Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome

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

• 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