Clinical Guides
Henoch-Schönlein Purpura (IgA Vasculitis)
A source-grounded paediatric guide to IgA vasculitis recognition, renal surveillance, complication triage and Indian clinical context, prepared for mandatory review.
MedNext Academy | 13 min read
Henoch-Schönlein Purpura (IgA Vasculitis)
A source-grounded paediatric guide to IgA vasculitis recognition, renal surveillance, complication triage and Indian clinical context, prepared for mandatory review.
Summary
Henoch-Schönlein purpura is the former name for immunoglobulin A vasculitis, an immune-mediated small-vessel vasculitis that most often affects children. The typical syndrome combines non-thrombocytopenic palpable purpura, usually over the lower limbs or buttocks, with one or more of abdominal pain, arthritis or arthralgia, kidney involvement, or biopsy evidence of IgA-predominant vasculitis. The rash can appear after abdominal or joint symptoms, so a child may need reassessment before the pattern becomes complete.
Most children recover, but reassurance must be paired with structured surveillance. Kidney involvement may initially be silent; blood pressure and urinalysis are therefore required at presentation and repeatedly after diagnosis. Macroscopic haematuria, proteinuria, hypertension, reduced kidney function or nephritic or nephrotic features require paediatric nephrology input. Severe abdominal pain can represent bowel oedema, bleeding or intussusception. Scrotal pain, neurological symptoms and pulmonary haemorrhage are uncommon but urgent.
Management is supportive for uncomplicated disease: hydration, activity according to comfort and safe analgesia. Non-steroidal anti-inflammatory drugs require caution and are avoided when kidney impairment or gastrointestinal bleeding is present. Corticosteroids may shorten selected severe abdominal or musculoskeletal symptoms but do not reliably prevent nephritis and should not be given prophylactically for that purpose. Renal immunosuppression is specialist-directed and depends on proteinuria, function and biopsy findings.
This guide uses current international recommendations and identifies the IAP treatment-guideline listing as Indian professional context. It does not assume uniform local testing or subspecialty access. Every child needs an explicit blood-pressure, urine and return-precaution plan. The draft remains quarantined pending paediatric review.
How Common Is It?
IgA vasculitis is the most frequently encountered systemic vasculitis of childhood, but it remains uncommon in general practice. The 2025 national recommendations report published incidence estimates ranging roughly from 6.8 to 30 per 100,000 children per year across studied populations. Rates vary with age, case definition, ethnicity, season, referral patterns and whether mild community cases are captured. It is most often diagnosed in school-aged children, although infants, adolescents and adults can be affected.
Many cases follow an upper respiratory infection, and seasonal clustering has been observed, but no single organism is necessary for diagnosis. The condition is not usually contagious; a preceding infection may act as an immune trigger rather than remain the cause of the vasculitic illness. Recurrence occurs in a meaningful minority, often within months, and a recurrent rash does not automatically imply progressive kidney disease.
Kidney involvement is the major determinant of long-term outcome. Reported nephritis frequency varies widely because studies use different thresholds for microscopic haematuria and proteinuria and follow children for different periods. Most clinically apparent renal findings emerge during early follow-up, but surveillance schedules are designed to capture delayed abnormalities. Severe long-term kidney impairment is uncommon overall yet clinically important.
No current national Indian incidence figure is asserted here. Hospital series overrepresent complicated disease, while outpatient series may miss children who never reach specialist care. For an individual child, the practical message is not a percentage: IgA vasculitis is usually self-limited, but urine and blood-pressure monitoring cannot be omitted merely because rash and joint pain have improved.
Risk Factors
A recent respiratory or gastrointestinal infection often precedes IgA vasculitis, and medicines, immunisation or other antigen exposure may occasionally be temporally associated. These observations do not establish an avoidable cause in an individual child. Families should not be told that a specific food, antibiotic or vaccine caused the illness without evidence. The pathogenesis reflects abnormal IgA-containing immune complexes and small-vessel inflammation in a susceptible host.
Risk factors for more significant renal involvement reported across cohorts include older age, persistent or recurrent purpura, severe gastrointestinal disease and renal abnormalities present at diagnosis. None is accurate enough to replace surveillance. A young child with mild rash can still develop haematuria or proteinuria, while many older children recover without chronic disease. Baseline blood pressure, urine dipstick and quantified protein when abnormal are more actionable than risk-factor counting.
