Erythema Marginatum Rheumatica
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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' criteria for the diagnosis of rheumatic fever.[1] The Jones' criteria date back to 1944 but were modified in 1992,[2] and again in 2015.[3]
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.
Erythema marginatum rheumatica epidemiology
In most developed countries, rheumatic fever has become very rare. Erythema marginatum rheumatica occurs in fewer than 6% of cases of rheumatic fever.[4] However, rheumatic fever remains prevalent amongst Aboriginal and Torres Strait Islander communities in Australia,[5] and amongst Māori and Pacific populations in New Zealand.[6]
In the 1990s there appeared to be a resurgence of rheumatic fever in the USA.[7] A large series from Pittsburgh reported erythema marginatum as being uncommon in patients with rheumatic fever.[8]
Erythema marginatum rheumatica symptoms (Presentation)
One of the best descriptions of erythema marginatum was given by Professor Perry of Bristol. It was based on a case series published in Archives of Disease in Childhood.[9] This is detailed below, under 'Further reading & references'.
Characteristically the eruption starts as an erythematous macule. It gradually spreads out and, as it does so, the skin in the centre of lesions returns to normal, thus forming the typical spreading marginate or annular eruption. Where the spreading circles of the rash meet they coalesce forming a larger ring, or a serpiginous rash.
The usual sites of its occurrence are on the front of the abdomen and front and back of the chest. It can develop on the limbs but almost never on the face. It may, and usually does, appear at the onset of an acute attack or relapse of rheumatism but it is frequently present when there are no other signs of active infection and the ESR is normal. Once it has occurred it tends to come and go lasting from one or two days to months or years.
Subcutaneous nodules may also appear in rheumatic fever. They are closely associated with carditis.[10]
Differential diagnosis
Consider drug reactions. It may look like urticaria that can also change quite rapidly but in erythema marginatum there is no pruritus.
Erythema marginatum is also recognised as the classical skin finding in hereditary angio-oedema.[11]
Investigations
Diagnosis is based on presence of additional clinical features suggestive of acute rheumatic fever, using modified Jones' criteria.
Evidence of streptococcal infection is the essential criterion and this can be established by:
- Throat swab which grows Group A beta-haemolytic streptococcus.
- Raised or increasing streptococcal antibody titre.
In uncertain cases, skin biopsy may allow early diagnosis.[12]
Associated diseases
It may be associated with carditis, arthritis, fever and Sydenham's chorea.
Erythema marginatum rheumatica treatment and 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.
Prevention
As for rheumatic fever.
Further reading and references
Jones TD; Diagnosis of rheumatic fever. JAMA 1944 126: 481-85
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 21268(15):2069-73.
Gewitz MH, Baltimore RS, Tani LY, et al; Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation. 2015 May 19131(20):1806-18. doi: 10.1161/CIR.0000000000000205. Epub 2015 Apr 23.
Grassi A, Fesslova V, Carnelli V, et al; Clinical characteristics and cardiac outcome of acute rheumatic fever in Italy in the last 15 years. Clin Exp Rheumatol. 2009 Mar-Apr27(2):366-72.
Jackson SJ, Steer AC, Campbell H; Systematic Review: Estimation of global burden of non-suppurative sequelae of upper respiratory tract infection: rheumatic fever and post-streptococcal glomerulonephritis. Trop Med Int Health. 2011 Jan16(1):2-11. doi: 10.1111/j.1365-3156.2010.02670.x.
Milne RJ, Lennon DR, Stewart JM, et al; Incidence of acute rheumatic fever in New Zealand children and youth. J Paediatr Child Health. 2012 Aug48(8):685-91. doi: 10.1111/j.1440-1754.2012.02447.x. Epub 2012 Apr 12.
Congeni BL; The resurgence of acute rheumatic fever in the United States. Pediatr Ann. 1992 Dec21(12):816-20.
Zomorrodi A, Wald ER; Sydenham's chorea in western Pennsylvania. Pediatrics. 2006 Apr117(4):e675-9. Epub 2006 Mar 13.
Perry CB; Erythema marginatum (rheumaticum). Arch Dis Child. 1937 Aug12(70):233-8.
Singhi AK, Bobhate P, Kappanayil M; Acute rheumatic fever: subcutaneous nodules and carditis. Circulation. 2010 Feb 23121(7):946-7. doi: 10.1161/CIR.0b013e3181d30e96.
Bygum A; Hereditary angio-oedema in Denmark: a nationwide survey. Br J Dermatol. 2009 Nov161(5):1153-8. doi: 10.1111/j.1365-2133.2009.09366.x. Epub 2009 Jun 22.
Troyer C, Grossman ME, Silvers DN; Erythema marginatum in rheumatic fever: early diagnosis by skin biopsy. J Am Acad Dermatol. 1983 May8(5):724-8.