Henoch-Schonlein Purpura
Epidemiology and
Etiology:
·
3-8 years most commonly
·
Strong background of atopy in 1/3rd
·
Post-URTI often
Common sites:
·
Skin
·
Extensor surfaces of arms and legs
·
buttocks
·
Kidneys (1/3rd)
·
GI tract
Gross features:
·
Purpuric skin lesions
Histologic
features:
·
Kidney:
·
Varying severity:
·
mild focal mesangial proliferation
·
diffuse mesangial proliferation
·
crescentic glomerulonephritis
·
vasculitis is rare
·
skin
·
subepidermal hemorrhages
·
necrotizing vasculitis involving small
vessels of the dermis
·
GI:
·
Vasculitis maybe
Immunophenotype:
Marker: |
Sensitivity: |
Specificity: |
IgA (on frozen tissue) |
|
|
IgG (on frozen
tissue) |
Some |
|
C3 (on frozen
tissue) |
Some |
|
·
IgA deposited in the
glomerular mesangium
(similar to IgA nephropathy)
·
IgA deposited in
small vessels of dermis
Molecular features:
·
Other features:
·
Abdominal pain, vomiting, intestinal bleeding
·
Nonmigratory arthralgias
·
Hematuria, proteinuria, nephritic syndrome
·
Rapidly progressive glomerulonephritis
in a minority
References:
·
Kumar V, Fausto N, Abbas
A. Robbins & Cotran Pathologic Basis of Disease,
Seventh Edition. 7th ed. Saunders; 2004:1552.