Horrible Spots and Pain
aka Pediatric Perplexity 014
A 4 year-old boy is brought to the emergency department by his parents with a history of increasing numbers of red spots on his legs over the past 6 days. They took him to two different family doctors and have tried various creams. The spots have spread to his buttocks and his arms, and now his legs are sore and look swollen. He has also had abdominal pains.
On examination he looks well with age-appropriate vital signs, but he is reluctant to move his lower limbs.
A urine dipstick shows 2+ RBCs.
His rash looks like this:
Questions
Q1. What is the likely diagnosis?
Answer and interpretation
Henoch-Schönlein Purpura (HSP)
HSP is an autoimmune, self-limiting, immunoglobulin A-mediated, small-vessel vasculitis. It typically affects children aged 2-8 years and is the most common vasculitis affecting children. It is often preceded by upper respiratory tract symptoms (occuring 1-3 weeks earlier).
The diagnosis is likely in the presence of the triad of:
- purpuric rash on the limbs (mainly lower) and buttocks (especially the dependent surfaces)
- joint pain/ swelling
- abdominal pain
HSP can occur in adults (sometimes associated with drugs or malignancy) and there is also an infantile form.
Although named for Eduard Heinrich Henoch and Johann Lukas Schönlein, HSP was actually first described by the brilliant English physician William Heberden in 1801.
Q2. What investigations are required?
Answer and interpretation
Check UEC
Renal function should be assessed if there is evidence of hematuria (90% of cases) or hypertension. About half of cases will develop nephritis, but only 1% will have longterm renal failure.
Other investigations may not be required. Sometimes the cause of purpura may be uncertain. In this case the patient should be investigated as discussed in Pediatric Perplexity 007 (e.g. FBC, blood culture). In HSP, there is no thrombocytopenia or coagulopathy.
Skin and renal biopsies are not usually necessary.
Q3. What is the usual time course of the illness?
Answer and interpretation
Typical time course:
- upper respiratory tract infection may precede the onset of symptoms by 1-3 weeks.
- joint pain usually lasts <48h
- abdominal pain usually lasts <72h
- resolution of rash by 4-6 weeks
Q4. What are the possible complications of this condition?
Answer and interpretation
- Renal – Haematuria (persistent or recurrent in 5%), proteinuria, nephrotic syndrome, isolated hypertension, renal insufficiency and renal failure (<1%). May not be present acutely, but become apparent during the convalescent period.
- Gastrointestinal – intussusception, bloody stools, haematemesis, spontaneous bowel perforation, and pancreatitis.
- Subcutaneous oedema – particularly affects the scrotum, hands, feet, and sacrum can be very painful and may present as an acute scrotum in boys
- Rare CNS and pulmonary complications can also occur.
- Recurrence – About half of cases of HSP have a recurrence in the first year.
Q5. What is the appropriate management, disposition and follow up?
Answer and interpretation
Management
- analgesia — usually oral
- consider prednisolone (see Q5)
- education and explanation
- seek and treat complications
Disposition
- most cases can be managed as an outpatient
- consider a surgical consult if gastrointestinal complications or an acute scrotum is suspected
- admission is required if:
- severe pain, e.g. unable to walk— requires bed rest and analgesia
- complications e.g. renal or abdominal
- social reasons
Follow up
- follow up by GP and general pediatrics outpatients for at least 6 months.
- some experts advise yearly urinalysis and blood pressure monitoring for life.
Q6. Is there a role for prednisolone in the management of this condition?
Answer and interpretation
The role of prednisolone is controversial.
Prednisolone may provide symptomatic relief of pain, but pain is usually relatively short-lived and corticosteroids could mask the onset of severe abdominal complications. Prednisolone may also reduce the risk of progression to end stage renal disease, but this was not confirmed in a recent systematic review of the limited trials that exist. Overall, prednisolone has not been shown to alter the natural history of HSP.
Most experts recommend its use if complications are present:
- severe abdominal complications (eg, intussusception or massive gastrointestinal bleed)
- significant renal disease (eg, rapidly progressive glomerulonephritis, severe nephrotic syndrome, renal insufficiency, or renal failure)
- severe orchitis, pulmonary hemorrhage, and severe CNS symptoms
A typical regimen for the treatment of HSP is:
- prednisolone 1 mg/kg daily for 2 weeks
In severe cases IV immunoglobulin and other immunosuppressants have been used, although their effectiveness is unknown.
References
- Arias-Santiago S, Aneiros-Fernández J, Girón-Prieto MS, Fernández-Pugnaire MA, Naranjo-Sintes R. Palpable purpura. Cleve Clin J Med. 2010 Mar;77(3):205-6. PMID: 20200171.
- Bogdanović R. Henoch-Schönlein purpura nephritis in children: risk factors, prevention and treatment. Acta Paediatr. 2009 Dec;98(12):1882-9. Epub 2009 Jul 24. Review. PMID: 19650836.
- Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP).Cochrane Database Syst Rev. 2009 Jul 8;(3):CD005128. Review. PMID: 19588365.
- Goel SS, Langford CA. A 72-year-old man with a purpuric rash. Cleve Clin J Med. 2009 Jun;76(6):353-60. PMID: 19487556.
- Reamy BV, Williams PM, Lindsay TJ. Henoch-Schönlein purpura. Am Fam Physician. 2009 Oct 1;80(7):697-704. PMID: 19817340. [NB. This review predates the Cochrane review indicating that prednisolone does not alter the progression of renal disease]
- Sohagia AB, Gunturu SG, Tong TR, Hertan HI. Henoch-schonlein purpura – a case report and review of the literature. Gastroenterol Res Pract. 2010;2010:597648. Epub 2010 May 23. PMC2874920.
CLINICAL CASES
Paediatric Perplexity
Chris is an Intensivist and ECMO specialist at the Alfred ICU in Melbourne. He is also a Clinical Adjunct Associate Professor at Monash University. He is a co-founder of the Australia and New Zealand Clinician Educator Network (ANZCEN) and is the Lead for the ANZCEN Clinician Educator Incubator programme. He is on the Board of Directors for the Intensive Care Foundation and is a First Part Examiner for the College of Intensive Care Medicine. He is an internationally recognised Clinician Educator with a passion for helping clinicians learn and for improving the clinical performance of individuals and collectives.
After finishing his medical degree at the University of Auckland, he continued post-graduate training in New Zealand as well as Australia’s Northern Territory, Perth and Melbourne. He has completed fellowship training in both intensive care medicine and emergency medicine, as well as post-graduate training in biochemistry, clinical toxicology, clinical epidemiology, and health professional education.
He is actively involved in in using translational simulation to improve patient care and the design of processes and systems at Alfred Health. He coordinates the Alfred ICU’s education and simulation programmes and runs the unit’s education website, INTENSIVE. He created the ‘Critically Ill Airway’ course and teaches on numerous courses around the world. He is one of the founders of the FOAM movement (Free Open-Access Medical education) and is co-creator of litfl.com, the RAGE podcast, the Resuscitology course, and the SMACC conference.
His one great achievement is being the father of three amazing children.
On Twitter, he is @precordialthump.
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