Una PFAPA che diventa un Behçet

Abstract

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pathogenesis is unknown. The paper reports the description of a clinical case of a 12-year-old girl diagnosed with PFAPA syndrome from the age of one and half years. The exacerbations did not decrease with growth as it usually happens, but genital ulcers appeared associated with HLA-B51 positivity, orienting the diagnosis towards Behçet’s spectrum disorders.