QuestionSBA
QuestionChoice
ITP is a disorder resulting in an isolated low platelet count and a tendency to bruise/bleed. A purpuric rash may be present but it is not typically palpable. Children may develop ITP post virally and recovery often occurs spontaneously. The additional clinical features here of joint pain and dipstick positive urine suggest an alternative diagnosis.
53333
b
Immune thrombocytopenic purpura (ITP)
QuestionChoice
Henoch-Schonlein Purpura is a small vessel vasculitis characterised by IgA deposition. It is associated with nephrotic and nephritic syndromes, as well as a rapidly progressive glomerulonephritis, due to glomerular IgA deposition. It presents with a triad of joint pain, abdominal pain, and palpable purpura.

A typical history involves a recent viral upper respiratory tract infection in a child, followed by the development of the characteristic clinical features. HSP is usually managed supportively as it often resolves spontaneously. Severe renal involvement (for example rapidly progressive, crescentic glomerulonephritis, or persistent nephrotic syndrome), or intractable symptoms not responding to analgesia may necessitate management with steroids.
53332
a
Henoch-Schonlein Purpura
QuestionChoice
Microscopic polyangiitis is a small vessel vasculitis associated with p-ANCA. It is a systemic condition that can affect any organ including the lungs, kidney, skin and nervous system, and may present with a palpable purpuric rash. However, the clinical features here are that of HSP, with a classical triad of abdominal pain, joint pain, and purpura, in a child who has recently recovered from a viral infection.
53336
e
Microscopic polyangiitis
QuestionChoice
This is incorrect because meningitis classically presents with a fever, headache, photophobia and neck stiffness. It can be difficult to elucidate these symptoms in children so it is important to distinguish between a vasculitic rash compared to a non-blanching purpuric rash in meningitis. There is increased awareness and education amongst parents about how to use the glass test to determine if their child's rash is non-blanching.
53335
d
Meningitis
QuestionChoice
This is incorrect because ALL presents with symptoms of anaemia, infection and bleeding. This is secondary to bone marrow failure. It can also present with infiltrative symptoms such as bony pain. It is very important to elucidate any red flag symptoms which point towards cancer such as weight loss or night sweats. The question stem points towards HSP due to the triad of proteinuria, joint pain and a palpable, purpuric rash.
53334
c
Acute lymphoblastic leukaemia (ALL)
QuestionComment
Triad: HSP

Hinge pain (arthralgias)
Stomach pain (abdo pain associated w/ intussusception)
Palpable purpura on buttocks / legs
57458
User
subscriber
Goldfish
Concept
Chapter
# Summary
 

Henoch-Schonlein Purpura (HSP) is the most common type of small vessel vasculitis in children, particularly in those aged 3-5 years. It is often preceded by a viral upper respiratory tract infection and presents with purpura or petechiae, abdominal pain, arthralgia, and nephritis. Investigations primarily involve physical examination and urine tests. Management strategies involve NSAIDs for analgesia and inflammation control, potential use of antihypertensives for blood pressure management, and long-term renal function monitoring post-episode.
 

# Definition
 

Henoch-Schonlein Purpura (HSP), also known as IgA vasculitis, is an immune-mediated small vessel vasculitis. It predominantly affects children, especially those aged 3-5 years, and is characterised by palpable purpura, arthritis or arthralgia, abdominal pain, and renal disease.
 

# Epidemiology
 

HSP is the most common vasculitis in children. Its peak incidence occurs in the age group of 3-5 years, affecting 10-20 per 100,000 children annually in the UK. The disease is slightly more common in males than females. Many cases occur in the fall and winter months, often following an upper respiratory tract infection.
 

# Aetiology

HSP is the result of IgA immune complex deposition in small blood vessels of the skin, kidneys, joints and gastrointestinal tract.
 
While the exact aetiology of HSP is unknown, it is thought to be immune-mediated. Often, the onset of HSP is preceded by an upper respiratory tract infection, suggesting a potential infectious trigger. Other proposed triggers include medications, vaccinations, and insect bites.
 

