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A case of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome

Korean Journal of Pediatrics 2006;49(9):991-995.
Published online September 15, 2006.
A case of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome
Joo Hee Chae, A Rum Hwang, So Hyun Park, Byung Kyu Suh
Department of Pediatrics, College of Medicine, The Catholic University of Korea, Seoul, Korea
PFAPA 증후군 1례
채주희, 황아름, 박소현, 서병규
가톨릭대학교 의과대학 소아과학교실
Correspondence: 
Byung Kyu Suh, Email: suhbk@catholic.ac.kr
Abstract
PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral prednisolone is quite effective in controlling the symptoms. We report a case of a 6-year-old girl who was diagnosed as having PFAPA syndrome after 2 years of episodes, by excluding other disease entities with similar clinical features. The patient was treated with oral prednisolone and her symptoms improved dramatically.
Key Words: Periodic fever , Aphthous stomatitis , Pharyngitis , Cervical adenitis , Prednisolone


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