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PFAPA SyndromeAka: Marshall's Syndrome

Epidemiology
1. Most common in age under 5 years
2. Most common cause of Recurrent Fever in children
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
Differential diagnosis
1. See Recurrent Fever
2. See Aphthous Ulcer
3. See Pharyngitis
4. Cyclic Neutropenia
5. Behcet's Disease
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
  3. Galanakis (2002) Arch Dis Child 86(6):434
References
1. John (2002) Pediatr Infect Dis J 21:1071

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This page was written by Scott Moses, MD. This page was last revised on 4/26/2008 9:16:41 PM. This page was last published on 4/29/2008. Please send comments and questions to the editor. Contact information may be found on the page About the Family Practice Notebook. These pages are best viewed with Microsoft Internet Explorer 6.0.