Pyoderma Gangrenosum Tanı ve Tedavisi

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.

Anahtar Kelimeler

• Pyoderma gangrenosum
• ülser
• immünosupresif

Diagnosis and Treatment of Pyoderma Gangrenosum

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.).

Anahtar Kelimeler

• Pyoderma gangrenosum
• ulcer
• immunosuppressive

Kaynakça

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