Recurrent painful oral ulcers with periodic high fever

Specialty: Pediatrics.

• Recurrent aphthous stomatitis with fever
• suspected PFAPA or herpetic stomatitis

Why it occurs

• Primary herpetic gingivostomatitis (first infection by Herpes Simplex Virus type 1 or HSV-1, very common in childhood, characterized by prolonged high fever, painful diffuse ulcerations throughout the oral cavity, swollen and bleeding erythematous gums, and painful cervical lymphadenopathy)
• PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis, characterized by predictable febrile outbreaks accompanied by superficial oral thrush, non-exudative pharyngotonsillitis and spontaneously resolving bilateral cervical adenitis)
• Herpangina or Hand-Foot-Mouth Disease (caused by Coxsackie virus group A or other enteroviruses, presents with sudden high fever and vesicles that quickly ulcerate selectively located on the soft palate, tonsillar pillars and uvula, with or without association with peripheral skin lesions)
• Idiopathic recurrent aphthous stomatitis (recurrent painful ulcers without fever, with a benign course associated with local trauma or genetic predisposition)
• Celiac disease or Inflammatory Bowel Disease (Crohn's disease, which may initially manifest itself as recurrent painful oral thrush and growth retardation).

Initial workup

The differential diagnosis is clinical in most cases. In case of doubt or suspicion of systemic pathology: Smear of the ulcers for Tzanck smear, PCR for Herpes Simplex Virus type 1 or enterovirus. Complete blood count, CRP, ESR (which rise during PFAPA attacks but remain strictly normal between them). Screening for celiac disease (IgA anti-tissue transglutaminase antibodies). Lymphocyte subpopulation count and immunoglobulin levels if infections are unusually frequent or severe.

Standard management

• Oral acyclovir — indicated of choice in severe primary herpetic gingivostomatitis diagnosed in the first 72 hours of the onset of symptoms to shorten the clinical course; dose of 80 mg/kg/day divided into 4 oral doses for 5-7 days
• Paracetamol — first-line analgesic and antipyretic; dose of 15 mg/kg orally every 4-6 hours) or Ibuprofen (dose of 10 mg/kg orally every 6-8 hours)
• Topical anesthetic solution for mucosal use without systemic lidocaine (such as children's hyaluronic acid gels to cover canker sores, facilitating feeding; avoid eye drops with lidocaine or benzocaine due to the risk of toxic systemic absorption and methemoglobinemia in children under 2 years of age).