The immediate complication profile depends on organ involvement. Marked colicky abdominal pain, gastrointestinal bleeding, vomiting or abdominal distension raises bowel oedema or intussusception. Scrotal involvement can mimic torsion. Reduced urine, oedema, hypertension, visible haematuria or heavy proteinuria indicates kidney disease. Rare pulmonary haemorrhage, central nervous system vasculitis or severe cardiac involvement causes high-acuity symptoms.
Children with pre-existing kidney disease, dehydration or medicines affecting renal perfusion require extra caution with analgesia. Social risk also matters: families living far from testing, unable to return weekly, or lacking a blood-pressure service need a feasible documented follow-up pathway. Loss to surveillance is a preventable risk. Ask about access and arrange monitoring rather than assuming it will happen elsewhere.
Diagnosis
IgA vasculitis is usually a clinical diagnosis. Purpura or petechiae with lower-limb predominance, not explained by thrombocytopenia, plus abdominal pain, arthritis or arthralgia, renal involvement, or IgA-predominant histology supports classification. Classification criteria assist consistency but do not replace judgement or exclude dangerous mimics.
History
Establish the sequence of rash, joint, abdominal and urinary symptoms. Ask about fever, preceding infection, new medicines, immunisation, recurrence, limb or scalp swelling, inability to walk, vomiting, melaena, haematochezia, severe colic, reduced intake, urine colour and volume, facial or leg oedema, headache and visual symptoms. Ask about scrotal pain, breathlessness, haemoptysis, seizure or focal neurology. Review prior renal disease, family kidney or autoimmune disease and access to follow-up.
Examination
Record temperature, heart rate, perfusion and blood pressure using a correctly sized cuff. Inspect the whole skin, including buttocks and pressure areas, and distinguish palpable non-blanching lesions from bruises or blanching rash. Assess hydration, oedema, joint swelling and mobility. Examine the abdomen for tenderness, guarding, distension or a mass and the testes when symptoms require urgent assessment. Look for pallor, hepatosplenomegaly, respiratory distress and neurological abnormality.
Investigations
Every child needs urinalysis and blood pressure. If urine is abnormal, quantify protein with a preferably first-morning protein-to-creatinine ratio, examine sediment and measure creatinine and estimated GFR. CBC helps exclude thrombocytopenia and anaemia; inflammatory markers, coagulation, complement, cultures or autoantibodies answer specific differentials. Ultrasound is indicated for suspected intussusception or scrotal pathology. Skin or kidney biopsy is reserved for atypical diagnosis or defined renal severity, not routine uncomplicated disease.
Differential Diagnosis
Meningococcaemia and other sepsis must be considered in a febrile, toxic child with a non-blanching rash. Do not label purpura as IgA vasculitis before assessing airway, circulation, mental state and infection risk. Immune thrombocytopenia, leukaemia, marrow failure and disseminated intravascular coagulation can cause petechiae or purpura; the platelet count and broader clinical picture distinguish them. Trauma, safeguarding concerns and pressure-related lesions require a careful distribution and history.
Other vasculitides and inflammatory disorders include systemic lupus erythematosus, ANCA-associated vasculitis, Kawasaki disease and polyarteritis nodosa. Low complement, persistent systemic inflammation, unusual organ disease, positive serology or an atypical rash should prompt reconsideration. Acute post-infectious glomerulonephritis and IgA nephropathy may cause haematuria and proteinuria without the classic systemic purpura pattern. Haemolytic uraemic syndrome combines anaemia, thrombocytopenia and acute kidney injury, often after diarrhoea.
Abdominal differentials include appendicitis, gastroenteritis, inflammatory bowel disease, pancreatitis, testicular torsion and surgical intussusception unrelated to vasculitis. IgA vasculitis can itself cause intussusception, so a known diagnosis does not make worsening colic benign. Joint pain may resemble septic arthritis, juvenile idiopathic arthritis, acute rheumatic fever or reactive arthritis.
