1. A Painful, Purpuric Rash
Lydia Burland
ST1 Paediatrics

2. Introduction Brief case study Common precipitants and presentation
Initial investigation and management
Indications for referral and follow up
Key facts for future practice

3. Case Study: Presentation
16 year old boy
Presented with a painful rash on both legs
started less than 12 hours ago
gradually spreading
becoming increasingly painful
now unable to weight bear

4. Case Study: Background
HPC:
1st presented 2 days ago with sore throat & fever
diagnosed with viral URTI
discharged with analgesia
PMH: normally fit and well
DH: no allergies or regular medications

5. Case Study: Examination
Obs stable and apyrexial
Bilateral, non-blanching purpuric lesions
Multiple blisters and bullae
Up to the knees
Extremely tender

7. Case Study: Investigations
WCC 15.5 (8.4)
Streptolysin O antibody 1310 (range <200)
ANA/ANCA/RhF negative
Throat swab negative
Urine dipstick negative
On presentation his WCC was raised, and had gone up since his prior admission 2 days earlier.
He also had a high levels of streptolysin antibodies (the normal range being less than 200 in children and 400 in adults) which indicate past or present infection. Levels rise after 1-3 weeks of strep infection, peak at weeks 3-5 and fall back within normal range at around 6 months.
All his other bloods were unremarkable and his throat swab and urine dip were negative.

8. Case Study: Initial Management
Henoch-Schönlein Purpura
5/7 Penicillin V
Regular paracetamol and NSAIDs
Topical betnovate and hydromol cream
Follow up in dermatology clinic in 10 days

9. Case Study: Further Progress
Re-admitted 8 days later with;
- Worsening, painful rash
- Arthralgia
- Abdominal pain
Admitted under rheumatology
Treated with IM depomedrone and reducing course of high dose prednisolone
Rheumatology follow up in 1 week
He was re-admitted under rheumatology 8 days later with worsening rash, which had spread to his upper limbs, arthralgia in his fingers, wrists, elbows, ankles and left knee and left-sided abdominal pain.
He was treated with IM and high dose oral steroids and oral morphine before discharge with follow up in one week.

10. Henoch-Schönlein Purpura
IgA mediated autoimmune vasculitis
Predominantly affects children aged 3-10 yrs
20x less common in adults
Often more severe disease in teenage/adult patients
Henoch-Schonlein Purpura is an IgA mediated autoimmune vasculitis common in children aged 3 to 10 years. Females and Caucasians are at a very slightly increased risk.
Though HSP is 20x less common in adults, it does present to adult services, especially in adolescent patients, and therefore it is important as acute adult physicians we are able to recognise the ways in which it presents, and what management needs to be initiated. Especially important is knowing which cases need further referral and follow up.

11. Henoch-Schönlein Purpura
Recognised precipitants for HSP include;
Infections, typically group A streptococcus, mycoplasma and Epstein-Barr virus
Vaccinations
Environmental exposure to certain allergens, pesticides and insect bites
50% have preceding URTI

12. Henoch-Schönlein Purpura
Mild illness with fever
Symmetrical erythematous macular rash
Back of legs
Buttocks
Ulnar surface of arms
Macules evolve into typical purpuric lesions
These may coalesce, resembling bruises
The majority of HSP cases present in the winter months, with 50% cases following an upper respiratory tract infection.
Most patients present with mild illness and fever, and a symmetrical macular rash on the back of the legs, buttocks and ulnar surface of the arms.
Over the course of 24 hours these macules then evolve into the typical purpuric lesions associated with HSP. They may coalesce to resemble extensive bruising.
In adolescents these may then progress to bullae, which are much rarer in children.

13. Henoch-Schönlein Purpura
50% of cases have renal involvement
10% serious
1% progress to ESRF
Rarer complications include;
Intussusception GI bleeding
MI Seizures
Bowel infarction Mononeuropathies
The main complication of HSP is renal involvement which occurs in 50% of cases.
It is more common in adolescents and adults than younger children, and is associated with increased severity with increasing age.
Renal involvement should be monitored with BP and urinalysis, initially at 7 and 14 days after presentation and then monthly until 12 months.
Of the other less common complications it is important to know that intussusception is more likely in adolescent age groups and therefore abdo USS is indicated in patients with abdominal pain.

14. Henoch-Schönlein Purpura
Usually self-limiting
Supportive treatment with analgesia
Corticosteroids if arthralgia and GI symptoms
Follow up if arthralgia or renal involvement
Excellent long-term prognosis
1/3 of patients experience recurrence
HSP is a self-limiting illness with no treatments proven to shorten the duration or reduce the incidence of complications, therefore treatment is usually supportive, with analgesia.
NSAIDs can be especially effective but need to be used with caution if there is evidence of renal insufficiency.
Corticosteroids may be useful for arthralgias and gastrointestinal dysfunction.
There is no consistent evidence for use of immunosuppressive treatments, though azathioprine and cyclophosphamide may be of some benefit.
Patients need rheumatology and/or renal follow up if they have arthralgia or renal involvement. Otherwise they can be followed up by their GP with repeat BP measurement and urine dips at 7 and 14 days, and 1, 3, 6 and 12 months.
The long-term prognosis is excellent, with the majority of cases resolving spontaneously without treatment.
Initial attacks may last up to several months, and one third of patients experience recurrence at some point.

15. Key Facts HSP follows a viral illness in >50% of cases
Adults are at higher risk of renal involvement and more severe renal disease
Treatment is supportive with analgesia
Steroids may help for arthralgia or GI symptoms
Rheumatology F/U if arthralgia or renal involvement
Urinalysis for 12 months after the episode

16. Thanks
Lydia Burland