Avar-Aydın Pınar Özge, Ozcakar Zeynep Birsin, Aydın Fatma, Karakaş Hatice Dilara, Çakar Nilgün, Yalçınkaya Fatoş
Department of Pediatric Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey.
Turk Arch Pediatr. 2022 Nov;57(6):599-602. doi: 10.5152/TurkArchPediatr.2022.22058.
Erysipelas-like erythema is the pathognomonic skin manifestation of familial Mediterranean fever although not frequently seen in the pediatric population. This study aims to describe the differences between patients presenting with and without erysipelas-like erythema and to examine the relation of erysipelas-like erythema with subclinical inflammation in a large pediatric cohort of familial Mediterranean fever patients.
This retrospective study from a single pediatric rheumatology referral center included familial Mediterranean fever patients with a follow-up for at least 6 months in the last 5 years. Patients were grouped according to the presence of erysipelas-like erythema and subclinical inflammation.
Among 515 patients with familial Mediterranean fever, 35 patients (6.8%) were found to present with erysipelas-like erythema, and the earliest age for erysipelas-like erythema was 2.9 years. All erysipelas-like erythema lesions were defined on lower extremities with concurrent arthritis in 21 patients (60.0%). Compared to other patients in the cohort, patients presented with erysipelas-like erythema had significantly higher frequencies of acute arthritis, subclinical inflammation, and biallelic exon 10 mutations, and they used significantly higher doses of colchicine at the latest visits (all P ≤ .002). Patients with subclinical inflammation more frequently presented with erysipelas-like erythema compared to others without subclinical inflammation (21.7% vs. 2.9%, P < .001).
Erysipelas-like erythema is an uncommon but important finding that can be a sign of severe disease course and subclinical inflammation in the pediatric population with familial Mediterranean fever.
丹毒样红斑是家族性地中海热的特征性皮肤表现，尽管在儿科人群中并不常见。本研究旨在描述有和没有丹毒样红斑的患者之间的差异，并在一大群家族性地中海热儿科患者中研究丹毒样红斑与亚临床炎症的关系。
这项来自单一儿科风湿病转诊中心的回顾性研究纳入了过去5年中随访至少6个月的家族性地中海热患者。根据是否存在丹毒样红斑和亚临床炎症对患者进行分组。
在515例家族性地中海热患者中，发现35例（6.8%）出现丹毒样红斑，丹毒样红斑的最早发病年龄为2.9岁。所有丹毒样红斑皮损均位于下肢，21例（60.0%）同时伴有关节炎。与队列中的其他患者相比，出现丹毒样红斑的患者急性关节炎、亚临床炎症和双等位基因外显子10突变的发生率显著更高，并且在最近一次就诊时他们使用秋水仙碱的剂量显著更高（所有P≤0.002）。与没有亚临床炎症的其他患者相比，有亚临床炎症的患者更频繁地出现丹毒样红斑（21.7%对2.9%，P<0.001）。
丹毒样红斑是一种罕见但重要的发现，它可能是家族性地中海热儿科患者严重病程和亚临床炎症的一个迹象。
