title: PFAPA syndrome mimicking familial Mediterranean fever: report of a Turkish child.
abstract: The PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitidis) syndrome is characterized by periodic fever, adenitis, pharyngitis, and aphthous stomatitis. Herein, we present a Turkish child with PFAPA syndrome mimicking familial Mediterranean fever because of a rare presentation. A 9-year-old boy was admitted with recurrent fever, aphthous stomatitis, sore throat, headache, and general body pains, lasting 2 to 3 days since 3.5 years of age. He was completely symptom-free between the attacks. He was diagnosed as having familial Mediterranean fever according to the clinical findings when he was 6 years of age and Colchicum tablet was administrated. Despite colchicines therapy for 8 months, his attacks did not subside; therefore, the drug was discontinued. He had high fever, a painful cervical lymphadenopathy, aphthous stomatitis, and tonsillo-pharyngitis. The patient was then diagnosed as having PFAPA syndrome. He was given a single dose of prednisolone (0.35 mg/kg/dose). His complaints dramatically and completely disappeared 3 h after administration of the drug. During the 8th month of follow-up, a similar febrile attack lasting only 1 day was noted and it was controlled with a single dose of prednisolone (0.5 mg/kg/day). At this writing the patient is in the 12th month of follow-up, and there have been no symptoms after the second attack. In conclusion, our patient shows that PFAPA syndrome can be confused with familial Mediterranean fever. We also would like to emphasize that the typical PFAPA syndrome can be easily diagnosed by detailed history-taking and physical findings.
authors.affiliation: Department of Pediatrics, Yüzüncü Yil University Faculty of Medicine, Van, Turkey.
authors.forename: Bülent
authors.initials: B
authors.lastname: Ataş
authors.forename: Hüseyin
authors.initials: H
authors.lastname: Caksen
authors.forename: Sükrü
authors.initials: S
authors.lastname: Arslan
authors.forename: Oğuz
authors.initials: O
authors.lastname: Tuncer
authors.forename: Ercan
authors.initials: E
authors.lastname: Kirimi
authors.forename: Dursun
authors.initials: D
authors.lastname: Odabaş
mesh_terms: D002648:Child; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis*; D005334:Fever / Q000175:diagnosis*; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D008199:Lymphadenitis / Q000175:diagnosis; D008297:Male; D010507:Periodicity; D010612:Pharyngitis / Q000175:diagnosis; D011239:Prednisolone / Q000008:administration & dosage; D013281:Stomatitis, Aphthous / Q000175:diagnosis; D013577:Syndrome; D014421:Turkey
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone