Erythema marginatum, also known as erythema rheumaticum Lehndorf-Leiner, is a characteristic annular erythema that is one of the manifestations of acute rheumatic fever and, less commonly, psittacosis or C1 inhibitor deficiency. ICD-10 code is L53.2.
This disease primarily affects individuals between the ages of 5 and 15 years, with no gender differences, and is more common in developing countries. It occurs in 1-18% of cases (typically about 10%) of individuals with acute rheumatic fever, an autoimmune disease triggered by group A beta-hemolytic streptococci.
However, cases of erythema marginatum in psittacosis and angioedema, both hereditary and acquired due to C1 inhibitor deficiency, have been reported. The exact etiopathogenesis of erythema marginatum is not fully understood.Erythematous patches usually appear 1-5 weeks after streptococcal pharyngitis infection and in the presence of fever (above 38.0°C). These patches spread rapidly (up to 15-20 mm in 12 hours) in a centrifugal pattern, forming circular, figurative, polycyclic, and snake-like lesions with healthy skin in the center and a thin (1-2 mm) flat red border at the periphery that blanches on diascopy. There is no peeling.
Pruritus and pain are not observed. The rash first appears on the trunk, then extends to the flexor surfaces of the limbs, and rarely to the dorsum of the hands, feet, and face. Often the lesions migrate - appearing and disappearing briefly in some areas and reappearing in others. In some cases, the rash is only visible in daylight.
The eruptions usually resolve within a few hours to 2-3 days, but may recur after several weeks (periodic erythema marginatum). With erythema marginatum, one or more signs of acute rheumatic fever are almost always present:
- Carditis (30-50%)
- Migratory polyarthritis (35-66%) involving knees, elbows, wrists, and ankles, with swelling, redness, and severe joint pain.
- Sydenham's chorea or Saint Vitus' dance (10-30%) - characterized by sudden, purposeless movements, emotional disturbances, and inappropriate behavior.
- Rheumatic nodules (0-10%) - painless subcutaneous nodules located above joints or tendons.
- Fever - 38.0°C.
- Arthralgia - joint pain without visible joint damage.
Diagnosis is based on the patient's medical history, clinical presentation, and laboratory data. Histological features are nonspecific and include a perivascular polymorphic infiltrate of neutrophils and mononuclear cells in dermis.
Evidence of recent streptococcal infection.
- Elevation or increased titers of anti-streptolysin-O or anti-deoxyribonuclease B.
- Positive results from throat swab cultures.
- Positive rapid streptococcal antigen test.
Duckett Jones Criteria for Rheumatism Diagnosis (2015):
Major criteria:
- Carditis.
- Polyarthritis.
- Chorea.
- Erythema Marginatum.
- Rheumatic nodules.
Minor criteria:
- Fever ≥ 38.0°C.
- Arthralgia.
- History of previous rheumatic fever or rheumatic heart disease.
- Leukocytosis, elevated erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) (ESR ≥ 30 mm/h and/or CRP ≥ 3.0 mg/dL).
- Prolonged P-R interval on electrocardiogram.
For initial acute rheumatic fever: 2 major criteria or 1 major and 2 minor criteria.
For recurrent rheumatic fever: 2 major criteria or 1 major and 2 minor criteria or 3 minor criteria.
- Urticaria
- Adult-Onset Still Disease
- Palpable migratory arciform erythema
- Erythema Annulare Centrifugum
- Erythema Multiforme
- Mononucleosis
- Annular erythema of infancy
- Eosinophilic annular erythema
- Erythema gyratum repens
- Erythema migrans