Bornholm disease

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Bornholm disease, also known as epidemic pleurodynia, is a virally-mediated myositis presenting as recurrent episodes of acute severe pleuritic pain. It is usually self-limiting, and serious morbidity is rare.

Epidemiology

Its true incidence is unknown and it is thought that it is underdiagnosed, at least partially due to the ignorance about the condition itself .

The diagnosis tends to peak in late summer and the early autumn . It is seen in both adults and children, especially in the very young and very old. It is more common in those that are immunosuppressed.

Clinical presentation

Bornholm disease is notable for its acute short episodes of marked pleuritic pain, although occasionally it can present as back or abdominal pain. Characteristically the pain is extremely severe, hence its old name, devil's grip disease. The episodes are generally short-lived, often only seconds, but can last as long as half an hour.

In the interval between episodes, the severe pain is replaced by a persistent pleuritic discomfort. As with pleuritic pain in general, the severity is exacerbated by coughing, laughing, breathing, and sneezing.

In addition to chest pain, other symptoms and signs include pyrexia (97%), sore throat (85%), headaches (50%), nausea and vomiting, diarrhea, otitis, dermatitis and scrotal pain.

Diagnosis

Bornholm disease is a clinical diagnosis, usually after more serious conditions, e.g. myocardial ischemia, pulmonary embolism, etc. have been excluded.

A full blood count may show a leukocytosis or leukopenia, however the white cell count can be normal. The C-reactive protein, erythrocyte sedimentation rate and/or creatine kinase may also be elevated.

Serology to the Coxsackievirus may be positive. The most specific test is obtaining a viral isolate from body fluids or soft tissue, e.g. pleural effusion, feces or the pharynx.

Pathology

Bornholm disease is an infectious myositis most frequently caused by the Coxsackieviruses, especially serotypes B1-B6. Echovirus (e.g. type 1) may also be the causative organism.

Radiographic features

Generally imaging shows no abnormalities. Occasionally non-specific signs include pleural effusion and/or pleural thickening, basal consolidation, and/or ground glass opacities.

Treatment and prognosis

Analgesia is the mainstay. The pain usually responds to non-steroidal anti-inflammatory drugs (NSAIDs) e.g. diclofenac. Rarely chest wall nerve blocks with local anesthesia are required to control the more severe pain.

The disease is usually self-limiting and generally the symptoms last 5-7 days, although longer courses have been reported.

Rarely more severe sequelae e.g. myocarditis and pericarditis have been reported. These are more common in the extremes of age, and also the immunocompromised. No fatalities have been reported.

History and etymology

The first description of the disease was in 1872 by Daae in Norway. This was a cluster of cases in a village called Bamble, hence its old name Bamble disease. A later epidemic on the island of Bornholm in Denmark gave rise to its usual moniker .

Differential diagnosis

Since the presentation of Bornholm disease mimics more severe pathologies, some life-threatening, it is often only considered after other diagnoses have been excluded.