Periodic fever, aphthous stomatitis, pharyngitis, and lymphadenopathy: a pediatric caseload
Ciambra, R.; Milocco, C.; Facchini, S.; Leone, V.; Locatelli, C.; Pocecco, M.
La Pediatria Medica e Chirurgica Medical and Surgical Pediatrics 25(3): 181-184
2003
ISSN/ISBN: 0391-5387 PMID: 14601235 Document Number: 558498
We report 5 patients (3F; 2M; age 19-60 months) affected by a syndrome characterized by recurrent episodes of abrupt onset of fever, aphtous stomatitis, pharyngitis and cervical adenopathy named by the acronym PFAPA (periodic fever, aphtous stomatitis, pharyngitis and adenopathy). The episodes had clockwork periodicity (3-4 weeks) and the fever was unaccompanied by remarkable respiratory signs or symptoms. All children were doing well between episodes. The inflammatory markers were elevated only during attacks in all children. Steroid therapy was highly effective in controlling symptoms in 5/5 patients and tonsillectomy was associated with remission in two of five patients. In one child the syndrome resolved spontaneously, in two patients resolved after tonsillectomy and in two children persisted. No long-term sequelae were observed.