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

Authors : Serkan Coşkun, Ayşe Tanatar

Pages : 154-158

Doi:10.35440/hutfd.1639567

View : 30 | Download : 39

Publication Date : 2025-03-26

Article Type : Research Paper

Abstract :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.
Keywords : ailevi akdeniz ateşi, anti-interlökin-1, erizipel benzeri eritem, kolşisin direnci