Erythema marginatum rheumatica is a rash that is associated with acute rheumatic fever. Rheumatic fever is a multisystem disease that occurs after infection with a Lancefield group A streptococcus.

The rash represents one of the major Jones' criteria¹ for the diagnosis of rheumatic fever. The Jones' criteria date back to 1944 but were modified in 1992.²

The term erythema annulare is sometimes used for erythema marginatum but erythema annulare centrifugum is classified as one of the figurate or gyrate erythemas. It may be due to a hypersensitivity, to malignancy, infection, drugs, or chemicals, or it may be idiopathic. Erythema marginatum is really, by definition, associated with rheumatic fever.

Epidemiology

In developed countries, rheumatic fever has become very rare and this rash occurs in no more than 2-5% of cases of rheumatic fever. However, rheumatic fever has become as common in New Zealand as in some developing countries, especially amongst the Maori children. In the 1990s there appeared to be a resurgence of rheumatic fever in the USA.³ Erythema marginatum was uncommon in patients with rheumatic fever reported in a large series from Pittsburgh.⁴

Most doctors in general practice in Great Britain will never see a case of rheumatic fever and the likelihood of seeing a case of erythema marginatum is even less.⁵

Presentation

There are light pink macules spreading outwards with a serpiginous, well-demarcated edge and clearing central portion. The rash changes from hour to hour and may seem to appear, disappear or move so rapidly that it can almost be seen doing so. It often involves multiple areas, usually on the trunk and occasionally over the proximal parts of the limbs. It is exacerbated by heat and fades when the patient is cool.

There are usually other symptoms of acute rheumatic fever but it can recur intermittently over weeks or even months.

It may appear with subcutaneous nodules which are firm, painless lumps, mainly on the hands, feet, occiput and back.⁶ They are usually 0.5 to 2 cm in diameter and often found in crops of about 3, appearing 2 to 3 weeks after the onset of fever. The rash occurs early in the disease and remains long past the resolution of other symptoms.

Differential diagnosis

Consider drug reactions. It may look like urticaria that can also change quite rapidly but in erythema marginatum there is no pruritus.

Investigations

Confirmation of diagnosis of acute rheumatic fever is by:

• Throat swab which grows Lancefield group A beta haemolytic streptococcus.
• Rapid antigen test.
• Raised or increasing streptococcal antibody titre.
• In uncertain cases, skin biopsy may allow early diagnosis.⁷

Associated diseases

It may be associated with carditis, arthritis, fever and Sydenham's chorea.

Management

There is no specific management of the rash but rheumatic fever must be treated as described in the Rheumatic Fever article. If the diagnosis is suspected it is wise to start a full course of penicillin as for rheumatic fever.

Complications

There are no specific complications of the rash but complications such as cardiac disease and Sydenham's chorea may occur as a result of the rheumatic fever.

Prognosis

As for Rheumatic Fever (see separate article).

Prevention

As for rheumatic fever.

Document references

1. Jones TD: Diagnosis of rheumatic fever. JAMA 1944; 126: 481-85
2. No authors listed; Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association. JAMA. 1992 Oct 21;268(15):2069-73. [abstract]
3. Congeni BL; The resurgence of acute rheumatic fever in the United States. Pediatr Ann. 1992 Dec;21(12):816-20. [abstract]
4. Zomorrodi A, Wald ER; Sydenham's chorea in western Pennsylvania. Pediatrics. 2006 Apr;117(4):e675-9. Epub 2006 Mar 13. [abstract]
5. Kenealy T, Sore throats, Clinical Evidence May 2005, published May 2006; (subscription required)
6. Erythema marginatum, Health Pictures.; picture of erythema marginatum from health-pictures.com
7. Troyer C, Grossman ME, Silvers DN; Erythema marginatum in rheumatic fever: early diagnosis by skin biopsy. J Am Acad Dermatol. 1983 May;8(5):724-8. [abstract]

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