Bornholm Disease

oPatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

Synonyms: epidemic myalgia, epidemic pleurodynia, Sylvest's disease, Bamble disease and devil's grip

Bornholm disease is a viral illness with myalgia, causing pain in the lower chest and upper abdomen. It is usually a self-limiting illness; rarely, there are complications due to the virus. The types of virus involved in Bornholm disease can cause severe illness in neonates.

The disease is usually caused by a Coxsackie group B virus; it is rarely caused by Coxsackie A or echoviruses.[1] All these belong to the group of enteroviruses.

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  • Bornholm disease mainly affects children and young adults.
  • There may be epidemics, usually in spring and autumn in temperate climates.
  • Coxsackie B viral infections are common and can cause a range of conditions from subclinical infection to myocarditis.

Transmission:

  • This occurs via the faeco-oral route, including shared drinks containers.
  • It is highly contagious.
  • The incubation period is a few days.
  • Pain in the lower chest/upper abdomen:[2]
    • Can be sudden in onset; may have spasmodic pain.
    • Typically, it is a pleuritic-type pain exacerbated by deep breathing or movement; it can be severe enough to cause dyspnoea.
    • Duration is normally a few days, but may be ≤3 weeks; it can recur/relapse.
  • Other symptoms:
    • Fever, headache or nonspecific abdominal pain - either as prodromal symptoms or with the onset of chest pain.
    • Myalgia elsewhere.
    • Symptoms and signs of complications (see 'Prognosis and Complications', below).
  • Examination - fever; localised tenderness at the site of the pain.

Usually the diagnosis is clinical, but investigations may be needed:

  • To exclude other important diagnoses or to assess complications - for example: ECG, CXR, investigations for pulmonary embolus, or other tests according to the clinical picture. CXR is normal in uncomplicated Bornholm disease.[2]
  • Viral studies may be useful if there are complications or vulnerable contacts, eg in neonates or late pregnancy.[3]

Other causes of chest pain or pleuritic pain - for example:[2]

  • Pulmonary embolus, pleural effusion.
  • Pneumonia.
  • Myocardial infarction or pericarditis.
  • Pneumothorax.
  • Tietze's syndrome.
  • Muscle strain or chest trauma.
  • Shingles.

Other causes of upper abdominal pain or subcostal pain - for example:

  • Peptic ulcer.
  • Cholecystitis, gallstones or hepatitis.
  • Subphrenic abscess.
  • Painful splenomegaly, eg with glandular fever or splenic injury.
  • Exclude other important diagnoses.
  • Supportive treatment - analgesia.
  • Consider the risk to neonates (see 'Pregnancy', 'Neonatal enteroviral infection' and 'Prevention' sections, below).

In general, the literature suggests that many or most cases are uncomplicated.

Reported complications, mainly from case reports, are:

  • Pericarditis and myocarditis; possibly, myocarditis is more common in young children and pericarditis in adults.
  • Transient paroxysmal tachycardia (one case report, probably due to myocarditis).
  • Orchitis.
  • Viral meningitis.

There are other recognised complications of Coxsackie and echoviral infections (the literature is less clear how these relate to Bornholm disease specifically):[2]

  • Neonatal systemic illness (see 'Neonatal enteroviral infection', below).
  • Respiratory infection.
  • Skin or oropharyngeal manifestations.
  • Transient paralytic illness.

Possible associations have been suggested between Coxsackie B virus and chronic fatigue syndrome and type 1 diabetes. Little evidence currently exists to support this.[4]

  • Enteroviral infections in pregnancy are common.
  • Most cases in pregnant women are probably not associated with significant maternal or fetal disease. However, there is a risk of severe illness in neonates (see below).
  • Enteroviral infections in pregnancy are not known to cause any fetal abnormalities.
  • Coxsackie virus B infections may increase the risk of spontaneous early abortions and (rarely) fetal myocarditis.[5]

Transmission:

  • Vertical transmission of Bornholm disease from mother to neonate has been documented.The virus is probably transmitted by vaginal or faeco-oral routes.[6] Other plausible routes of transmission are transplacental spread, contact with maternal body fluids or respiratory transmission.[7][8]
  • Enterovirus may be present in breast milk while the mother has an enteroviral infection, but it is not known whether breast milk is a significant mode of transmission.[7]

Clinical features and management:

  • Neonatal enteroviral infection varies in severity, from asymptomatic to severe or fatal systemic illness.[7] The infant may have hepatitis, myocarditis, and meningoencephalitis.[8]
  • The timing of delivery in relation to maternal illness is probably important, as it determines whether the neonate has received any maternal antibodies to the virus.[6]
  • Immunoglobulin is advised for prophylaxis of exposed neonates (see 'Prevention', below).
  • Treatment is supportive. In case reports, other treatments used include intravenous gammaglobulin combined with human leukocyte interferon, or the anti-picornaviral drug, pleconaril.[9] (Pleconaril is not currently licensed in the UK.)
  • Neonatal exposure:
    • For neonates exposed to enteroviral infections, the Health Protection Agency advises immunoglobulin prophylaxis, quoting its use in an outbreak of echoviral infection.
  • General precautions - handwashing and good hygiene; avoid sharing utensils used for food and drink.

The disease was described by doctors Homan and Daae in Norway in 1872, and was called Bamble disease since their first case lived in Bamble. The name Bornholm disease was given by a Danish doctor, Sylvest, who observed the illness on the island of Bornholm in Denmark in the 1930s. UK epidemics occurred in 1956 and 1963.

Further reading & references

  1. Kaushik P et al, Coxsackieviruses, Medscape, Sep 2011
  2. Kumar P, Clarke M; Clinical Medicine, 6th Ed, (2005). WB Saunders: London
  3. Guidance on Viral Rash in Pregnancy; Health Protection Agency (January 2011)
  4. Green J, Casabonne D, Newton R; Coxsackie B virus serology and Type 1 diabetes mellitus: a systematic review of Diabet Med. 2004 Jun;21(6):507-14.
  5. Ornoy A, Tenenbaum A; Pregnancy outcome following infections by coxsackie, echo, measles, mumps, hepatitis, polio and encephalitis viruses. Reprod Toxicol. 2006 May;21(4):446-57. Epub 2006 Feb 9.
  6. Tang JW, Bendig JW, Ossuetta I; Vertical transmission of human echovirus 11 at the time of Bornholm disease in Pediatr Infect Dis J. 2005 Jan;24(1):88-9.
  7. Maus MV, Posencheg MA, Geddes K, et al; Detection of echovirus 18 in human breast milk. J Clin Microbiol. 2008 Mar;46(3):1137-40. Epub 2008 Jan 16.
  8. Cheng LL, Ng PC, Chan PK, et al; Probable intrafamilial transmission of coxsackievirus b3 with vertical Pediatrics. 2006 Sep;118(3):e929-33. Epub 2006 Aug 14.
  9. Bryant PA, Tingay D, Dargaville PA, et al; Neonatal coxsackie B virus infection-a treatable disease? Eur J Pediatr. 2004 Apr;163(4-5):223-8. Epub 2004 Feb 18.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Gurvinder Rull
Current Version:
Peer Reviewer:
Dr Adrian Bonsall
Last Checked:
19/01/2012
Document ID:
635 (v23)
© EMIS