II. Epidemiology

  1. Most common in age under 5 years
  2. Most common cause of Recurrent Fever in children

III. Signs: (PFAPA acronym)

  1. Periodic Fever (PF)
    1. High fever lasting 3-6 days
    2. Recurrs every 3-4 weeks
  2. Aphthous Ulcer (A)
    1. Small lesions which are often asymptomatic
  3. Pharyngitis (P)
  4. Cervical Lymphadenopathy (A)
  5. Child is well between monthly exacerbations

IV. Differential diagnosis

V. Management

  1. Prednisone 2 mg/kg at start of symptoms
  2. Cimetidine has been used for prophylaxis
  3. Tonsillectomy effective in small studies
    1. No recurrence after 10 months
    2. Berlucchi (2003) Ann Otol Rhinol Laryngol 112:365-9 [PubMed]
    3. Galanakis (2002) Arch Dis Child 86(6):434-5 [PubMed]

Images: Related links to external sites (from Bing)

Related Studies (from Trip Database) Open in New Window

Ontology: PFAPA syndrome (C2938935)

Concepts Disease or Syndrome (T047)
English Syndrome of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis, PFAPA syndrome, pfapa syndrome, pfapa syndrome (diagnosis)
Dutch PFAPA-syndroom, syndroom van periodieke koorts, afteuze stomatitis, faryngitis, cervicale adenitis
Spanish Síndrome PFAPA, Síndrome de fiebre periódica, estomatitis aftosa, faringitis, adenitis cervical
Portuguese Síndrome de PFAPA, Síndrome de febre periódica, estomatite aftosa, faringite, adenite cervical
Italian Sindrome PFAPA, Sindrome della febbre periodica, stomatite aftosa, faringite, adenite cervicale
German Syndrom periodischen Fiebers, apthoeser Stomatitis, Pharyngitis, Zervikaladenitis, PFAPA-Syndrom
French Syndrome de PFAPA, Syndrome de fièvre périodique, stomatite aphteuse, pharyngite, adénopathie cervicale
Japanese PFAPA症候群, 周期熱・アフタ性口内炎・咽頭炎・頚部リンパ節炎症候群, PFAPAショウコウグン, シュウキネツアフタセイコウナイエンイントウエンケイブリンパセツエンショウコウグン
Czech PFAPA syndrom, Syndrom periodické horečky, aftózní stomatitidy, faryngitidy a krční adenitidy
Hungarian Periódikus láz, aphthás stomatitis, pharyngitis, cervicalis adenitis tünetegyüttes, PFAPA syndroma