Cutaneous Manifestations of Acute Rheumatic Fever

Introduction:

Acute rheumatic fever (ARF) is an autoimmune inflammatory disease that can occur after an untreated or inadequately treated infection with certain strains of group A streptococcus bacteria, such as streptococcal pharyngitis (commonly known as strep throat) or less commonly, streptococcal skin infections. Acute rheumatic fever primarily affects children and young adults. The immune system's response to the streptococcal infection triggers an inflammatory reaction that can lead to widespread inflammation and damage to various organs, including the heart, joints, central nervous system, and skin.

What Are the Symptoms of Acute Rheumatic Fever?

The symptoms of acute rheumatic fever can vary among individuals, and not all symptoms may be present in every case. The onset of acute rheumatic fever usually occurs a few weeks after a group A streptococcal infection. Common symptoms of acute rheumatic fever include:

• Fever - A high body temperature is a common sign of acute rheumatic fever.

• Joint Pain - Acute rheumatic fever typically affects the larger joints, such as the knees, ankles, elbows, and wrists. Joint pain often shifts from one joint to another. The affected joints may be swollen, tender, and warm to the touch.

• Cardiac Symptoms - Acute rheumatic fever can involve the heart and cause inflammation of the heart muscle (myocarditis) or heart valves (valvulitis). Symptoms may include chest pain, shortness of breath, heart palpitations, and fatigue. In severe cases, it can lead to rheumatic heart disease.

• Sydenham’s Chorea - This is a neurological manifestation characterized by involuntary, purposeless movements of the limbs and facial muscles. It may also involve muscle weakness, slurred speech, and emotional disturbances.

• Skin Manifestations - Certain skin symptoms can occur in acute rheumatic fever, such as a rash known as erythema marginatum. Skin (subcutaneous) nodules may also be present in some individuals.

What Is Erythema Marginatum in Acute Rheumatic Fever?

Erythema marginatum is a specific type of skin rash that can occur in individuals with acute rheumatic fever. Erythema marginatum is rare in adults but occurs in about 10 % of first attacks of acute rheumatic fever in children.

Symptoms of erythema marginatum in acute rheumatic fever include:

• Characterized erythematous (red), non-painful, and non-pruritic (not itchy) lesions.

• Lesions are typically macular (flat) and may have a serpiginous (wavy, snake-like) pattern.

• The rash is commonly found on the trunk or proximal extremities (such as the upper arms and thighs).

• Lesions tend to extend outward with central clearing, creating a well-defined border between the affected and unaffected skin.

• The rash comes and goes over time.

What Are Subcutaneous Nodules in Acute Rheumatic Fever?

Subcutaneous nodules are small, painless lumps that develop beneath the skin. In the context of acute rheumatic fever, subcutaneous nodules are an uncommon manifestation, occurring in less than 2 % of patients.

Symptoms of subcutaneous nodules in acute rheumatic fever include:

• Characterized lesions of variable size, ranging from a fraction of an inch to approximately two inches in diameter.

• They tend to appear in clusters.

• They are typically found over joints, such as the elbows, knees, ankles, and knuckles. They can also occur on the back of the scalp and the vertebrae (backbone).

• The duration of these nodules can range from a few days to several months.

• The nodules are firm and round and can be easily moved beneath the skin.

• Subcutaneous nodules are often observed in cases of severe acute rheumatic fever and indicate involvement of the heart.

How to Diagnose Cutaneous Manifestations of Acute Rheumatic Fever?

The diagnosis of erythema marginatum and subcutaneous nodules in acute rheumatic fever is primarily clinical. The presence of these specific cutaneous manifestations, along with other clinical features and Jones diagnostic criteria, helps support the diagnosis of acute rheumatic fever. Additionally, doctors may consider laboratory investigations to assess for evidence of preceding streptococcal infection or to rule out other potential causes.

• Medical History - The doctor will gather information about the patient's medical history, including any recent symptoms of sore throat or group A streptococcus infection. The presence of preceding streptococcal pharyngitis or skin infection is important in diagnosing acute rheumatic fever and its cutaneous manifestations.

• Jones Criteria - Diagnosis of acute rheumatic fever requires the presence of 2 major criteria or 1 major criterion along with 2 minor criteria, along with specific laboratory findings. Evidence of a preceding group A streptococcus infection is one of the major criteria alongside symptoms like polyarthritis (arthritis affects a minimum of five joints), carditis (heart inflammation), subcutaneous nodules (lump under the skin), and Sydenham's chorea (uncontrollable, jerky movements of arms and legs). Minor criteria include fever, joint pain, increased inflammatory markers, and prolonged PR interval.

• Clinical Evaluation - The doctor will perform a thorough physical examination and assess the appearance, distribution, and pattern of the skin lesions associated with acute rheumatic fever.

• Additional Tests - Additional tests may be performed to support the diagnosis and assess the severity of acute rheumatic fever.

  • Blood tests can measure markers of inflammation, such as erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), which may be elevated.

  • Antibody tests, such as antistreptolysin O (ASO) or anti-deoxyribonuclease B (anti-DNase B) titers, can detect evidence of recent streptococcal infection.

  • Electrocardiogram (ECG) and echocardiogram can help identify heart involvement.

  • Imaging techniques such as Doppler and color flow mapping can detect minor valvular defects.

How to Manage Cutaneous Manifestations of Acute Rheumatic Fever?

While cutaneous manifestations of acute rheumatic fever may be asymptomatic and resolve on their own, some individuals may require specific treatment. Treatment for cutaneous manifestations in acute rheumatic fever varies based on the specific manifestation and associated symptoms.

• Erythema Marginatum - This rash is typically non-pruritic and may fade and reappear over time. In most cases, it resolves spontaneously without treatment. However, if the rash is bothersome or persists for an extended period, symptomatic relief measures may be recommended.

• Subcutaneous Nodules - These nodules are usually painless and resolve on their own within a few weeks to months. Treatment may not be required unless there are associated symptoms or complications. In cases where nodules are causing discomfort or complications, medications may be prescribed.

• Antibiotics - Prompt treatment with antibiotics, usually Penicillin or other suitable antibiotics depending on allergies, is essential to eradicate the group A streptococcus infection that triggers acute rheumatic fever. This helps prevent further complications and reduce the risk of recurrent episodes.

• Anti-Inflammatory Medications - Aspirin is used to reduce inflammation and alleviate symptoms associated with cutaneous manifestations. These medications can help relieve pain, swelling, and discomfort. Corticosteroids may be considered in severe cases or when complications are present.

• Supportive Care - Proper hygiene and skin care are important in managing cutaneous manifestations. This includes keeping the affected areas clean, using mild soaps, and avoiding harsh chemicals or irritants that may worsen the skin condition. Moisturizers or soothing lotions may be recommended to alleviate dryness or itching.

Conclusion:

Cutaneous manifestations of acute rheumatic fever are typically self-resolving and benign, often resolving alongside treatment of the underlying infection. However, regular medical follow-up is essential to monitor the progression of these manifestations and ensure appropriate management. This allows for potential adjustments in medication dosages and the implementation of additional interventions if necessary.