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Late relapse of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (pfapa) syndrome: a case report

  • Authors Details :  
  • 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 title : Yemen Journal of Medicine

Publisher : Mansa STM Publishers

Online ISSN : 2583-4614

Page Number : 454-457

Journal volume : 04

Journal issue : 02

23 Views Case Report

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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DOI : https://doi.org/10.63475/yjm.v4i1.0202

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