PFAPA SYNDROME - PERIODIC FEVER, ADENITIS , PHARYNGITIS AND APHTHOUS STOMATITIS - CASE-REPORTS AND LITERATURE-REVIEW

A syndrome involving periodic fever, pharyngitis, adenitis and aphthou s stomatitis is described in 8 children. Attacks are characterized by abrupt onset of fever and, in addition to the above symptoms, by malai se, headache and abdominal pain. Mild leukocytosis and elevation of th e erythrocyte sedimentation rate are found in the laboratory. Patients exhibit normal growth and development and are otherwise healthy. PFAP A is clinically benign, with no long-term sequelae. Recognition and di agnosis of the syndrome eliminate the need for intensive work-up. The cause remains unknown. No evidence linking bacterial, viral, or fungal pathogens to this syndrome has been found. No patient has exhibited a typical lymphocytosis or neutropenia, and all patients had normal leve ls of immunoglobulin. All had received antibiotics early in the course of their illness but without effect. Cimetidine has been discussed in the literature as a possible treatment, but the results are controver sial.