekrarlayıcı seyir gösterebilir. İmmün patogenez komplekstir.
İnflamazom yapıları başta olmak üzere doğal (innate) immün sistemin aberan aktivasyonu mevcuttur. Adaptif i mmünitede ise Th1 ve
Th17 (STAT1 ve STAT4) yönünde polarizasyon görülür. Olguların ~%50’sinde eşlik eden immün-aracılı hastalık vardır. Bu hastalıklar en sık
inflamatuvar bağırsak hastalıkları olmak üzere, inflamatuvar artritler, hematolojik hastalıklar, monoklonal gamopatiler ve solid malignitelerdir.
PG’nin farklı klinik varyantları tanımlanmıştır, en sık görülen ülseratif (klasik form) tiptir. Büllöz, püstüler, vejetatif (süperfisiyel granülomatöz),
peristomal ve postoperatif formlar diğer klinik varyantlarıdır. PG nadiren ilaç ilişkili olarak da ortaya çıkabilir. PG bir ekartasyon tanısıdır, tanıda
diğer ülser nedenlerinin dışlanması gerekir. Tanıda yardımcı diagnostik kriterler tanımlanmıştır. İmmünosupresif tedavi, uygun yara bakımı,
eşlik eden komorbiditenin tedavisi, ağrı tedavisi ve psikososyal destek, relapsların önlenmesi, tedavisi tedavi komponentlerini oluşturmaktadır.
İmmünosupresif tedavilerden erken dönemde sistemik steroid ve/veya siklosporin gibi hızlı etkili bir ajanla başlanıp sonrasında etkisi daha
uzun sürede ortaya çıkan ancak yan etki profili daha güvenli immünosupresiflerden birine (biyolojik ajanlar, azatioprin, mikofenolat mofetil,
metotreksat vb.) geçilebilir.

Kaynakça

  • 1. Brocq L, Simon C. Contribution à l’étude du phagédénisme. Bull Soc Méd Hop Paris. 1908: 290-307.
  • 2. Brocq L. A new contribution to the study of geometric phagedenism, Ann Dermatol Syphiligr. 1916; 9: 39.
  • 3. Brunsting L, Goeckerman W. O′ leary PA: Pyoderma (Echthyma) Gangrenosum, Arch Dermatol Syph. 1930; 22: 655-680.
  • 4. Maverakis E, Marzano AV, Le ST, et al. Pyoderma gangrenosum, Nature Reviews Disease Primers. 2020; 6: 81.
  • 5. Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review, American journal of clinical dermatology. 2012; 13: 191- 211.
  • 6. Krüger S, Breuer C, Schwarz ER, et al. Multiple aseptic pulmonary nodules with central necrosis in association with pyoderma gangrenosum, Chest. 2001; 119: 977-978.
  • 7. Miserocchi E, Modorati G, Foster CS, et al. Ocular and extracutaneous involvement in pyoderma gangrenosum, Ophthalmology. 2002; 109: 1941-1943.
  • 8. Vignon-Pennamen MD. The extracutaneous involvement in the neutrophilic dermatoses, Clinics in dermatology. 2000; 18: 339-347.
  • 9. Xu A, Balgobind A, Strunk A, et al. Prevalence estimates for pyoderma gangrenosum in the United States: An age- and sex-adjusted population analysis, J Am Acad Dermatol. 2020; 83: 425-429.
  • 10. 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: 2166-2170.
  • 11. Ortega-Loayza AG, Davis MD. Neutrophilic Dermatoses. Dermatology. Fifth Edition 2024; 1-2: 450-469.
  • 12. Graham JA, Hansen KK, Rabinowitz LG, et al. Pyoderma gangrenosum in infants and children. Pediatric dermatology. 1994; 11: 10-17.
  • 13. Park AN, Raj A, Bajda J, et al. Narrative Review: Pyoderma Gangrenosum. Cureus. 2024; 16.
  • 14. Hobbs MM, Ortega‐Loayza AG. Pyoderma gangrenosum: from historical perspectives to emerging investigations. International Wound Journal. 2020; 17: 1255-1265.
  • 15. Farasat S, Aksentijevich I, Toro JR. Autoinflammatory diseases: clinical and genetic advances, Archives of dermatology. 2008; 144: 392-402.
  • 16. Landis ET, Taheri A, Jorizzo JL. Gulliver's sign: A recognizable transition from inflammatory to healing stages of pyoderma gangrenosum, J Dermatolog Treat. 2015; 26: 171-172.
  • 17. Alavi A, French LE, Davis MD, et al. Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment, Am J Clin Dermatol. 2017; 18: 355-372.
  • 18. Su WP, Davis MD, Weenig RH, et al. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria, Int J Dermatol. 2004; 43: 790-800.
  • 19. Jockenhöfer F, Wollina U, Salva KA, et al. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019; 180: 615-620.
  • 20. Maverakis E, Ma C, Shinkai K, et al. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts, JAMA Dermatol. 2018; 154: 461- 466.
  • 21. Ormerod AD, Thomas KS, Craig FE, 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.
  • 22. Brooklyn TN, Dunnill MG, Shetty A, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 2006; 55: 505- 509.

