A Unique Pathognomic Skin Manifestation of Familial Mediterranean Fever: Erysipelas-Like Erythema

Yıl 2025, Cilt: 22 Sayı: 1, 154 - 158, 26.03.2025

Serkan Coşkun , Ayşe Tanatar

https://doi.org/10.35440/hutfd.1639567

Öz

Background: The present study aimed to describe the differences between patients with and with-out ELE in patients with Familial Mediterranean Fever (FMF) and to determine the relationship between erysipelas-like erythema (ELE) and demographic, clinical, genetic and treatment character-istics of the patients.
Materials and Methods: The medical files of patients who were followed up with a diagnosis of FMF in the Pediatric Rheumatology Department of Gaziantep City Hospital between October 2023 and October 2024 were retrospectively analysed.
Results: Of 409 patients with FMF, 205 (50.1%) were male. The median age at diagnosis was 6 (min-imum:1-maximum:18) years. FMF attack was accompanied by fever in 325 (79.5%), abdominal pain in 302 (73.8%), arthralgia in 121 (29.6%), arthritis in 56 (13.7%), chest pain in 62 (15.6%) and ELE in 55 (13.4%) patients.
When comparing patients with ELE to those without, the frequencies of chest pain, arthralgia, and arthritis were significantly higher in the ELE group (p=0.014, p<0.001, p<0.001, respectively).
Pathological mutations were significantly more common in FMF with ELE than those without one (p<0.001). Additionally, among FMF patients, the use of anti-interleukin-1 (anti-IL-1) treatment combinations due to colchicine resistance was significantly higher in those with ELE (p<0.001).
Conclusions: The present study showed that ELE may be associated with subclinical inflammation, arthritis, colchicine resistance, pathogenic mutations, and severe disease scores in FMF patients. Based on these findings and existing literature, we believe that the presence of ELE in FMF patients is significant in terms of diagnosis, prognosis, and predicting the course of the disease.

Anahtar Kelimeler

anti-interleukin-1 , colchicine resistance , erysipelas-like erythema , familial mediterranean fever

Ailevi Akdeniz Ateşinin Eşsiz Patognomik Cilt Bulgusu: Erizipel Benzeri Eritem

Öz

Amaç: Bu çalışmanın amacı erizipel benzeri eritemi olan ve olmayan Ailevi Akdeniz Ateşi (AAA) has-taları arasındaki farklılıkları tanımlamak ve erizipel benzeri eritem ile hastaların demografik, klinik, genetik ve tedavi özellikleri arasındaki ilişkiyi ortaya koymaktır.
Materyal ve Metod: Ekim 2023 ve Ekim 2024 tarihleri arasında Gaziantep Şehir Hastanesi Çocuk Romatoloji bölümünde AAA tanısıyla takip edilen hastaların dosyaları retrospektif olarak incelendi.
Bulgular: Ailevi Akdeniz Ateşi olan 409 hastanın 205'i (%50,1) erkekti. Hastaların tanı yaşı ortanca 6 (minimum:1-maksimum:18) yıldı. Hastaların 325'inde (%79,5) ateş, 302'sinde (%73,8) karın ağrısı, 121'inde (%29,6) artralji, 56'sında (%13,7) artrit, 62'sinde (%15,6) göğüs ağrısı ve 55'inde (%13,4) erizipel benzeri eritem FMF atağına eşlik ediyordu.
Erizipel benzeri eritemi olan hastalar olmayanlarla karşılaştırıldığında, göğüs ağrısı, artralji ve artrit sıklıkları erizipel benzeri eritemi olan grupta anlamlı olarak daha yüksekti (sırasıyla p=0.014, p<0.001, p<0.001).
Ailevi Akdeniz Ateşi hastalarında patolojik mutasyonlar ELE’si olanlarda ELE’si olmayanlardan daha yaygındı (p<0,001). Ayrıca AAA hastaları arasında kolşisin direnci nedeniyle anti-interlökin-1 tedavi kombinasyonlarının kullanımı erizipel benzeri eritemili hastalarda anlamlı derecede daha yüksekti (p<0.001).
Sonuç: Bu çalışma erizipel benzeri eritemin AAA hastalarında subklinik inflamasyon, artrit, kolşisin direnci, patojenik mutasyonlar ve ciddi hastalık skorları ile ilişkili olabileceğini göstermiştir. Bu bulgulara ve mevcut literatüre dayanarak AAA hastalarında erizipel benzeri eritem varlığının tanı, prognoz hem de hastalık seyrinin öngörülmesi açısından öneme sahip olduğana inanıyoruz.

Anahtar Kelimeler

ailevi akdeniz ateşi , anti-interlökin-1 , erizipel benzeri eritem , kolşisin direnci

Kaynakça

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