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Pyoderma gangrenosum in a patient with familial Mediterranean fever and chronic inflammatory seronegative arthropathy: a unique triad
  1. Maria Constantinou1 and
  2. Konstantinos Parperis2,3
  1. 1Medical School, University of Cyprus Medical School, Nicosia, Cyprus
  2. 2Department of Medicine, University of Arizona College of Medicine Phoenix, Phoenix, Arizona, USA
  3. 3Department of Medicine, University of Cyprus Medical School, Nicosia, Cyprus
  1. Correspondence to Dr Konstantinos Parperis; parperis.konstantinos-marinos{at}


Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterised by recurrent fever attacks and serositis. Chronic inflammatory seronegative arthropathy affects the spine and peripheral joints and rarely coexists with FMF. Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that manifests as an ulcerative skin disease that uncommonly occurs in patients with FMF. In this case report, we describe a male patient in his 60s with a history of FMF and chronic inflammatory seronegative arthropathy who developed ulcerative skin lesions consistent with PG. A genetic evaluation revealed a pathogenic variant (V726A) and two variants of uncertain significance (F479L and E167D) mutations in the MEFV gene. We hypothesised that the triad of FMF, chronic inflammatory seronegative arthropathy and PG might be linked to the V726A variant, while the presence of the other two variants may have amplified the clinical presentation. Further studies are warranted to confirm our observation.

  • Genetics
  • Dermatology
  • Rheumatology

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  • Contributors All the authors, KP, MC had substantial contributions to the conception or design of the work, or the acquisition, analysis, or interpretation of data for the work. Additionally, drafting the work or revising it critically for important intellectual content, final approval of the version to be published and all agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.