Papular urticaria, erythema multiforme, Gianotti-Crosti syndrome and drug eruptions can resemble early skin disease but have different morphology and blanching. Atypical distribution, thrombocytopenia, persistent high fever, organomegaly, profound anaemia, low complement or failure to follow the expected course are signals to reopen the diagnosis rather than force every feature into IgA vasculitis.
Management
Begin by grading severity and confirming that follow-up is possible. A well child with typical rash, comfortable mobility, stable hydration, normal blood pressure and no significant urine abnormality can often be managed as an outpatient with written safety-netting. Encourage fluids and normal nutrition as tolerated. Rest may improve painful dependent purpura, but prolonged immobilisation is unnecessary once comfortable. Document a schedule for urine and blood-pressure checks rather than saying only to follow up.
Use paracetamol as a common first analgesic when suitable. An NSAID can help joint pain in selected children but should be avoided with gastrointestinal bleeding, significant abdominal disease, dehydration, hypertension, renal impairment or important proteinuria. Opioid-level pain, guarding or recurrent colic requires diagnostic reassessment, not escalating home analgesia.
Current national recommendations support considering a short corticosteroid course for selected severe abdominal pain or troublesome musculoskeletal disease after exclusion of surgical pathology and with clinician supervision. Steroids are not recommended simply to prevent future nephritis. Intussusception needs urgent imaging and paediatric surgical management. Testicular symptoms require torsion exclusion.
Renal management is risk-stratified. Persistent proteinuria, nephrotic-range proteinuria, reduced GFR, hypertension or nephritic and nephrotic syndromes need nephrology assessment, quantified urine protein and possible biopsy. Renin-angiotensin system blockade and immunosuppression belong to specialist protocols with monitoring. Hospitalise children with severe pain, bleeding, inability to maintain hydration, renal dysfunction, hypertension, neurological or pulmonary symptoms, or unreliable urgent follow-up. Reassess recurrence with the same organ screen rather than treating rash alone.
Prescribing Information
This educational section does not provide a regimen for an individual child. Before prescribing analgesia, confirm hydration, renal function where clinically indicated, urine findings, gastrointestinal bleeding risk, allergy, asthma sensitivity, concurrent medicines and the possibility of a surgical abdomen. Paracetamol products can be duplicated across fever and cold preparations; calculate a weight-appropriate dose from a verified paediatric reference and document maximum daily exposure.
NSAIDs reduce inflammatory joint pain but can worsen renal perfusion and gastrointestinal injury. Avoid them when creatinine is abnormal, urine protein is significant, the child is dehydrated, blood pressure is high, bleeding is present or abdominal disease is severe. Do not interpret temporary pain relief as evidence that intussusception or testicular torsion has been excluded.
Corticosteroids require a defined indication, baseline infection and blood-pressure assessment, a verified weight-based protocol and follow-up for response and adverse effects. Explain that symptom improvement does not remove the need for urine surveillance and that prophylactic steroids have not reliably prevented nephritis. Repeated or prolonged courses need specialist review because infection, hypertension, glucose disturbance, mood change, gastrointestinal symptoms and adrenal suppression become relevant.
ACE inhibitors, angiotensin-receptor blockers and immunosuppressants are nephrology-directed for defined renal disease. They require product-specific renal, potassium, blood-pressure, infection, vaccination, reproductive and interaction checks. Antibiotics do not treat IgA vasculitis unless a separate bacterial infection is diagnosed. Avoid empiric anticoagulants, herbal immune remedies and topical steroid treatment for systemic purpura. Every prescription should state its target symptom, review point and reasons for urgent reassessment.
When to Refer
Seek same-day paediatric assessment for severe or escalating abdominal pain, vomiting, gastrointestinal bleeding, abdominal distension, guarding, inability to drink, scrotal pain or swelling, significant oedema, visible haematuria, reduced urine, hypertension, severe headache or an atypical presentation. Surgical referral is urgent when intussusception, perforation, appendicitis or testicular torsion is possible. A known vasculitis diagnosis does not safely exclude these emergencies.
Refer to paediatric nephrology for persistent or increasing proteinuria, nephrotic-range protein loss, reduced estimated GFR, macroscopic haematuria with renal dysfunction, nephritic or nephrotic syndrome, hypertension, oedema or an abnormal renal course. IPNA recommendations use repeated first-morning urine protein-to-creatinine results, kidney function and clinical syndrome to guide biopsy and treatment. Include trends rather than a single dipstick.
