Erythema anulare rheumaticum L53.2; I00+L54.0;

Bright 1831; Chealde 1889; Leiner and Lehndorff 1922;

Erythema marginatum was first described by Bright in 1831 and is known under various names. Although it is a rare manifestation (<6% of cases), erythema marginatum is still considered a specific lesion of poststreptococcal rheumatic fever (RF) and is a major criterion in the new 2015 revision of the Jones criteria. RF and erythema marginatum usually occur in children and less frequently in adults.

Characteristic but not pathognomonic immunological reaction to toxins of beta-hemolytic group A streptococci. The starting point is streptococcal angina or pharyngitis.

Erythema anulare rheumaticum is one of the 5 main criteria for rheumaticfever defined by the American Heart Association and occurs in 10% of patients (Gewitz MH et al. 2015).

The disease can occur worldwide, but is becoming increasingly rare in industrialized countries. Given the decline in the prevalence of RF, it is important to consider this diagnosis when fever and joint pain occur after angina, especially in patients from developing countries. The infection is not immunizing and the exanthema may recur in the event of re-infection.

The cause of rheumatic fever is angina tonsillaris or streptococcal pharyngitis caused by beta-hemolytic streptococci of the Lancefield group A. The rheumatic fever is a consequence of an infection-induced autoimmune reaction to the intrinsically local streptococcal infection.

Infection-associated anular erythema, which morphologically does not differ from erythema anulare rheumaticum, also occurs associatively in serum sickness, psittacosis, trypanosomiasis and other diseases.

Mainly upper abdominal area (especially periumbilical) and back. Also buttocks and back of the hands (face always excluded) can be affected.

Manifestation on the joints (polyarthritis), heart (endo-myo-pericarditis), on the skin in the form of red-brownish, non-itching, anular or polycyclic erythema and so-called rheumatoid nodules as well as on the CNS as chorea minor. With particular emphasis on the edges, the manifestations are also referred to as erythema marginatum rheumaticum.

The exanthema is usually accompanied by episodes of fever and is more pronounced in the late afternoon. The characteristic skin changes frequently occur at the beginning of acute rheumatic fever, often as a concomitant symptom of joint and heart involvement (almost always coinciding with endocarditis).

In the context of acute rheumatic fever, non-figured exanthema may also occur, small red urticarial patches, papules or disc-shaped plaques on the knees and elbows, which regress within days or a few weeks without forming anular formations(erythema papulatum: Cockayne 1912).

Elevated ESR, CRP, leukocytes, ASL titers,

Superficial, perivascular and interstitial dermatitis; mainly perivascularly oriented, neutrophilic and round cell infiltrates (Troyer C et al. 1983).

Direct immunofluorescence (DIF) is negative.

Urticaria; tinea corporis; contact dermatitis; erythema anulare centrifugum; erythema gyratum repens.

Paediatric treatment of rheumatic fever.

If the peripheral areas of the anular erythema are palpable, this form is also called erythema marginatum rheumaticum (erythema annulare marginatum).

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4. Gewitz MH et al. (2015) 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. Circulation131: 1806-1818.
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7. Troyer C et al. (1983) Erythema Marginatum in Rheumatic Fever: Early Diagnosis by Skin Biopsy. J Am Acad Dermatol 8: 724-728.