Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome

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Revision as of 16:12, 12 July 2023 by Aidan (talk | contribs) (Created page with "== Background == * A cause of periodic fever syndrome that occurs mainly in young children * Rare in adults, though case reports exist == 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 == * Re...")
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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