Abstract
Late Relapse of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome: A Case Report
Ahmad Ibrahim Zaal¹,²,³, Amad Radwan Jamal Eddin⁴, Maryam Mohanad Al Ansari⁴, Manar Husam Hussein⁴, Ikram Hamoud⁵, Rabih R. Andari¹, David Aman Eddin¹, Manaf Shaker Alkassab¹, Maher Mohamad Najm⁶,⁷
Keywords: Periodic fever, aphthous stomatitis, pharyngitis, adenitis, PFAPA
DOI: 10.63475/yjm.v4i1.0202
DOI URL: https://doi.org/10.63475/yjm.v4i1.0202
Publish Date: 22-09-2025
Download PDFPages: 443 - 446
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Author Affiliation:
1 Consultant, Department of Pediatrics,Dr Sulaiman Al-Habib Hospital, Dubai, United Arab Emirates
2 Associate Professor, Department of Pediatrics, Faculty of Medicine, Damascus University, Damascus, Syria
3 Assistant Professor, Department of Pediatrics, College of Medicine, University of Sharjah, Sharjah, United Arab Emirates
4 Medical Student, College of Medicine, University of Sharjah, Sharjah, United Arab Emirates
5 Specialist, Department of Pediatrics, Dr Sulaiman Al-Habib Hospital, Dubai, United Arab Emirates
6 Consultant, Department of Pediatrics, Hamad Medical Corporation, Doha, Qatar
7 Assistant Professor, College of Medicine, Qatar University, Doha, Qatar
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.