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PFAPA Syndrome
Aka: PFAPA Syndrome, Marshall's Syndrome, PFAPA- See Also
- Epidemiology
- Most common in age under 5 years
- Most common cause of Recurrent Fever in children
- 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
- Periodic Fever (PF)
- Differential diagnosis
- See Recurrent Fever
- See Aphthous Ulcer
- See Pharyngitis
- Cyclic Neutropenia
- Behcet's Disease
- 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]
- References