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We just read the short report of Galanakis et al.1 We have been involving in periodic fevers management for many years. At present, PFAPA is an unclear cause of periodic fever, classified among non-hereditary fevers. It is an unclear nosological entity. Pharyngitis, cervical lymphoadenopathies and oral aphthae are exclusive findings in PFAPA. Among periodic fevers, cervical lymphoadenopathies and episodic fever can occur in patients with HyperIg D and periodic syndrome (HIDS), and less in Familial Mediterranean Fever (FMF). Oral aphthae (as minor sign), cervical adenopathies, and isolated fever can be in children affected by FMF. Pharyngitis, oral aphtae, cervical adenopathies, and recurrent fever also characterise Crohn’s disease (CD). Lastly, oral aphthae and recurrent febrile attacks characterise the onset of Behçet’s disease (BD) in children. The efficacy of steroids does not confirm the diagnosis of PFAPA; BD and CD are 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 and CD are multifactorial diseases. 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 Galanakis’ series (Greece), we not be surprised if some cases had BD or 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, in these patients. A possible genetic screening for gene causing FMF, HIDS, or immunological assay for HLA B51 could also be useful.
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