# Signs and Symptoms
 

The primary presentation of HSP includes:
 

 -  Purpura or petechiae, primarily located on the buttocks and lower limbs
 -  Diffuse abdominal pain
 -  Arthralgia or arthritis, predominantly in the knees and ankles
 -  Renal involvement: Nephritis (haematuria and/or proteinuria)
 -  Patients may present with a fever
 -  Scrotal pain
 -  Often, a history of a recent upper respiratory tract infection
 

# Differential Diagnosis
 

Differential diagnoses for HSP based on its signs and symptoms include:
 

 -  **Leukocytoclastic vasculitis**: Presents with palpable purpura, but typically lacks the systemic features of HSP
 -  **Idiopathic thrombocytopenic purpura (ITP)**: Presents with petechiae and purpura, but lacks abdominal pain, arthralgia, and renal involvement
 -  **Meningococcaemia**: Presents with purpuric rash and fever but also includes symptoms like rapid disease progression, severe illness, and potential neurological symptoms.
 

# Investigations
 

The diagnosis of HSP is primarily clinical, based on the characteristic presentation and the EULAR criteria:

- Palpable purpura **AND** at least one of the following:

    - Diffuse abdominal pain
    - Arthritis or arthralgia 
    - Renal involvement
            - Haematuria +/- proteinuria 
    - Biopsy showing IgA deposition 

However, investigations can be used to monitor disease progression and complications:
 

 -  Urinalysis: To monitor for nephritis (haematuria and proteinuria)
 -  Blood pressure: To assess for hypertension secondary to renal involvement
 -  Bloods may include U&Es, ESR and creatinine
 -  In severe cases, a kidney biopsy may be necessary
 

# Management
 

The management of HSP involves symptom control and monitoring for complications:
 

 -  Analgesia and anti-inflammatory effects are typically achieved with paracetamol 
       -  Avoid Ibuprofen if significant proteinuria or hypertension
       -  Prednisolone is not routinely used. It may be used in cases of severe or persistent abdominal or joint pain. 
 -  Antihypertensives may be needed to control blood pressure in cases of significant renal involvement
 -  Following an episode of HSP, regular urine tests and blood pressure monitoring should be conducted for 12 months to monitor for potential renal impairment.

HSP is not communicable, so children can return to school when they feel well enough.
 
#  Complications
 
 - Oliguria 
 - Hypertension 
 - End-stage renal disease may occur in a small number of patients 
 - Myocardial infarction
 - Intussusception 
 - Seizures 
 

# Prognosis
 

 -  The majority of HSP cases recover completely within 4 weeks.
 -  A recurrence of HSP is seen in about 50% of patients. 
 -  Long-term renal impairment occurs in approximately 20% of patients with significant proteinuria.


# References

[NHS Guidelines on Henoch ](https://www.nhs.uk/conditions/henoch-schonlein-purpura-hsp/)

[Information from Great Ormond Street Hospital](https://www.gosh.nhs.uk/conditions-and-treatments/conditions-we-treat/henoch-sch-nlein-purpura-hsp)
 
[East and North Hertfordshire NHS Trust Guidance](https://www.enherts-tr.nhs.uk/wp-content/uploads/2022/10/Management-of-Henoch-Schonlein-purpura-in-Children-and-Young-People-under-16-years.pdf)
 
[Patient Info Henoch Schonlein Purpura](https://patient.info/doctor/henoch-schonlein-purpura-pro)

[EULAR Criteria for Henoch Schonlein Purpura](https://pubmed.ncbi.nlm.nih.gov/20413568/)
452
454
Henoch Schonlein Purpura
Topic
29
Paediatrics
Condition
310
Henoch-Schonlein purpura
UkmlaTopic
5
Child health
10727
Henoch-Schonlein Purpura is a small vessel vasculitis in children, presenting with a triad of joint pain, abdominal pain, and palpable purpura.
A 9-year-old girl presents to A&E with her mother with a 1 day history of a rash on her lower limbs. She has been recovering from a cold recently. She denies previous similar episodes. She limps whilst walking around the room and is gripping her abdomen in pain. On examination, there are no findings on auscultation of her chest. Her knee is tender but not visibly swollen. Her rash is palpable and purpuric bilaterally on her lower limbs. A urine dipstick reveals protein +++ and ++ haematuria.

Which of the following is the most likely diagnosis?
a