Infectious Disease Book

http://www.fpnotebook.com/

PFAPA Syndrome

Aka: PFAPA Syndrome, Marshall's Syndrome
  1. Epidemiology
    1. Most common in age under 5 years
    2. Most common cause of Recurrent Fever in children
  2. 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
  3. Differential diagnosis
    1. See Recurrent Fever
    2. See Aphthous Ulcer
    3. See Pharyngitis
    4. Cyclic Neutropenia
    5. Behcet's Disease
  4. 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
      3. Galanakis (2002) Arch Dis Child 86(6):434-5
  5. References
    1. John (2002) Pediatr Infect Dis J 21:1071-80

Navigation Tree