PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND CERVICAL ADENITIS- A CASE REPORT

P. Correia, J.G. Marques, P. Valente

Infectious Disease Unit, Department of Paediatrics, Santa Maria Hospital, Lisbon, Portugal

A syndrome characterised by periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA Syndrome) was described in 1987. Episodes recur every 3 to 8 weeks and resolve spontaneously in 3 to 6 days. As there are no specific laboratory tests, it is necessary to exclude other syndromes of recurrent fever, especially Cyclic Neutropenia. Corticosteroids, in one or two small doses, can abort the episodes. The etiology of PFAPA is unknown but immune disregulation is a possible cause. In the long run, PFAPA is self-limiting and sequelae do not develop. The authors describe the case of a 7 year old female referred to their unit because of recurrent fever. Family and past medical history were irrelevant. At the age of 2, she began episodes of high fever wich recurred every 3 weeks and lasted for 4 to 6 days. Malaise, aphthous stomatitis, pharyngitis and cervical adenitis accompanied these episodes. Antibiotics were not helpful. Between episodes she was completely asymptomatic. Blood tests, including cultures, immunological and serological studies, were all negative. One year after the onset of these episodes, the patient had an eleven month symptom free period, followed by the reappearance of symptoms with the same periodicity. PFAPA seems to be more common than generally appreciated. Its diagnosis enables us to reassure the family and obviates the need for further unnecessary diagnostic tests. A prolonged symptom free period during the course of the syndrome, as documented in our patient, has not previously been described in the literature.