[Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) in adults]

The PFAPA syndrome is characterized by a regular appearance of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis and is associated with a distinctive poor general condition of the patients. Since its first description in 1987 the syndrome has usually been described in children. But we here report its diagnosis in three adult patients.

At the Department of Otorhinolaryngology/Head and Neck Surgery of the University Medical Center Freiburg, data on 36 patients with PFAPA syndrome were collected between 2004 and 2010.  Data analysis was based on structured questionnaires, medical files of patients and a systematic assessment of the relevant international literature up to April 2011.

The average age of the initial occurrence of PFAPA syndrome in the three patients was in the first few months after birth (month 2, 6 and 7). A tonsillectomy had been performed on all three patients in early childhood. As a result two patients had remission of symptoms for several years (4.5 and 12 years) with recurrence of PFAPA attacks while the third patient had no changes in symptoms.

The PFAPA syndrome is an illness that first manifests itself in children. However, it can persist until adulthood or reappear after phases of remission. A knowledge of this periodic fever syndrome and extensive and close cooperation of general practitioners, paediatricians, internists and ENT specialists will be helpful in early diagnosis of this disorder.