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.