Late Relapse of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome: A Case Report

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ID: 311572
2025
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Abstract
PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) is the most common periodic fever disorder in childhood, typically resolving by early adolescence. Relapse after a prolonged symptom-free interval is rare and has been reported following tonsillectomyrelated remission. We describe a 13-year-old girl with intact tonsils who experienced recurrence of PFAPA a decade after initial remission. She presented with a 6-day history of high-grade fever, aphthous ulcers, pharyngitis, and cervical adenitis unresponsive to antibiotics. Investigations showed elevated inflammatory markers, a negative infectious workup, and significant cervical lymphadenopathy on ultrasound. A single dose of prednisolone led to the rapid resolution of symptoms followed by sustained remission on follow-up. This case highlights the diagnostic challenges of late PFAPA relapse and underscores the importance of clinical recognition, exclusion of mimicking conditions, and corticosteroid responsiveness in guiding management.
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Authors Ahmad Ibrahim Zaal, Amad Radwan Jamal Eddin, Maryam Mohanad Al Ansari, Manar Husam Hussein, Ikram Hamoud, Rabih R. Andari, David Aman Eddin, Manaf Shaker Alkassab, Maher Mohamad Najm
Journal Yemen Journal of Medicine
Year 2025
DOI
10.63475/yjm.v4i1.0202
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