Rheumatology or general paediatrics should review an atypical rash, prolonged systemic inflammation, recurrent severe disease, diagnostic uncertainty or important joint disease. Dermatology may help when biopsy is required to demonstrate IgA-predominant leukocytoclastic vasculitis. Neurology and intensive care are required for seizures, focal deficits, encephalopathy or severe hypertension. Respiratory compromise or haemoptysis needs emergency assessment for rare pulmonary haemorrhage.
A routine referral can also be necessary when local primary care cannot provide serial urine and blood-pressure surveillance. The referral should state symptom onset, rash distribution, abdominal and joint findings, blood pressure percentiles, urinalysis, quantified protein, creatinine, CBC, treatments and response. Give the family an urgent route back while waiting; referral paperwork is not a safety net.
Red Flags
A toxic child with fever and rapidly spreading non-blanching purpura has possible invasive infection and needs emergency sepsis assessment. Do not wait for the rash to become palpable or for urine testing. Altered consciousness, neck stiffness, poor perfusion, respiratory distress or rapidly progressive lesions are especially concerning. Thrombocytopenia, severe anaemia or organomegaly also points away from uncomplicated IgA vasculitis.
Abdominal red flags include severe colicky pain, bilious vomiting, blood in vomit or stool, distension, guarding, a palpable mass, shock or pain that wakes the child repeatedly. These may indicate intussusception, bowel ischaemia, bleeding or perforation. New scrotal pain or swelling requires immediate torsion exclusion. Steroids or analgesics must not delay imaging and surgical review.
Renal red flags are visible haematuria, oliguria, oedema, rapid weight gain, severe or symptomatic hypertension, heavy proteinuria, rising creatinine or a nephritic or nephrotic presentation. Headache, vomiting, visual change, seizure or confusion may reflect hypertensive emergency or central nervous system disease. Breathlessness, hypoxaemia or haemoptysis raises rare pulmonary haemorrhage.
After discharge, families should return urgently for reduced urine, facial swelling, severe headache, abdominal or scrotal pain, bleeding, breathing difficulty, drowsiness or seizure. A fading rash is not proof that renal risk has ended. Failure to attend planned monitoring should trigger active contact when possible. Recurrent purpura should prompt repeat blood pressure and urinalysis, not automatic reuse of an old steroid or analgesic prescription.
Indian Clinical Context
The Indian Academy of Pediatrics currently lists Henoch-Schönlein purpura as Standard Treatment Guideline 32 in its 2022 treatment-guideline series. The listing confirms professional recognition and local relevance, but the public index does not establish a national MoHFW surveillance schedule or guarantee that every linked document is updated continuously. This draft therefore anchors detailed renal recommendations to the newer 2024 IPNA and 2025 national evidence-based publications and labels them as international comparators.
Indian care settings differ greatly in access to paediatric nephrology, first-morning urine protein measurement, ambulatory blood-pressure monitoring, biopsy and paediatric surgery. A safe plan must use the tests and referral network actually available. At minimum, blood pressure and urine dipstick should be arranged at presentation and through the local follow-up pathway. An abnormal dipstick should lead to quantified protein and kidney-function assessment where possible, not repeated reassurance without escalation.
Travel distance, school attendance, wages lost for visits and the price of laboratory testing affect adherence. Give dates, not vague instructions, consolidate tests when safe and identify the nearest service for abdominal or renal emergencies. Explain the condition in the family's preferred language and avoid attributing it to food, cold weather or a caregiver's action.
This guide does not assert a national Indian incidence or recommend a locally unverified drug brand. Institutional protocols should reconcile specialist thresholds with current resources. When a local pathway conflicts with newer kidney guidance, document the difference and seek paediatric nephrology advice rather than blending incompatible schedules silently.
NMC Competency Mapping
The NMC CBME Curriculum 2024 maps this disorder directly to Paediatrics Topic 21. Competency PE21.2 requires the learner to describe the etiopathogenesis, diagnosis and management of Henoch-Schönlein purpura. It is a knowledge-domain competency at Know level, not listed as core and not requiring certification. That formal level should not be mistaken for permission to omit clinical safety skills during paediatric teaching.
