PFAPA syndrome (Paediatrics)

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PFAPA syndrome (periodic fever, aphthae, pharyngitis and cervical adenopathy) or Marshall syndrome is one of the causes of recurrent or periodic fevers in paediatrics.

It is a chronic condition of unknown aetiology. Although it is unlikely to have a genetic cause, it is usually grouped with the hereditary periodic fever syndromes. The syndrome usually begins between the ages of 2 and 5 and is more common in boys.

The child has an episode of fever about once a month, lasting between 3 and 6 days. The syndrome causes fatigue, chills and sometimes abdominal pain and headache, as well as fever, sore throat, thrush and swollen lymph nodes. The child is healthy between successive episodes and growth is normal.

Diagnosis is based on a medical history and physical examination. Blood tests are done to measure the levels of substances that indicate inflammation (markers).

There is no need for treatment, but corticosteroids may help relieve the child's symptoms.

Bibliographic references
  1. Shai Padeh, MD. Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA syndrome). Uptodate. Mar 29, 2017.
  2. Kastner DL, Aksentijevich I, Goldbach-Mansky R. Autoinflammatory disease reloaded: a clinical perspective. Cell 2010; 140:784.
  3. Padeh S, Stoffman N, Berkun Y. Periodic fever accompanied by aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA syndrome) in adults. Isr Med Assoc J 2008; 10:358.
  4. Sampaio IC, Rodrigo MJ, Monteiro Marques JG. Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome. Pediatr Infect Dis J 2009; 28:254.
  5. Padeh S. Periodic fever syndromes. Pediatr Clin North Am 2005; 52:577.
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Symptoms

    Localised abdominal pain


    Sore throat


    Skin rash


    Watery diarrhoea


    Mouth ulcer or sore

Symptoms to watch out for

Fever that doesn't subside with antipyretics (paracetamol, ibuprofen)

Self-care

Relative rest, rest until symptoms subside.
Consume over-the-counter pain relievers or anti-inflammatories.
Check with your general practitioner about the prescription for oral corticosteroids.