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Recurrent Fever of Unknown Origin (FUO) Due to Periodic Fever, Aphthous
Stomatititis, Pharyngitis and Adenitis (FAPA) Syndrome in an Adult
#MMPMID26237061
Muñoz-Gómez S
; Cunha BA
J Clin Med
2013[Aug]; 2
(3
): 45-8
PMID26237061
show ga
FAPA syndrome (periodic fever, aphthous stomatititis, pharyngitis and adenitis)
is a relatively new entity described in pediatric patients. In adults, reports of
FAPA are limited to rare case reports. The differential diagnosis of FAPA in
adults includes Behcet's syndrome, familial Mediterranean fever (FMF), Hyper IgD
syndrome and juvenile rheumatoid arthritis (JRA), i.e., adult Still's disease.
With FAPA syndrome, between episodes patients are completely asymptomatic and
serologic inflammatory markers such as erythrocyte sedimentation rate (ESR),
C-reactive protein (CRP) and white blood cell (WBC) count are normal. The
etiology of FAFA is unknown, but lack of secondary cases or clustering in close
contacts, lack of seasonality, and the lack of progression for years argue
against an infectious etiology. We describe an extremely rare case of an adult
with a recurrent FUO with profuse night sweats and prominent chills due to FAPA
syndrome.