Henoch-Schonlein purpura (HSP)

Henoch-Schonlein purpura (HSP), or hemorrhagic vasculitis, is an inflammatory condition affecting small blood vessels (capillaries, venules, and arterioles) in the skin, joints, intestines, and kidneys, less commonly involving the lungs and central nervous system. The disease predominantly affects children, presenting with red rashes on the skin (usually on the legs and buttocks) and moderate to severe colicky abdominal pain and joint pain.

Symptoms of Henoch-Schonlein purpura:

This pathology is classified as systemic vasculitis, indicating involvement of multiple organs and tissues in the pathological process. Synonyms include hemorrhagic vasculitis, Henoch-Schonlein purpura, capillarotoxicosis, allergic purpura, and anaphylactoid purpura.


The prevalence of this condition among children is 13.5–25 cases per 100,000. It primarily affects boys aged 4–12 years, although the gender difference disappears by adolescence. In adults, Henoch-Schonlein purpura is more than 10 times less common (affecting 13–14 people per million), but it tends to have a more severe course than in children.

Causes of Henoch-Schonlein purpura:

The causes of this condition remain unknown. In 40% of adults and children, the disease develops after exposure to various triggers and risk factors. Possible precursors to the onset of the disease include:

  • Acute bacterial and viral infections, often of the upper respiratory tract. Children with HSP often have detectable mycobacteria tuberculosis, hepatitis B and C viruses, Epstein-Barr virus, herpes simplex virus types 1 and 2, cytomegalovirus, as well as chlamydia and toxoplasma. Unlike children, the onset of the disease in adults is less associated with seasonality and preceding infections.
  • Medications: antibiotics, angiotensin-converting enzyme inhibitors, nonsteroidal anti-inflammatory drugs (NSAIDs), and others.
  • Vaccination (against measles, mumps, typhoid, paratyphoid, etc.), especially following or immediately after acute respiratory viral infections. It is worth noting that there is currently no data indicating that any specific vaccine definitively causes the disease.
  • Food allergy.
  • Insect bites.
  • Hypothermia.
  • Trauma.
  • Occasionally, hemorrhagic vasculitis complicates pregnancy, periodic illness, cirrhosis of the liver, and malignant neoplasms.
Skin lesions in Henoch-Schönlein disease

Symptoms of Henoch-Schonlein purpura (HSP), or hemorrhagic vasculitis:

  1. Fever: Fever can occur in both children and adults. In children, the disease often starts acutely with body temperature rising to 38°C or higher. Fever is less common in adults.
  2. Skin involvement: The most common manifestation of the disease is hemorrhagic rash, observed in all patients. The rash appears as reddish discoloration of the skin or mucous membranes due to bleeding, usually from capillaries. Bleeding can manifest as flat or pinpoint spots (petechiae), or slightly raised lesions (palpable purpura). The rash is symmetrical and accompanied by itching. Rashes often appear on the legs, initially on the feet and shins, then spreading to the thighs and buttocks. Less commonly, rashes are observed on the abdomen, back, and arms. Sometimes, petechiae and purpura are accompanied by bright pink or red (erythematous) patches, papules, and blisters.
  3. Joint involvement: The second most common symptom of HSP is joint syndrome, which develops in 60–100% of patients, more frequently in adults. It manifests as joint pain and less commonly inflammation (arthritis) of mainly large joints: ankles, knees, less frequently wrists, and elbows. Patients may experience muscle pain, swelling of the feet, and periarticular tissues. Joint symptoms usually occur simultaneously with skin rashes but can be the initial manifestation of the disease. In adults, arthritis can be prolonged, which is not characteristic of children.
  4. Abdominal pain and other gastrointestinal manifestations: These are observed in 2/3 of patients. The pain is often colicky but can also be constant. Sometimes it is mild, while other times it is so severe that patients cry out in pain. Nausea and repeated vomiting, leading to dehydration, may also occur. In rare cases, HSP is accompanied by gastrointestinal bleeding, characterized by black, tarry stools and “coffee ground” vomiting.
  5. Renal involvement: Kidney involvement most commonly manifests as isolated urinary syndrome, i.e., increased protein and red blood cells in the urine. Therefore, kidney damage may only be suspected in severe cases when renal and extrarenal manifestations of nephritis occur: visible blood in the urine, elevated blood pressure, edema, decreased urine output, and the development of renal failure.

