Medical ConditionsImmune & periodontal: PFAPA syndrome
Medical ConditionsImmune & periodontal: PFAPA syndrome
PFAPA syndrome
Overview
Board takeaway: Recognize the recurrent pattern, exclude competing causes, and defer elective dental care during an active febrile episode.
What is PFAPA syndrome?
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis is an autoinflammatory syndrome usually beginning in early childhood. Episodes recur with a recognizable pattern; children generally grow normally and are well between attacks. There is no single confirmatory test.
Which findings support PFAPA and which alternatives must be considered?
Several-day fever episodes with one or more of oral aphthae, pharyngitis, and cervical lymphadenopathy support the pattern. Recurrent infection, cyclic neutropenia, other autoinflammatory syndromes, and systemic disease must be considered. Persistent symptoms between episodes, impaired growth, unusual infections, or cytopenias require further assessment.
What oral findings occur during PFAPA episodes?
Small aphthous ulcers may involve the inner lips, cheeks, tongue, or pharynx. Inflamed tonsils and pharyngitis may accompany them. Pain can reduce fluid intake and hygiene. The oral findings alone do not distinguish PFAPA from other causes of fever and ulcers.
How should dental care be handled during a PFAPA episode?
Defer elective procedures while the child is febrile or substantially unwell. Assess hydration and oral pain, provide gentle hygiene and appropriately selected local symptom relief, and coordinate with the medical team. Do not label an unexplained fever PFAPA without medical evaluation. Urgent dental infection still needs timely, coordinated treatment.
How is PFAPA treated and what is the prognosis?
The treating clinician may use a single corticosteroid dose to abort an episode; this can shorten the interval to the next episode. Colchicine or cimetidine may be considered for prevention, and tonsillectomy is an option for selected patients. Many children outgrow the syndrome, although recurrence can persist. Repeated episodic steroids do not automatically establish a dental stress-dose requirement.