Summary:
Marshall Syndrome PFAPA (Periodic Fever, Aphthous
Stomatitis, Pharyngitis, Adenitis) Syndrome is a chronic
condition, typically starting 2-5 years old, in which fever
occurs periodically (lasts for 3-7 days), accompanied by
aphthous-like ulcers, pharyngitis and/or cervical adenitis.
The patients have no clinical symptoms between episodes
and it is required to exclude all other diseases before
confirming the diagnosis. The dramatic response to
treatment with steroid helps diagnosing PFAPA. We are
presenting the case of an 8 years male patient, with the
history of recurrent episodes of fever (onset at the age of
two), oral ulcer and difficulty in deglutition who
constantly received antibiotic therapy and or antifungal
prescribed by different doctors. Clinically the patient was
febrile, mildly pale, cervical lymphadenopathy, aphthous
ulcers in the tongue with inflamed tonsils, covered with
thick exudates and pharyngeal wall was inflamed and
folliculated. There was just palpable liver. Laboratory
investigations performed but it was without serological
confirmation. Throat swab culture was negative. The child
received steroid (Prednisolone) with favorable outcome.
Subsequently, the patient presented with similar episodes
of fever which disappeared within 24 hours of single dose
of prednisolone. After Ranitidine (H2 blocker)
prophylaxis the patient remains asymptomatic for about
one and half years. With our best knowledge this is the
first case report of Marshall Syndrome or PFAPA Syndrome
in Bangladesh.
Key Words: Marshall syndrome, PFAPA syndrome.
(J Bangladesh Coll Phys Surg 2016; 34: 222-224)