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DOI: 10.1055/s-0038-1629315
Das PFAPA-Syndrom
Rezidivierende Fieberschübe, V. a. chronische Angina tonsillaris und nuchale Lymphknotenschwellungen bei einem 4-jährigen JungenThe PFAPA syndrome – periodic fever, suspection of chronic angina tonsillaris and cervical adenopathy in a four year old boyPublication History
Eingereicht am:19 September 2011
angenommen am:28 January 2012
Publication Date:
31 January 2018 (online)
Zusammenfassung
Das PFAPA-Syndrom ist eines der häufigeren rezidivierenden Fiebersyndrome, wird jedoch meist erst spät diagnostiziert oder gar nicht erkannt. Die betroffenen Patienten leiden neben einer zervikalen Adenopathie, einer aphthösen Stomatitis und einer ausgeprägten Pharyngitis an periodischen Fieberepisoden, die alle 26 Wochen auftreten. In diesen Phasen liegt ein ausgeprägtes Krankheitsgefühl vor, die Patienten sind schwer beeinträchtigt. Die Ätiologie der Erkrankung ist bislang unbekannt. Anhand des Krankheitsverlaufes eines 4-jährigen Jungen werden das klinische Bild, die Diagnosestellung und die Therapiemöglichkeiten dieser Erkrankung sowie die notwendige Differenzialdiagnostik aufgezeigt.
Summary
The PFAPA syndrome is one of the more frequent fever syndroms but is often diagnosed late in patients history or not at all. Next to suffering of cervical adenopathy, aphthous stomatitis and a severe pharyngitis the patients suffer from periodic fever every 2–6 weeks. During these phases the patients are severe affected with an distinctive malaise. Based on the example of a 4 year old boy, the clinical picture, the act of diagnosis, the proper therapy of this disease and the differential diagnoses are illustrated.
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