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

Bright 1831; Chealde 1889; Leiner and Lehndorff 1922;

Specific but non-pathognomic immunological reaction to toxins of group A beta-hemolytic streptococci. The starting point is streptococcal angina or pharyngitis.

Erythema anulare rheumaticum is one of the 5 main criteria of rheumatic feverdefined by the "American Heart Association", occurring in 10% of patients.

The disease can occur worldwide, but is becoming increasingly rare in industrialized countries. It mainly affects children.

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 at the joints (polyarthritis), heart (endo-myo pericarditis), at the skin in the form of red-brownish, non-pruritic, anular or polycyclic erythema and so-called rheumatoid nodules as well as at the CNS as chorea minor.

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

In the course of acute rheumatic fever, non-figured exanthema may also occur, small red urticarial patches, papules or 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; nonspecific pattern; round cell and neutrophil infiltrates.

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).

1. Barlow T (1883) Erythema marginatum. Br Med J 509
2. Chockalingam A et al (2004) Rheumatic heart disease occurrence, patterns and clinical correlates in children aged less than five years. J Heart Valve Dis 13: 11-14
3. Lehndorff H, Leiner C (1922) Erythema annulare. Z Kinderheilkd (Berlin) 32: 46
4. Rullan E et al (2001) Rheumatic fever. Curr Rheumatol Rep 3: 445-452