Periodic Fever, Aphthous stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) is also known as Marshall Syndrome. This is a somewhat more common periodic fever syndrome, but many physicians are often unaware of this condition.
PFAPA is a chronic condition- often-noted first in children under five that is characterized by periodic episodes of high fever occurring every four weeks. The fever quickly rises to 103-106 degrees and stays high for three to six days. The child is otherwise healthy in between episodes of fever, with no runny nose or respiratory symptoms of illness during febrile attacks. PFAPA syndrome may be accompanied by mouth sores, a red and inflamed throat, and swollen lymph glands. Fevers resolve on their own spontaneously, and the periodic fevers may persist for several years but seem to have no significant health effects. This is a more common periodic fever syndrome, but physicians are often unaware of the syndrome. http://www.pfapa.net
Diagnosis is often made after patients have had more than thee episodes of flares with fevers, lasting up to five days and recurring often at regular intervals without any signs of acute illness during the febrile episodes. Pharyngitis (inflamed throat) plus adenopathy or aphthous ulcers that are not associated with an illness are noted. Elevated C-reactive protein (CRP), Erythrocyte Sedimentation Rate (ESR), are often elevated during the febrile attacks. Many children have less frequent episodes by adolescence, and some have seemed to outgrow this condition.
Read our saidsupport.org blog to learn more about PFAPA, and how this disease compares to HIDS.
Our growing list of research about PFAPA is on our saidsupport.org blog.