II. Epidemiology
- Most common in age under 5 years
- Most common cause of Recurrent Fever in children
III. Signs: (PFAPA acronym)
-
Periodic Fever (PF)
- High fever lasting 3-6 days
- Recurs every 3-4 weeks
-
Aphthous Ulcer (A)
- Small lesions which are often asymptomatic
- Pharyngitis (P)
- Cervical Lymphadenopathy (A)
- Child is well between monthly exacerbations
IV. Differential diagnosis
- See Recurrent Fever
- See Aphthous Ulcer
- See Pharyngitis
- Cyclic Neutropenia
- Behcet's Disease
V. Management
- Prednisone 2 mg/kg may be considered at start of symptoms
- Agents that have been tried for prophylaxis
-
Tonsillectomy effective in small studies
- No recurrence after 10 months
- Berlucchi (2003) Ann Otol Rhinol Laryngol 112:365-9 [PubMed]
- Galanakis (2002) Arch Dis Child 86(6):434-5 [PubMed]