Complications of Henoch-Schonlein purpura (HSP), or hemorrhagic vasculitis:

Skin manifestations of Henoch-Schonlein purpura typically resolve without sequelae, but sometimes the pathology progresses with the appearance of blood-filled blisters or areas of skin necrosis. Such complications are characteristic of a fulminant course of the disease but can also develop with a protracted course.

Complications affecting the gastrointestinal tract include intussusception (the telescoping of one portion of the intestine into another), intestinal obstruction, intestinal perforation leading to peritonitis (inflammation of the abdominal lining), and gastrointestinal bleeding. Moderately severe, non-life-threatening bleeding occurs in 50% of cases, while dangerous bleeding occurs in 5%. Intussusception is likely associated with excessive contraction of the intestinal wall. Abdominal colicky pain typically accompanies intussusception. Intestinal obstruction is caused by either intussusception or a large hematoma in the intestinal wall.

Hemorrhagic rash and areas of skin death

Renal complications include a special form of nephritis called subacute malignant glomerulonephritis, which leads to renal failure over several months.

Other complications are related to rare forms of hemorrhagic vasculitis:

  • Pulmonary involvement may lead to pulmonary hemorrhage, sometimes fatal. Pulmonary symptoms are associated with inflammation of the capillaries between the alveolar septa and deposition of IgA in them.
  • Pathological changes in the central nervous system (CNS) are very rare and manifest as vasculitis of the brain vessels. This may cause epileptiform seizures, intense headaches due to intracranial bleeding, and bleeding into the substance of the brain and spinal cord leading to stroke (infarction). Clinically, this condition presents with paralysis (muscle weakness), polyneuropathy (nerve damage), and coma.

Diagnosis of Henoch-Schonlein purpura (HSP), or hemorrhagic vasculitis:

In children, the main diagnostic criteria for Henoch-Schonlein purpura are those proposed by the European League Against Rheumatism (EULAR), the Pediatric Rheumatology International Trials Organization (PRINTO), and the Pediatric Rheumatology European Society (PReS).

The primary criterion that raises suspicion of the pathology is the typical skin-hemorrhagic syndrome (rash, purpura, etc.). If there are no skin manifestations, it indicates another condition. Additional diagnostic criteria include:

  • Abdominal pain (diffuse or colicky with acute onset) and/or gastrointestinal bleeding.
  • Acute joint pain without swelling or arthritis with acute onset, swelling, and joint dysfunction.
  • Renal involvement: proteinuria > 0.3 g/day or albumin/creatinine ratio in the morning urine > 30 mmol/mg; visible blood in the urine or > 5 erythrocytes per high-power field on laboratory examination.
  • Pathomorphological changes: deposition of IgA in blood vessels or kidneys.

For diagnosing the disease in adults, the criteria developed by the American College of Rheumatology (ACR) are used:

  • Onset of the disease before the age of 20.
  • Palpable purpura.
  • Acute abdominal pain.
  • Granulocytes in the walls of arterioles and venules detected by biopsy.

The presence of two or more criteria in a patient allows a diagnosis of Henoch-Schonlein purpura with an accuracy of over 87%.

Other diagnostic methods are supplementary and are conducted to determine the activity and severity of the disease.

Additional investigations include:

Laboratory tests:

  • Complete blood count may show elevated levels of leukocytes, neutrophils, eosinophils, and platelets.
  • Biochemical analysis may reveal elevated levels of C-reactive protein (CRP), IgA, immune complexes, and cryoglobulins; significantly increased levels of anti-streptolysin-O (ASLO), indicating streptococcal infection, may be observed in children.
  • Urinalysis shows changes only in the presence of nephritis: protein and blood appear in the urine. In case of kidney dysfunction, creatinine and urea levels may increase. Rheumatoid factor is elevated in 30-40% of patients.

