What Is It, Causes, Signs and Symptoms, Diagnosis, and More
Author:Maria Emfietzoglou, MD
Editors:Alyssa Haag,Józia McGowan, DO,Kelsey LaFayette, DNP, ARNP, FNP-C
Illustrator:Jessica Reynolds, MS
Copyeditor:Stacy Johnson, LMSW
What is erythema marginatum?
Erythema marginatum is a type of skin rash that typically presents as a round lesion with a pale center and a slightly raised, red border. It typically appears on the limbs or trunk. Usually, it indicates the presence of an underlying disease, such as rheumatic fever, hereditary angioedema, Lyme disease, or an allergic reaction to medications.
What causes erythema marginatum?
Several medical conditions can cause erythema marginatum. However, the most common is rheumatic fever, an inflammatory disease that can damage the heart tissue and lead to rheumatic heart disease. Rheumatic fever develops after streptococcal pharyngitis, or strep throat, which is inflammation of the throat due to the bacteria Streptococcus pyogenes (i.e., Group A streptococcus), characterized by fever and sore throat.
Erythema marginatum may also be a sign of hereditary angioedema, a condition usually caused by a deficiency of the C1 esterase inhibitor. This protein regulates the complement system (i.e., part of the immune system involved with antibodies and phagocytic cells). Angioedema typically refers to localized skin swelling; however, some angioedema attacks may be characterized as anaphylactic reactions, which are systemic, severe, and often life-threatening.
Another cause of erythema marginatum is Lyme disease, an infectious disease caused by the bacterium Borrelia burgdorferi. It is a vector-borne infection transmitted by the bite of an infected deer tick, which is usually found in wooded areas, thick brush, and tall grass. It’s important to note that in Lyme disease, most individuals present with the characteristic bull’s eye rash, also known as erythema migrans, but may also experience erythema marginatum.
What are the signs and symptoms of erythema marginatum?
Erythema marginatum typically appears as a skin rash on the trunk or limbs, characterized by a pink, faint center (i.e., central clearing) surrounded by a slightly raised red border. Erythema marginatum can be present for hours to days and can be accompanied by pruritus or pain.
In cases of rheumatic fever, individuals may also have a fever and a variety of other clinical findings, most commonly migratory polyarthritis of the joints, where multiple large joints (e.g., elbows, knees) become transiently inflamed and painful. Some individuals may also have pancarditis, or inflammation of all three layers of the heart tissue, including the endocardium, myocardium, and pericardium. Chest pain and a friction rub from the inflamed layers rubbing against one another can result. In addition to joint and heart problems, individuals with rheumatic fever can develop subcutaneous nodules or firm lumps of collagen under the skin. They can also have Sydenham chorea, characterized by rapid movements of the face and the arms.
In cases of hereditary angioedema, the rash does not cause intense itching but rather tenderness and burning. Angioedema typically causes facial, tongue, larynx, abdomen, arms, or leg swelling. When it involves the gastrointestinal mucosa, it can cause nausea, vomiting, or diarrhea. It may disrupt breathing and be life-threatening if it involves the upper part of the larynx.
Individuals with Lyme disease may have fever, fatigue, headache, and lymphadenopathy. Other clinical manifestations include carditis, or inflammation of the heart, which typically manifests as a conduction disorder (e.g., atrioventricular block); meningitis, or inflammation of the meninges; palsy of the facial cranial nerve, also known as Bell palsy, where facial muscles become weak or even paralyzed; as well as arthritis, or inflammation of the joints.Individuals with erythema marginatum caused by allergic reactions may also experience other symptoms depending on the severity of the reaction, which can include urticaria, also called hives, which are slightly raised, well-defined wheals that are 1 mm to 10 cm in diameter. They are usually red; blanch with pressure; are extremely itchy; can present anywhere in the body; recur rapidly. In more severe cases, an allergic reaction can lead to anaphylaxis, characterized by dyspnea, hypotension, or syncope.
How is erythema marginatum diagnosed?
Diagnosis of erythema marginatum and identification of the underlying medical condition is based on the patient's medical history, physical examination, and various diagnostic tests. Other types of skin rashes should also be considered and excluded, which include erythema migrans, which tends to stay localized to one location; erythema multiforme, which can spread over the body, but appears as raised, crusted patches; and erythema annulare centrifugum, which appears as itchy scales usually on the face.
Rheumatic fever is typically suspected if there is a history of Group A streptococcal infection. The major criteria for rheumatic fever diagnosis, collectively known as the Jones criteria, include five signs: non-pruritic erythema marginatum, arthritis, carditis, subcutaneous nodules, and Sydenham chorea. Some minor criteria help make the diagnosis, such as fever; joint pain; changes in an individual’s electrocardiogram (ECG); elevated inflammatory markers, like C-reactive protein; and a blood test that shows evidence of previous streptococcal infection, known as anti-streptolysin-O, or ASO.Blood tests to assess levels of complement C1 inhibitor may also be ordered if hereditary angioedema is suspected. Diagnosis for Lyme disease can be confirmed using tests that detect antibodies against the Borrelia burgdorferi, such as enzyme-linked immunosorbent assay, ELISA, immunofluorescence assay, or IFA. These can be further followed by Western blot, a gold standard in detecting Borrelia burgdorferi IgG antibodies. Finally, the diagnosis of an allergic reaction is typically based on the patient's history and physical exam.
How is erythema marginatum treated?
Treatment of erythema marginatum depends on the underlying cause. In rheumatic fever, treatment includes penicillin, an antibiotic used to treat the underlying streptococcal infection, and anti-inflammatory medications, like aspirin, to relieve fever and pain and reduce inflammation. Although hereditary angioedema cannot be cured, some medicines can be used to treat the attacks, including icatibant, a medication that can reduce swelling, and C1 esterase inhibitor replacement, which can increase the levels of C1 esterase inhibitor in the body. In addition, there are medications, like plasma kallikrein inhibitors (e.g., berotralstat) and androgenic hormones (e.g., danazol), that can be used to prevent future attacks. Finally, fresh frozen plasma is sometimes used for those with hereditary angioedema during acute attacks to improve symptoms.
Lyme disease management includes oral antibiotics, such as doxycycline, cefuroxime, and amoxicillin, and intravenous antibiotics, such as ceftriaxone, for individuals with systemic complications, such as carditis or meningitis.
Finally, for allergic reactions, treatment can include withdrawing the offending agent (when applicable), as well as a variety of medications, such as antihistamines (e.g., loratadine) and corticosteroids (e.g., prednisone). Severe allergic reactions can be treated with epinephrine.
What are the most important facts to know about erythema marginatum?
Erythema marginatum is a skin rash that typically appears on the limbs or trunk, and it has a pink, pale center surrounded by a red, slightly raised border. In most cases, it indicates the presence of an underlying disease, such as rheumatic fever, hereditary angioedema, Lyme disease, or an allergic reaction. Depending on the underlying cause, erythema marginatum may be accompanied by various other symptoms, including joint pain in a rheumatic fever; facial swelling in hereditary angioedema; facial nerve palsy in Lyme disease; and dyspnea if there is an underlying severe allergic reaction. Diagnosis is primarily based on the patient's history, physical examination, and blood tests to confirm certain underlying medical conditions. Treatment depends on the cause and can include a variety of medications.
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Related linksBorrelia burgdorferi (Lyme disease)
Rheumatic heart disease
Type I hypersensitivity
Resources for research and reference
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