Erysipelas-like erythema in children with familial Mediterranean fever

Clin Exp Rheumatol. Sep-Oct 2020;38 Suppl 127(5):101-104. Epub 2020 Oct 28.

Abstract

Objectives: Erysipelas-like erythema (ELE) is a well-known pathognomonic skin lesion associated with familial Mediterranean fever (FMF). The aim of this study was to describe the clinical and demographic features and phenotypic differences between paediatric FMF patients with and without ELE.

Methods: We retrospectively collected the medical charts of paediatric patients who had been diagnosed with FMF and followed by the Paediatric Rheumatology Department of Gazi University, Turkey, from 2006 to 2016.

Results: Among 782 FMF patients, 59 (33 males and 26 females; median age, 11.1±5.1) were found to have ELE. More patients had arthritis in the ELE group than in the other group (p=0.011). Arthritis occurred in the ankle (77.4%), knee (19.3%) and hip (3.2%) joints. The coexistence of arthritis and ELE was seen in 12 (20.3%) patients. All ELE plaques were located on the lower legs and dorsum of the feet. Eleven patients (18.6%) presented with ELE as the initial symptom and were diagnosed with FMF, and 48 (81.4%) patients experienced ELE attacks while receiving colchicine therapy. The median dose of colchicine at last visit, PRAS activity score and M694V homozygous mutation status were significantly higher in the ELE group than in the other group (p=0.041, p=0.001 and p=0.023, respectively).

Conclusions: ELE is an uncommon but important feature of FMF. In patients with ELE, arthritis is more frequently encountered, and M694V homozygous mutation is more frequently found. FMF patients with ELE have more severe disease activity, and they use higher doses of colchicine in relation to this severe disease course.

MeSH terms

  • Adolescent
  • Child
  • Erysipelas* / diagnosis
  • Erysipelas* / drug therapy
  • Erysipelas* / epidemiology
  • Erythema / diagnosis
  • Erythema / drug therapy
  • Erythema / epidemiology
  • Familial Mediterranean Fever* / complications
  • Familial Mediterranean Fever* / diagnosis
  • Familial Mediterranean Fever* / drug therapy
  • Female
  • Humans
  • Male
  • Mutation
  • Pyrin / genetics
  • Retrospective Studies
  • Turkey / epidemiology

Substances

  • Pyrin