PFAPA syndrome

Introduction: PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. It usually happens to children younger than 5 years of age. Case report: Herein, we present a case of 14-year-old Taiwanese male who had been suffering from periodic oral aphthous ulcerations and high fever almost every month since he was 1 year old. At the time of examination, yellowish crusts on upper and lower vermillions noted with pain, along with several ruptured vesicles over soft palate and pharyngeal area were seen. Multiple oral aphthous ulcers were noted throughout oral mucosa, including tongue border, buccal mucosa and labial mucosa. Under thorough history taking and medical records, we excluded other diseases, including cyclic neutropenia. A diagnosis of PFAPA syndrome with impetigo on upper and lower vermillion and herpangina was rendered. The patient was treated with supporting treatment on the first week until herpangina resolved, followed by oral prednisolone (5mg, 3 times per day for 5 days) and topical triamcinolone rinse (4mg in 20ml water, topical rinse, 4 times daily for 7 days) and the patient responded well. Thereafter we recommended ranitidine therapy (150mg, once per day for 3 months) as prophylaxis for the recurrent aphthous stomatitis. The patient was doing well without recurrence of aphthous ulcers. The patient is currently under close follow up and ranitidine therapy would be indicated if the symptoms recurred. Conclusion: Through this case, we want to prove that typical PFAPA syndrome could be easily diagnosed through careful examination of patient's history and clinical features.