Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome

From IDWiki

Background

Clinical Manifestations

  • Fever lasting 3 to 5 days, up to 40.5 degrees Celsius
  • Erythematous or exudative pharyngitis (90% of patients)
  • Cervical lymphadenopathy (75%)
  • Oral aphthosis (50%)
  • Possibly also headache, abdominal pain, nausea, vomiting, chills, malaise, myalgia, and arthralgia

Diagnostic Criteria

  • Regularly recurring fevers with an early age of onset (<5 years of age)
  • Constitutional symptoms in the absence of upper respiratory infection with at least 1 of the following clinical signs:
    • Aphthous stomatitis
    • Pharyngitis
    • Cervical lymphadenitis
  • Exclusion of cyclic neutropenia
  • Completely asymptomatic interval between episodes
  • Normal growth and development