Genital ulcers as an unusual sign of periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome: a novel symptom?

Periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy (PFAPA) syndrome, which is characterized by periodic episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis, is of unknown etiology and manifests usually before 5 years of age. A patient with periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome simultaneously presenting with genital ulcers has not been reported previously. We describe a 12-year-old Chinese girl with a 2-year history of periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome who exhibited vulvar ulcers accompanying an episode of febrile periodic fever, aphthous stomatitis, pharyngotonsillitis, and cervical adenopathy. Although during a 1-year follow-up this girl did not manifest typical symptoms/signs of Behçet's disease except recurrent oral aphthae and genital ulcers, it is possible that periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome and Behçet's disease could have overlapping manifestations. Furthermore, this report would add to the evidence of a wide variation in the clinical symptomatology of PFAPA syndrome.