Erythema marginatum

Presentation

The rings are barely raised and are non-itchy. The face is generally spared.

The condition is characterised by circular, non-pruritic, erythematous rashes that form on the trunk and extremities of the body. The rash has a known serpiginous edge, and often appears and disappears spontaneously over time. Histological examination of the rash identifies infiltration of mononuclear cells and neutrophils in the papillary and upper half of the reticular dermis layer.

Associated conditions

It occurs in less than 10% of patients with acute rheumatic fever (ARF), but is considered a major Jones criterion when it does occur. The four other major criteria include carditis, polyarthritis, Sydenham's chorea, and subcutaneous nodules. In this case, it is often associated with Group A streptococcal infection, otherwise known as Streptococcus pyogenes infection, which can be detected with an ASO titer.

It is an early feature of acute rheumatic fever, though not pathognomonic of it. It some cases it may be associated with mild myocarditis (inflammation of heart muscle).

The condition is also seen as a precursor to or accompanying an attack of angioedema, and is seen in conditions like allergic drug reactions, sepsis, and glomerulonephritis.

It often occurs as a harbinger of attacks in hereditary angioedema. In this case, it may occur several hours or up to a day before an attack.

Diagnosis

Types

Some sources distinguish between the following:

• "Erythema marginatum rheumaticum"
• "Erythema marginatum perstans" The diagnosis of erythema marginatum can be made during examination of the skin's appearance. A skin biopsy may be performed if needed to confirm the diagnosis. Medical history and family history may also be considered.

Treatment

Erythema marginatum can be treated with hydrocortisone and adrenocorticotropic hormone (ACTH).

In cases where the condition is associated with ARF and severe carditis, corticosteroids are indicated alongside the classic treatment protocol for ARF which is a 10-day course of oral Penicillin. Alternatively, one dosage of Penicillin G benzathine may be injected intramuscularly followed by a daily course of oral Amoxicillin for a total of 10 days. In cases of Penicillin allergy, a Cephalosporin or Macrolide may be considered. To avoid recurrences of ARF, secondary prevention is warranted. This may include a period of antibiotic prophylaxis determined by the presence of carditis and the amount of remaining heart damage.

References

References

1. Bygum, Anette. (March 2011). "Rapid resolution of erythema marginatum after icatibant in acquired angioedema". Acta dermato-venereologica.
2. Hinzey, E. (June 2023). "Erythema Marginatum". Patient Education Reference Center.
3. {{DorlandsDict. three/000036710. erythema marginatum
4. Bolognia, Jean L.. (2022). "Dermatology Essentials". Elsevier.
5. (October 2004). "Erythema marginatum and hereditary angioedema". South. Med. J..
6. Kliegman, R.M.. (2011). "Group A streptococcus. In: Nelson textbook of pediatrics". Elsevier Saunders.
7. Vijayan, Vini. (July 2023). "Erythema Marginatum". The Journal of Pediatrics.
8. Sharma, Shreya. (December 2015). "Erythema Marginatum". Indian Pediatrics.
9. Tani, L.T.. (2003). "Rheumatic fever in children younger than 5 years: is the presentation different?". Pediatrics.
10. Wolfson, Allan B.. (2012). "Harwood-Nuss' Clinical Practice of Emergency Medicine". Wolters Kluwer Health.
11. [http://www.japi.org/january2007/PC-45.pdf Erythema Marginatum Pictorial CME]
12. Burke, J. B.. (1955-08-01). "Erythema Marginatum". Archives of Disease in Childhood.
13. (2004). "Oski's Essential Pediatrics". Lippincott Williams & Wilkins.
14. (2021). "Group A Streptococcal Infections. In: Red Book: 2021-2024 Report of the Committee on Infectious Diseases". American Academy of Pediatrics.