Post-streptococcal small-vessel vasculitis in a 16-month-old with associated intussusception and hypertension: Henoch Schönlein purpura?
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Published on: January 27, 2015
Sara Banbury1, David M Milgraum2, Daniel J Lewis3
1Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Insights
Henoch Schönlein purpura (HSP), or IgA vasculitis, usually affects older children. This case highlights HSP in a younger child, emphasizing the need for early diagnosis in all pediatric age groups.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Nephrology
Background:
- Henoch Schönlein purpura (HSP), also known as IgA vasculitis, is a common childhood vasculitis.
- It typically presents in children aged 3-15 years, with a peak incidence between 4-6 years.
- Classic symptoms include palpable purpura, joint pain, abdominal pain, and kidney involvement.
Observation:
- This report details a 16-month-old patient with clinical manifestations suggestive of HSP.
- The patient presented with symptoms consistent with HSP but had negative IgA immunofluorescence.
- This presentation is notably younger than the typical demographic for HSP.
Findings:
- The case demonstrates that IgA vasculitis can occur in infants younger than typically reported.
- Negative IgA immunofluorescence does not exclude a diagnosis of HSP in the appropriate clinical context.
- The constellation of purpura, arthralgia, abdominal pain, and renal involvement remains key for diagnosis.
Implications:
- Early recognition of vasculitis in young children is crucial for timely intervention.
- Prompt diagnosis and management can prevent long-term complications, especially renal sequelae.
- This case broadens the age spectrum considered for Henoch Schönlein purpura diagnosis.
Abstract:
Henoch Schönlein purpura (HSP), also known as IgA vasculitis, is a systemic small-vessel vasculitis typically occurring in children 3-15 years of age, with peak incidence at 4-6 years. It is characterized by a constellation of symptoms including palpable purpura, arthralgias or arthritis, abdominal pain including intussusception, and renal involvement. We report a patient with these clinical findings whose IgA immunofluorescence was negative but with a presumptive diagnosis of HSP at 16 months of age, significantly younger than the classic population. This condition rarely affects this age group, and we highlight the importance of considering vasculitis in children of all ages, as a failure to diagnose could lead to insufficient long-term monitoring, particularly regarding renal function.
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