Instrumental examinations:

  • Endoscopic examinations of the gastrointestinal tract. Esophagogastroduodenoscopy may reveal superficial and multiple petechiae, erosions, ulcers, and hematoma-like protrusions up to 1 cm in diameter in the upper gastrointestinal tract. Colonoscopy may detect 1-2 cm ulcers in the large intestine, predominantly in the sigmoid and rectum.
Differential diagnosis:

During diagnosis, the physician excludes diseases that also manifest with hemorrhagic rash. Such diseases include infective endocarditis, systemic lupus erythematosus, meningococcemia, chronic liver diseases, nonspecific ulcerative colitis, Crohn’s disease, malignancies, thrombocytopenic purpura, and others.


Treatment of Henoch-Schonlein purpura (HSP), or hemorrhagic vasculitis:

The main treatment goals are to alleviate symptoms and prevent complications, aiming for remission and preventing relapses.

Non-pharmacological therapy:
During the acute phase of the disease (at the onset or during a recurrence), bed rest is recommended, typically lasting 2-4 weeks, depending on the severity of symptoms. Patients may gradually resume activity, and therapeutic exercises may be prescribed based on individual capabilities.

Dietary modifications are crucial, focusing on a hypoallergenic diet, which involves excluding:

  • Obligatory allergens such as nuts (especially peanuts), milk and dairy products, gluten, soy, sesame, mustard, fish and shellfish, eggs, cocoa, coffee, citrus fruits, etc.
  • Individual allergens.
  • Extractive substances found in spices and other products that irritate the gastrointestinal mucosa and increase its permeability to food allergens.

For severe abdominal pain, particularly with signs of gastrointestinal bleeding, a diet known as Diet No. 1 according to Pevzner is prescribed. This diet involves consuming easily digestible foods: all meals should be pureed, porridge-like, or liquid in consistency; foods can be boiled or steamed; the eating schedule includes 5-6 small meals per day, and food should be consumed warm.

In cases of glomerulonephritis with nephrotic syndrome, patients are typically placed on a low-salt, low-protein diet.

Pharmacological therapy:
All patients, regardless of the form of the disease, are prescribed antiplatelet therapy to prevent thrombosis, using medications such as Curantil (Dipyridamole), Trental (Pentoxifylline), etc. In cases of mild HSP, antiplatelet therapy is usually sufficient to alleviate symptoms. In severe cases, the physician may prescribe two drugs from the antiplatelet group. The pediatric dosage is calculated based on the child’s weight.

In moderate to severe forms of the disease and hypercoagulability, anticoagulants may be used. Heparin is often administered subcutaneously four times a day for 10-14 day courses. In cases of specific nephritis, the course may last up to one and a half months. Low-molecular-weight heparins (e.g., Fraxiparine) may also be used, administered once daily with fewer complications.

Glucocorticoids are indicated for persistent, relapsing skin purpura, presence of necrosis (black crusts) on the skin, severe abdominal pain, and nephritis. Treatment is typically conducted alongside heparin therapy. According to some authors, early administration of glucocorticoids, especially in patients with abdominal pain, reduces the risk of kidney involvement.

For severe kidney involvement, high doses of glucocorticoids and immunosuppressants such as Azathioprine, Cyclophosphamide (Cytoxan) are prescribed, which suppress the immune system. Plasma exchange may also be considered.

For persistently recurrent skin syndromes in adults, Sulfasalazine and Colchicine are occasionally used.

Symptomatic treatment includes antihistamines (Zyrtec, etc.), enterosorbents, and non-steroidal anti-inflammatory drugs (NSAIDs).

In case of infection foci in the oral cavity, nasopharynx, bile ducts, etc., antibacterial therapy is administered.