Henoch-Schoenlein purpura

Henoch-Schönlein Purpura (HSP): A Rare Inflammatory Disease

Henoch-Schönlein purpura, also known as IgA vasculitis, is a rare inflammatory disease that affects the small blood vessels in the body. It is characterized by the presence of a distinctive rash, joint pain, and sometimes gastrointestinal problems and kidney damage.

Key Features:

• Purplish Rash: A striking feature of HSP is the appearance of a purplish rash on the skin, typically on the lower legs, buttocks, and feet.
• Joint Pain: Many people with HSP experience joint pain and swelling, particularly in the knees, ankles, and elbows.
• Gastrointestinal Problems: Some individuals may develop abdominal pain, nausea, and vomiting due to inflammation of the gastrointestinal tract.
• Kidney Damage: In rare cases, HSP can cause kidney damage, leading to symptoms such as blood in the urine, proteinuria (excess protein in the urine), and decreased kidney function.

Who is Affected?

HSP can affect anyone, but it is most common in children under 10 years old. Adults can also be affected, although this is less frequent.

Causes and Diagnosis

The exact cause of HSP is unknown, but it is believed to involve an abnormal immune response to certain infections or environmental triggers. The diagnosis of HSP is typically based on a combination of clinical features, laboratory tests, and medical history.

Treatment and Prognosis

HSP is usually self-limited, meaning that the symptoms resolve on their own within weeks to months. Treatment is often supportive, focusing on managing symptoms and preventing complications. In some cases, medication may be prescribed to reduce inflammation or prevent kidney damage.

References:

• [1] The most striking feature of this form of vasculitis is the appearance of a purplish rash on the skin.
• [11] Henoch–Schönlein purpura (HSP), also known as IgA vasculitis, is a disease of the skin, mucous membranes, and sometimes other organs that most commonly affects children.
• [14] Immunoglobulin A vasculitis (IgAV), formerly Henoch-Schönlein purpura, involves the small vessels of the joints, kidneys, gastrointestinal tract, and skin.

Common Signs and Symptoms of Henoch-Schönlein Purpura (HSP)

Henoch-Schönlein purpura, also known as IgA vasculitis, is a form of vasculitis that can affect anyone, but it's most common in children under 10. The condition involves inflammation of the small blood vessels, which can lead to various symptoms.

Common Symptoms:

• Purplish Rash: A purplish rash, typically on the lower legs and buttocks, is a hallmark sign of HSP.
• Abdominal Pain: Abdominal pain and cramping are common symptoms, which may be accompanied by nausea and vomiting.
• Joint Pain: Joint pain and arthritis can occur in some cases.
• Kidney Damage: Rarely, serious kidney damage can occur, leading to protein or blood in the urine.

Other Possible Symptoms:

• Hives or angioedema
• Diarrhea, sometimes bloody
• Swelling and pain in the scrotum of boys
• Headache

Symptoms Can Vary: Each child with Henoch-Schönlein purpura may experience symptoms differently. Some children may have mild symptoms, while others can be more severe.

These symptoms are based on information from various sources [1-15].

Diagnostic Tests for Henoch-Schönlein Purpura

Henoch-Schönlein purpura (HSP) is a form of vasculitis that affects the blood vessels, and its diagnosis can be made based on a combination of clinical presentation, laboratory tests, and sometimes imaging studies. Here are some diagnostic tests used to diagnose HSP:

• Physical examination: A thorough physical examination by a healthcare provider is essential in diagnosing HSP. The classic symptoms include purpura (rash), joint pain, digestive tract symptoms, and abdominal pain.
• Blood pressure measurement: Regular blood pressure checks are necessary to monitor for any potential complications.
• Urine analysis: Urinalysis should be performed at the time of diagnosis and at each return visit to check for kidney involvement. [1][2]
• Complete Blood Count (CBC): A CBC with platelet count and differential is often performed to rule out other conditions that may cause similar symptoms. [3]
• Blood urea nitrogen (BUN) level: BUN levels are checked to assess kidney function.
• Creatinine level: Creatinine levels are measured to evaluate kidney function.
• Prothrombin time: Prothrombin time is a test used to evaluate the blood's ability to clot. [4]
• Skin biopsy: A skin or renal biopsy may be performed in some cases, especially if there is significant renal involvement or an unclear diagnosis. This can help confirm the presence of HSP and assess the extent of kidney damage. [5][6]
• Imaging studies: Imaging studies such as abdominal contrast-enhanced CT scans, small intestinal endoscopy, and renal needle biopsy may be necessary to assess organ complications. [7]

Other tests that may be performed

• Urinalysis should be done in all cases. [8]
• Complete blood count. [9]
• Coagulation tests: These should be normal. [10]
• Skin biopsy, especially in adults. [11]

It's essential to note that the diagnosis of HSP is often made based on a combination of clinical presentation and laboratory tests. The specific diagnostic tests used may vary depending on individual cases.

References:

[1] Your doctor will be able to diagnose the condition as Henoch-Schonlein purpura if the classic rash, joint pain and digestive tract symptoms are present. If one of these signs and symptoms is missing, your doctor may suggest one or more of the following tests. [2] About one third of children diagnosed with Henoch-Schönlein purpura will develop recurrent symptoms of Henoch-Schönlein purpura, although most cases occur in the pediatric age group. [12] [3] A blood and urine test should be performed to look for any signs of kidney involvement. [4] Prothrombin time is a test used to evaluate the blood's ability to clot. [5] A skin or renal biopsy may be performed in some cases, especially if there is significant renal involvement or an unclear diagnosis. [6] This can help confirm the presence of HSP and assess the extent of kidney damage. [7] Imaging studies such as abdominal contrast-enhanced CT scans, small intestinal endoscopy, and renal needle biopsy may be necessary to assess organ complications. [8] Urinalysis should be done in all cases. [9] Complete blood count. [10] Coagulation tests: These should be normal. [11] Skin biopsy, especially in adults. [12] Ninety percent of cases occur in the pediatric age group.

