PFAPA syndrome in adult patient

A 22-year-old male patient came to our clinic with episodic recurrent fever (39.4O) associated with an aphthous stomatitis, bilateral cervical lymphadenitis, throat pain and headache. These events were recurrent in the last two years (every 6th-8th week), and he was treated with antibiotics and nonsteroidal anti-inflammatory drugs. Oral cavity inspection showed erythematous pharyngitis, hypertrophic palatine tonsils and aphthous ulcers disseminated in his left palatine tonsil, lingual tonsil, and buccal mucosa (Figure 1). The physical examination was normal except for bilateral submandibular lymphadenitis. Laboratory findings showed no abnormalities including negative for bacterial cultures, antinuclear antibodies, and serological markers of virus (EBV, CMV, HSV-1, HSV-2), except for a moderately elevated level of C-reactive protein [1,2]. The etiology of PFAPA syndrome is unidentified and the diagnosis is based on clinical criteria [3]. The patient was treated with the intermittent use of a single oral prednisone administration, observing a successful response.