Pyoderma Gangrenosum Tanılı Hastaların Klinik Özelliklerinin, Komorbiditelerinin ve Tedavi Yanıtlarının Retrospektif Olarak İncelenmesi

Yıl 2024, , 481 - 486, 30.09.2024

Tubanur Çetinarslan , Abdullah Kutay Masat , Mustafa Turhan Şahin , Aylin Türel Ermertcan

https://doi.org/10.34087/cbusbed.1515660

Öz

Giriş ve Amaç: Pyoderma gangrenozum [PG], hızlı ilerleyen, düzensiz eritematöz kenarlı ağrılı deri ülserleri ile seyreden nadir bir nötrofilik dermatozdur. PG'nin nedeni tam olarak anlaşılamamıştır, ancak genellikle otoinflamatuar bir bozukluk olarak kabul edilir. Klinik bulguların yanı sıra histopatolojik inceleme tanı koymada yardımcı olabilir.
Gereç ve Yöntemler: 2012-2024 yılları arasında kliniğimize başvuran ve klinik ve histopatolojik olarak PG tanısı alan hastalar çalışmamıza dahil edildi. Çalışmamızda hastaların yaş, cinsiyet, lezyon yerleşim yeri, tetikleyici faktör, ek hastalıkları, aldıkları tedavi ve tedaviye yanıtları incelendi.
Bulgular: Çalışmaya toplam 25 PG tanılı hasta dahil edildi. 25 hastanın 17’si kadın, 8’i erkek idi. Olguların tamamında histopatolojik inceleme ile PG tanısı desteklendi. 17 hastada tetikleyici faktör bulunamazken, en çok suçlanan tetikleyici faktör cerrahi işlemler idi ve bunu sırasıyla travma ve egzoz yanığı takip etmekteydi. Hastalara PG tedavisinde en sık sistemik metilprednizolon verilirken [18 hasta], bunu sırasıyla topikal steroid [9 hasta], intravenöz immunglobulin [İVİG] [8 hasta], siklosporin [5 hasta], intralezyonel triamsinolon asetonid [3 hasta], topikal takrolimus [2 hasta], mikofenolat mofetil [1 hasta] ve topikal kalsipotriol [1 hasta] takip etmekteydi. Hastaların 13’ünde sistemik steroidler ile diğer tedaviler kombine kullanılırken, 11’i monoterapi ile tedavi edildi. Çalışmamızdaki olguların 3’ü sadece sistemik steroidlere, 5’i hem sistemik steroidlere hem siklosporine dirençliydi ve bu 8 hastada İVİG tedavisi ile yanıt alındı.
Sonuç: PG tedavisinde sistemik steroidler ve siklosporinin kanıt düzeyi 1b olup birinci basamak tedavide yer almaktadırlar. PG’da birinci basamak tedavi seçenekleri olan sistemik steroidler ve siklosporine dirençli olgularda, İVİG kanıt düzeyi 3a olmasına rağmen, iyi bir seçenek olarak görünmektedir.

Anahtar kelimeler: pyoderma gangrenosum, intravenöz immunglobulin, İVİG, nötrofilik dermatoz, ülser, inflamatuar bağırsak hastalığı

Anahtar Kelimeler

pyoderma gangrenosum, intravenöz immunglobulin, İVİG, nötrofilik dermatoz, ülser, inflamatuar bağırsak hastalığı

Retrospective evaluation of clinical characteristics, comorbidities and treatment responses of patients with pyoderma gangrenosum

Öz

Aim; Pyoderma gangrenosum [PG] is a rare neutrophilic dermatosis characterized by rapidly progressive, painful skin ulcers with irregular erythematous margins. The etiology of PG is not fully understood, but it is generally considered as an autoinflammatory disorder. In addition to clinical findings, histopathological examination may help in the diagnosis.
Method; Patients diagnosed clinically and histopathologically with PG were included. The patients' age, gender, lesion location, triggering factor, comorbidities, treatment methods and response rate to treatment were recorded.
Results; A total of 25 patients diagnosed with PG were included [17 female, 8 male]. The diagnosis of PG was supported by histopathological examination in all cases. There was no history of triggering factor in 17 patients. On the other hand, the most common triggering factor was surgical procedures, followed by trauma and burn. Systemic methylprednisolone was most frequently used therapeutic agent [18 patients], and followed by topical steroid [9 patients], intravenous immunoglobulin [IVIG] [8 patients], cyclosporine [5 patients], intralesional triamcinolone acetonide [3 patients], topical tacrolimus [2 patients], mycophenolate mofetil [1 patient] and topical calcipotriol [1 patient]. Systemic steroids and other treatment modalities were used in combination in 13 patients, while 11 were treated with monotherapy. In our study, 3 cases were resistant to systemic steroids, and 5 were resistant to both systemic steroids and cyclosporine, and these 8 patients responded to IVIG treatment.
Conclusion; In the treatment of PG, systemic steroids and cyclosporine have a level of evidence of 1b and are included in the first-line treatment. The level of evidence for IVIG in PG, is 3a. In cases resistant to systemic steroids and cyclosporine, which are first-line treatment options in pyoderma gangrenosum, IVIG treatment is an effective therapeutic agent. In cases resistant to systemic steroids and cyclosporine, which are first-line treatment options in PG, IVIG seems to be a good option, although the evidence level is 3a.

Keywords: pyoderma gangrenosum, intravenous immunoglobulin, IVIG, neutrophilic dermatosis, ulcer, inflammatory bowel disease

Anahtar Kelimeler

pyoderma gangrenosum, intravenous immunoglobulin, IVIG, neutrophilic dermatosis, ulcer, inflammatory bowel disease

Kaynakça

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