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Re: Differential diagnosis of Periodic Fevers

Dear Editor

We just read the short report of Galanakis et al.[1] We have been involving in periodic fevers management for many years. PFAPA is not the only inherited cause of periodic fever, known at present. It is an unclear nosological entity. Pharyngitis, cervical lymphoadenopathies and oral aphthae are not pathognomonic for PFAPA. Among periodic fevers, cervical lymphoadenopathies and episodic fever can occur in patients with HyperIg D and periodic syndrome (HIDS), oral aphthaeare a minor sign and isolated fever can be a frequent finding in children affected by Familial Mediterranean Fever (FMF); lastly, oral aphthae and recurrent febrile attacks characterise the onset of Behcet disease (BD) in children. The efficacy of steroids does not confirm the diagnosis of PFAPA; Behcet disease is responsive to steroids, too. The lack of familiar involvement is not a criteria to exclude an inherited disorder, as FMF and HIDS are recessive and BD is a multifactorial disease. Furthermore, the initial clinical picture of these disorders can be atypical and incomplete and can change during the clinical course.

So, considering the provenance of this series (Greece), we would wonder if some cases had BD and FMF, that will be recognised in the future. Nowadays, with increased diagnostic sensitivity and multiethnic societes, periodic fevers are being recognised outside their traditional area of incidence. Close follow-up is essential in further years, especially in patients with incomplete picture of PFAPA. A possible genetic screening for gene causing FMF, HIDS or immunological assay for HLA B51 could also be useful.

Reference

[1] Galanakis E, Papadakis CE, Giannoussi E, Karatzanis AD, Bitsori M, Helidonis ES. PFAPA syndrome in children evaluated for tonsillectomy. Arch Dis Child 2002;86:434-5.