At Know level, students should explain IgA-containing immune-complex small-vessel inflammation; recognise palpable purpura with lower-limb predominance; connect abdominal, joint, skin and renal manifestations; and distinguish non-thrombocytopenic purpura from sepsis, ITP and haematological disease. They should state that urinalysis and blood pressure are required even when the rash is typical and the child feels well.
Clinical teaching should extend the competency through supervised application: assess hydration and perfusion, measure paediatric blood pressure correctly, inspect rash distribution, examine joints and abdomen, recognise intussusception and scrotal emergencies, interpret urinalysis and quantify protein when abnormal. Learners should communicate a follow-up and red-flag plan without falsely promising that steroids prevent nephritis.
Integration includes immunology, pathology, nephrology, rheumatology, dermatology, radiology, paediatric surgery, pharmacology and communication. Assessment can use a vignette in which abdominal symptoms precede rash or renal abnormalities appear after symptom resolution. Formal mapping should preserve PE21.2 exactly and can cross-reference PE20.3, PE20.4 and PE20.8 for proteinuria, haematuria and urine examination skills.
Key Exam Pearls for NEET PG
IgA vasculitis is the preferred name; Henoch-Schönlein purpura is the older eponym. The defining rash is palpable purpura or petechiae, usually dependent and lower-limb predominant, not caused by thrombocytopenia. The classic associated domains are joints, gastrointestinal tract and kidneys. Rash may follow abdominal pain, so early disease can mimic a surgical abdomen.
The practical baseline pair is urinalysis plus blood pressure. CBC helps show a normal platelet count and identify anaemia. Serum IgA may be elevated but is neither required nor diagnostic. Skin biopsy shows leukocytoclastic vasculitis with IgA-predominant deposition when morphology is atypical. Kidney histology resembles IgA nephropathy and is reserved for defined renal severity.
Intussusception is a major abdominal complication and may be small-bowel rather than the usual idiopathic ileocolic pattern. Severe colic, vomiting, bleeding or a mass needs urgent ultrasound and surgical review. Scrotal involvement mimics torsion, which must be excluded. Renal severity, particularly persistent proteinuria, hypertension and reduced GFR, drives long-term prognosis.
Treatment is supportive in uncomplicated disease. NSAIDs are avoided with renal impairment or gastrointestinal bleeding. Corticosteroids may help selected severe abdominal or joint symptoms but do not reliably prevent nephritis. Never allow symptom resolution to cancel renal monitoring. For NMC, retain PE21.2 and integrate PE20.3, PE20.4 and PE20.8. In an exam answer, always include the monitoring plan and the red flags for sepsis, intussusception and nephritis.
Frequently Asked Questions
Is Henoch-Schönlein purpura the same as IgA vasculitis?
Yes. IgA vasculitis is the preferred descriptive name for the condition formerly called Henoch-Schönlein purpura. It is a small-vessel vasculitis associated with IgA deposition and commonly affects skin, joints, bowel and kidneys. The eponym remains common in examinations and older guidance, so learners should recognise both names.
Why are urine and blood pressure checked after the rash improves?
Kidney involvement may appear after the skin and joint symptoms settle and can initially cause no symptoms. Repeated urinalysis and blood-pressure checks detect haematuria, proteinuria or hypertension early. A fading rash therefore does not end follow-up, and an abnormal result should be quantified and reviewed rather than simply repeated indefinitely.
Do corticosteroids prevent kidney disease in IgA vasculitis?
Routine prophylactic corticosteroids have not reliably prevented nephritis and are not prescribed solely for that purpose. A clinician may consider them for selected severe abdominal or musculoskeletal symptoms after excluding surgical disease. Established renal involvement is treated according to severity by paediatric nephrology, sometimes using biopsy-directed protocols.
Which symptoms require emergency reassessment in a child with IgA vasculitis?
Emergency review is needed for toxic appearance, rapidly spreading purpura, severe or colicky abdominal pain, bilious vomiting, gastrointestinal bleeding, guarding, scrotal pain, reduced urine, swelling, visible haematuria, severe headache, breathing difficulty, haemoptysis, drowsiness or seizure. These can indicate sepsis, intussusception, torsion, significant nephritis or rare organ complications.
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