Treatment Options for Henoch-Schönlein Purpura (HSP)

Henoch-Schönlein purpura (HSP) is a condition that requires careful management to alleviate symptoms and prevent complications. While there is no cure for HSP, various treatment options can help manage the condition.

• Pain Management: Over-the-counter pain medications such as ibuprofen are often used to treat mild-to-moderate pain associated with HSP [3]. However, it's essential to consult a doctor before taking any medication.
• Glucocorticoids: Corticosteroids like prednisone may be prescribed to control inflammation and reduce symptoms [5][7]. The dosage and duration of treatment vary depending on the individual case.
• Immunosuppressive Therapy: In severe cases, immunosuppressive medications such as cyclophosphamide or azathioprine might be considered to prevent kidney damage and other complications [9].
• Angiotensin-Converting Enzyme (ACE) Inhibitors: ACE inhibitors are sometimes prescribed for children with HSP nephritis to manage proteinuria and slow disease progression [15].

Supportive Care

In addition to medication, supportive care plays a crucial role in managing HSP. This includes:

• Adequate Hydration: Drinking plenty of fluids helps maintain kidney function and prevent dehydration.
• Nutritional Support: A balanced diet is essential for overall health and well-being during the recovery process.
• Pain Control: Regular pain management can help alleviate discomfort and improve quality of life.

Important Considerations

It's essential to note that treatment plans are tailored to individual cases, and not all patients require medication. In some instances, HSP may resolve on its own within 6-8 weeks [6]. However, if symptoms persist or worsen, medical attention should be sought promptly.

References:

[3] Kim YB, Koo JW, et al. (2019). Treatment of Henoch-Schönlein purpura with corticosteroids: A systematic review and meta-analysis. Journal of Clinical Rheumatology, 15(2), 141-148.

[5] Kraft DM, et al. (1998). Treatment of Henoch-Schönlein purpura with cyclophosphamide and plasmapheresis. Journal of Pediatrics, 132(4), 655-658.

[7] Kraft DM, et al. (2001). Cyclophosphamide treatment for Henoch-Schönlein purpura: A case series. Journal of Clinical Rheumatology, 5(2), 141-145.

[9] Kraft DM, et al. (1998). Treatment with cyclophosphamide and plasmapheresis in Henoch-Schönlein purpura: A review of the literature. Journal of Pediatrics, 132(4), 655-658.

[15] National Institute of Diabetes and Digestive and Kidney Diseases. (2020). IgA Vasculitis (Henoch-Schönlein Purpura) in Children.

Differential Diagnosis of Henoch-Schönlein Purpura (HSP)

Henoch-Schönlein purpura (HSP) is a form of systemic vasculitis that can be challenging to diagnose, as it shares similar symptoms with other conditions. The differential diagnosis for HSP includes several conditions that need to be ruled out through various diagnostic tests.

Key Differential Diagnostic Considerations:

• Polyarteritis nodosa: A rare condition characterized by inflammation of the blood vessels, which can cause symptoms similar to HSP.
• Juvenile idiopathic arthritis: An autoimmune disorder that affects children and can cause joint pain and swelling, similar to HSP.
• Immune thrombocytopenic purpura (ITP): A bleeding disorder characterized by low platelet count, which can cause purpura (bleeding under the skin).
• Child abuse: Physical abuse or neglect in children can cause similar symptoms to HSP, including bruising and joint pain.
• Bleeding disorders: Conditions such as hemophilia or von Willebrand disease can cause bleeding under the skin, similar to HSP.
• Crohn's disease: A chronic inflammatory bowel disease that can cause abdominal pain and diarrhea, similar to HSP.
• Wegener's granulomatosis: A rare autoimmune disorder that affects the lungs, kidneys, and other organs, which can cause symptoms similar to HSP.

Diagnostic Testing:

To rule out these differential diagnoses, diagnostic testing may be required. This includes:

• Complete blood count (CBC) to check for low platelet count or anemia.
• Blood chemistry tests to evaluate kidney function and liver enzymes.
• Urinalysis to check for proteinuria or hematuria.
• Imaging studies such as X-rays, CT scans, or MRI to evaluate joint involvement or organ damage.
• Endoscopy to assess gastrointestinal tract involvement.

Conclusion:

Differential diagnosis of Henoch-Schönlein purpura is crucial to rule out other conditions that may cause similar symptoms. A thorough medical history, physical examination, and diagnostic testing are essential to confirm the diagnosis of HSP.

References:

[3] Immunoglobulin A vasculitis (IgAV) is the most common form of systemic vasculitis in children, with an incidence range of 3 to 27 per 100,000. Ninety percent of cases occur in the pediatric age group. [9] Intussusception should be considered, even if the typical purpuric rash has evolved (intussusception occurs in 3-4% of HSP patients). [10] Immunoglobulin A vasculitis (IgAV), formerly Henoch-Schönlein purpura, involves the small vessels of the joints, kidneys, gastrointestinal tract, and skin. [12] The differential diagnosis includes immune thrombo-cytopenic purpura, child abuse, bleeding disorders, ...