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ICD Hub / Diseases of the skin (L00–L99) / EA50.0 - Erythema marginatum rheumaticum
ICD-11

ICD-11 Codes
for EA50.0 - Erythema marginatum rheumaticum
Teodor Jurukovski January 1, 1970
Fact checked by: Teodor Jurukovski
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Synonyms and Alternative Terms for Erythema Marginatum Rheumaticum

Erythema marginatum rheumaticum is also known by several other clinical terms, including Erythema marginatum, Erythema annulare rheumaticum, and Erythema marginatum (rheumaticum). It is a specific type of skin reaction.

Clinical Overview of Erythema Marginatum Rheumaticum

The ICD-11 code for Erythema marginatum rheumaticum is EA50.0. This condition is a cutaneous exanthem, a type of rash, that can occur in up to 10% of individuals experiencing rheumatic fever. It is classified as a toxin-mediated cutaneous reaction stemming from distant or systemic bacterial infections. Erythema marginatum rheumaticum is recognised as one of the major diagnostic criteria for acute rheumatic fever, often appearing as a transient skin reaction associated with inflammation.

Causes and Risk Factors for Erythema Marginatum Rheumaticum

Erythema marginatum rheumaticum is primarily linked to acute or chronic rheumatic fever, which is itself a consequence of infection with group A streptococcus bacteria. While less common, it can also be associated with rare genetic conditions like hereditary angioedema or the infectious disease psittacosis. Risk factors for developing rheumatic fever, and consequently this rash, include living in overcrowded conditions. The condition is more frequently observed in children aged between 5 and 15 years old.

Common Symptoms and Presentation of Erythema Marginatum Rheumaticum

This condition typically presents as a rash characterised by pink or red macules (flat spots) or papules (small lumps) that spread outwards. These lesions often form annular (ring-like) or polycyclic plaques with central clearing, meaning the centre of the rash returns to normal as the edges advance. The rash is usually painless, does not itch, and may turn white when pressure is applied. It commonly appears on the trunk and limbs, sparing the face. The patches can merge to form larger, snake-like patterns and may appear in groups over several days to weeks. The rash is often more noticeable when exposed to heat and can persist intermittently for weeks to months, sometimes lasting from a few hours to several weeks.

Diagnosis of Erythema Marginatum Rheumaticum

The diagnosis of Erythema marginatum rheumaticum is often made through visual assessment of the characteristic red, ring-like skin patches. For a definitive diagnosis of associated acute rheumatic fever, clinicians typically use the modified Jones criteria, which involve a combination of major and minor clinical findings, alongside evidence of a recent streptococcal infection (e.g., positive throat swab or elevated antibody titres). Additional investigations may include blood tests, echocardiography, and sometimes a skin biopsy to examine for inflammation around blood vessels.

Treatment and Management Considerations for Erythema Marginatum Rheumaticum

There is no specific treatment for the erythema marginatum rash itself, as it tends to resolve on its own. Management focuses on treating the underlying condition, most commonly acute rheumatic fever. This typically involves a full course of antibiotics, such as penicillin, to eradicate the streptococcal infection. While the rash may persist independently, treating the underlying rheumatic fever is crucial to prevent complications like carditis or Sydenham's chorea.

Frequently asked questions

What is the ICD-11 classification for Erythema marginatum rheumaticum?
Erythema marginatum rheumaticum is classified under EA50 Toxin-mediated cutaneous reactions to distant or systemic bacterial infection.
How is Erythema marginatum rheumaticum described in ICD-11?
It is described as a cutaneous exanthem occurring in rheumatic fever, characterized by widespread pink or red macules or papules that expand centrifugally with central clearing to form annular or polycyclic erythematous plaques, which may persist intermittently for weeks to months.
What is the ICD-11 code for Erythema marginatum rheumaticum?
The ICD-11 code for Erythema marginatum rheumaticum is EA50.0.
When should the ICD-11 code EA50.0 be used?
The ICD-11 code EA50.0 should be used for the specific condition Erythema marginatum rheumaticum, which is a cutaneous manifestation associated with rheumatic fever.
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