The Periodic Fever, Adenitis, Pharyngitis, Aphtous Stomatitis Syndrome: A Case Report

Introduction: The PFAPA (Periodic Fever, Adenitis, Pharyngitis, Aphtous Stomatitis) syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis, and adenopathy. It is a benign sporadic condition, the cause and physiopathology of which is still unknown. It affects children younger than 5 years, with an average age of 2.8 years, and slight predominance in men. The diagnostic criteria are clinical, with no specific complementary examinations existing. A response to corticoid corroborates the diagnosis, suggesting mediation of cytokines as mechanism and not an infectious process.

Objective: This study aims to report a case of the PFAPA syndrome.

Case Report: A 2-year-and-11-month-old patient presented at least one episode of tonsillitis a month with similar characteristics: lush exudate, high fever, lasting between 4 and 6 days, poor general condition, refusal to eat and sore throat, enlargement of the anterior cervical lymph nodes, in addition to aphthous lesions in the oral mucosa and lateral border of the tongue. Rapid tests for streptococci gave negative results on five occasions. After various treatments with antibiotics without any effective improvement, PFAPA was suspected and prednisone was started, with rapid interruption of the symptoms after 12 hours. The patient’s condition evolved well with a reduction in the intervals of crisis and a calmer atmosphere in the family.

Conclusion: The PFAPA syndrome is an entity of undefined etiology, but it is well-characterized clinically. Recognition of the pathology and diagnosis is important in diminishing the distress of the family and in improving the child’s quality of life.

Keywords: PFAPA, periodic fever, aphthous stomatitis.