Diagnosis and Treatment of Pyoderma Gangrenosum

Yıl 2025, Cilt: 8 Sayı: 2, 85 - 89, 28.08.2025

Öz

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis. PG cutaneous lesions begin as erythematous papules, pustules, nodules or
bullae and progress to rapidly expanding, painful, erythematous ulcers with violaceous, undermined edges. Lesions are most commonly
located on the lower extremities, and pathergy positivity is a characteristic feature. The course of the disease is variable. Lesions may regress
spontaneously or may have a chronic, relapsing course. The immune pathogenesis is complex. There is aberrant activation of the innate
immune system, primarily in inflammatory structures. In adaptive immunity, polarization is seen towards Th1 and Th17 (STAT1 and STAT4).
~50% of cases have concomitant immune-mediated disease. These diseases are most commonly inflammatory bowel disease, inflammatory
arthritis, hematological diseases, monoclonal gammopathies and solid malignancies. Clinical variants of PG have been defined, t he most
common being the ulcerative (classic form). Bullous, pustular, vegetative (superficial granulomatous), peristomal and postoperative forms are
other clinical variants. PG can rarely occur in association with drugs. PG is an exclusion diagnosis, other causes of ulcers must be excluded for
diagnosis. Supportive diagnostic criteria have been defined for diagnosis. Immunosuppressive treatment, appropriate wound care, treatment
of accompanying comorbidities, pain management and psychosocial support, prevention and treatment of relapses constitute the treatment
components. Immunosuppressive treatment can be started with a rapid-acting agent such as systemic steroid and/or cyclosporine in the
early period and then switched to one of the immunosuppressive agents that have a longer-lasting effect but a safer adverse effect profile
(biological agents, azathioprine, mycophenolate mofetil, methotrexate, etc.).

Kaynakça

  • 1. Brocq L, Simon C. Contribution à l’étude du phagédénisme. Bull Soc Méd Hop Paris. 1908: 290-307.
  • 2. Brocq L. A new contribution to the study of geometric phagedenism, Ann Dermatol Syphiligr. 1916; 9: 39.
  • 3. Brunsting L, Goeckerman W. O′ leary PA: Pyoderma (Echthyma) Gangrenosum, Arch Dermatol Syph. 1930; 22: 655-680.
  • 4. Maverakis E, Marzano AV, Le ST, et al. Pyoderma gangrenosum, Nature Reviews Disease Primers. 2020; 6: 81.
  • 5. Ahronowitz I, Harp J, Shinkai K. Etiology and management of pyoderma gangrenosum: a comprehensive review, American journal of clinical dermatology. 2012; 13: 191- 211.
  • 6. Krüger S, Breuer C, Schwarz ER, et al. Multiple aseptic pulmonary nodules with central necrosis in association with pyoderma gangrenosum, Chest. 2001; 119: 977-978.
  • 7. Miserocchi E, Modorati G, Foster CS, et al. Ocular and extracutaneous involvement in pyoderma gangrenosum, Ophthalmology. 2002; 109: 1941-1943.
  • 8. Vignon-Pennamen MD. The extracutaneous involvement in the neutrophilic dermatoses, Clinics in dermatology. 2000; 18: 339-347.
  • 9. Xu A, Balgobind A, Strunk A, et al. Prevalence estimates for pyoderma gangrenosum in the United States: An age- and sex-adjusted population analysis, J Am Acad Dermatol. 2020; 83: 425-429.
  • 10. 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: 2166-2170.
  • 11. Ortega-Loayza AG, Davis MD. Neutrophilic Dermatoses. Dermatology. Fifth Edition 2024; 1-2: 450-469.
  • 12. Graham JA, Hansen KK, Rabinowitz LG, et al. Pyoderma gangrenosum in infants and children. Pediatric dermatology. 1994; 11: 10-17.
  • 13. Park AN, Raj A, Bajda J, et al. Narrative Review: Pyoderma Gangrenosum. Cureus. 2024; 16.
  • 14. Hobbs MM, Ortega‐Loayza AG. Pyoderma gangrenosum: from historical perspectives to emerging investigations. International Wound Journal. 2020; 17: 1255-1265.
  • 15. Farasat S, Aksentijevich I, Toro JR. Autoinflammatory diseases: clinical and genetic advances, Archives of dermatology. 2008; 144: 392-402.
  • 16. Landis ET, Taheri A, Jorizzo JL. Gulliver's sign: A recognizable transition from inflammatory to healing stages of pyoderma gangrenosum, J Dermatolog Treat. 2015; 26: 171-172.
  • 17. Alavi A, French LE, Davis MD, et al. Pyoderma Gangrenosum: An Update on Pathophysiology, Diagnosis and Treatment, Am J Clin Dermatol. 2017; 18: 355-372.
  • 18. Su WP, Davis MD, Weenig RH, et al. Pyoderma gangrenosum: clinicopathologic correlation and proposed diagnostic criteria, Int J Dermatol. 2004; 43: 790-800.
  • 19. Jockenhöfer F, Wollina U, Salva KA, et al. The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum. Br J Dermatol. 2019; 180: 615-620.
  • 20. Maverakis E, Ma C, Shinkai K, et al. Diagnostic Criteria of Ulcerative Pyoderma Gangrenosum: A Delphi Consensus of International Experts, JAMA Dermatol. 2018; 154: 461- 466.
  • 21. Ormerod AD, Thomas KS, Craig FE, 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.
  • 22. Brooklyn TN, Dunnill MG, Shetty A, et al. Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial. Gut. 2006; 55: 505- 509.
Toplam 22 adet kaynakça vardır.

Ayrıntılar

Birincil Dil Türkçe
Konular Klinik Tıp Bilimleri (Diğer)
Bölüm Derleme
Yazarlar

Ece Erbağcı 0000-0002-2887-0492

Yayımlanma Tarihi 28 Ağustos 2025
Gönderilme Tarihi 26 Haziran 2025
Kabul Tarihi 16 Temmuz 2025
Yayımlandığı Sayı Yıl 2025 Cilt: 8 Sayı: 2

Kaynak Göster

EndNote Erbağcı E (01 Ağustos 2025) Pyoderma Gangrenosum Tanı ve Tedavisi. Ege Tıp Bilimleri Dergisi 8 2 85–89.


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