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

Authors

• 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

diogomarques_89@hotmail.com

Keywords

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.