Makindo Medical Notes

In adults, Henoch-Schonlein Purpura may present more insidiously with potential myocardial involvement.

About

• Henoch-Schonlein Purpura (HSP) is a systemic small vessel leukocytoclastic vasculitis, often presenting with a palpable purpuric rash due to bleeding into the dermis.
• This condition affects the skin, joints, kidneys, and gastrointestinal (GI) tract, primarily involving small blood vessels.

Aetiology

• IgA immune complex deposition in small blood vessels, particularly venules, capillaries, and arterioles.
• Often associated with preceding infections such as Group A Streptococci and Mycoplasma.

Clinical Presentation

• Typical purpuric rash primarily on lower limbs and buttocks, sparing the trunk and face.
• Most commonly seen in children but can occur at any age.
• May present with abdominal pain, PR bleeding, or complications like intussusception in severe cases.

Investigations

• Blood Tests: FBC, U&E, and clotting profiles, particularly if non-classical purpura or if the patient has systemic symptoms or renal involvement.
• Urinalysis: Check for haematuria and proteinuria; significant proteinuria is marked as 2+ or higher.
• Throat Swab & ASOT: Check for possible infectious triggers like Group A Streptococcus.

Renal Disease Findings

• Normal complement C3 and C4 levels, distinguishing it from post-streptococcal glomerulonephritis.
• Skin Biopsy: Immunofluorescence may reveal leukocytoclastic vasculitis with IgA and C3 deposits.
• Renal Biopsy: In cases of significant renal involvement, biopsy typically shows a focal segmental proliferative glomerulonephritis with mesangial IgA deposition, similar to IgA nephropathy.

Management

• Generally, HSP has a good prognosis, with most cases resolving conservatively with supportive care.
• In mild, classical cases, only urinalysis may be needed, with escalation in management if symptoms worsen or with significant joint involvement, malaise, proteinuria, or haematuria.
• For ongoing renal monitoring, patients or parents can perform daily urine dipsticks for several weeks. Escalation is needed if signs of renal disease appear.
• In rare cases leading to rapidly progressive glomerulonephritis, steroids or immunosuppressants may be indicated. Approximately 1-5% may progress to end-stage renal failure.