Original article
Syndrome of periodic fever, pharyngitis, and aphthous stomatitis

https://doi.org/10.1016/S0022-3476(87)80285-8Get rights and content

A syndrome of periodic fever that resembles human cyclic neutropenia in its clinical presentation has been identified in 12 children observed at two major referral centers. Attacks characterized by abrupt onset of fever, malaise, chills, aphthous stomatitis, pharyngitis, headache, and tender cervical adenopathy occur at 4- to 6-week intervals over periods of years. These episodes of illness resolve spontaneously in 4 to 5 days. Mild leukocytosis and elevation of the erythrocyte sedimentation rate during attacks are the only laboratory abnormalities. Affected children grow normally, are not unusually susceptible to infection, and exhibit no long-term sequelae. Attacks may be aborted by short courses of prednisone but do not respond to nonsteroidal anti-inflammatory agents. This syndrome is sporadic and appears to be much more common than cyclic neutropenia.

References (33)

  • McArdleB

    Familial periodic paralysis

    Br Med Bull

    (1956)
  • WrightDG et al.

    Human cyclic neutropenia: clinical review and long-term follow-up of patients

    Medicine

    (1981)
  • ChajekT et al.

    Behçet's disease: report of 41 cases and a review of the literature

    Medicine

    (1975)
  • WrightVA et al.

    Behçet's syndrome

    Bull Rheum Dis

    (1978)
  • ReimannHA

    Periodic fever, an entity: a collection of 52 cases

    Am J Med Sci

    (1962)
  • BondyPK et al.

    The possible relationship of etiocholanolone to periodic fever

    Yale J Biol Med

    (1958)
  • Cited by (0)

    View full text