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. His rash looks like this:

HSP

Photo by okwikikim – click image for source

A urine dipstick shows 2+ RBCs.

Questions

Q1. What is the likely diagnosis?

Henoch-Schonlein Purpura

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

The rash may not appear until after the onset of the other symptoms. It starts as erythematous papules quickly followed by palpable purpura, which are visible, unblanching hemorrhages in the skin and mucous membranes, up to 10 mm in diameter. The rash may also resemble urticaria.

Although named for Eduard Heinrich Henoch and Johann Lukas Schönlein, HSP was actually first described by the brilliant English physician William Heberden in 1802.

HSP can occur in adults (sometimes associated with drugs or malignancy) and there is also an infantile form.

Q2. What investigations are required?

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?

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?

  • 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?

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?

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]
  • Royal Children’s Hospital Melbourne. RCH Clinical Practice Guidelines.
  • 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. PMID: 20508739;  PMCID: PMC2874920.

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