Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults

  • Diogo Paixão Marques Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Sara Rocha Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Marta Manso Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal
  • Raquel Domingos Internal Medicine Department, Hospital de Egas Moniz, Lisbon, Portugal

Keywords

Periodic fever with pharyngitis,, aphthous stomatitis and cervical adenitis, PFAPA syndrome, adults

Abstract

Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made.

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  • Published: 2019-03-08

    Issue: LATEST ONLINE (view)

    Section: Articles

    How to cite:
    Marques, D., Rocha, S., Manso, M., & Domingos, R. (2019). Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults. European Journal of Case Reports in Internal Medicine, 2. https://doi.org/https://doi.org/10.12890/2019_001041

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