HENOCH-SCHNLEIN PURPURA

Morning Report
July 6, 2007
Sima Patel, MD

DEFINITION
Also called anaphylactoid purpura
HSP is a systemic vasculitic syndrome
with:

Palpable purpura
Arthralgias
GI involvement
Glomerulonephritis

BACKGROUND
First described in 1801 by William
Heberden, a physician in London, who
wrote about a case of a 5 year old boy
with hematuria, abdominal pain, joint
pains and a skin rash.
In 1837, Johann Schnlein and later in
1874, Edouard Henoch described multiple
case reports of similar cases. They also
showed an association of an upper
respiratory infection preceding
development of symptoms.

EPIDEMIOLOGY

90% of cases reported in children

Peak in children aged 4-7

Male:Female (1.5:1)
50% follow a URI
Renal disease is more severe in adults

PATHOGENESIS
Likely mechanism thought to be an
immune-complex mediated disease with
deposits in the glomerular capillaries,
dermal capillaries and GI tract.
Mesangial deposits of IgA are the same as
those seen in IgA nephropathy

PRECIPITATING ANTIGENS

INFECTIONS

URI
Measles
Rubella
Parvovirus B19
Mycoplasma
Coxsackie virus
Toxocara
Amebiasis
Salmonella

C.difficile
H.pylori
Adenovirus
Legionella
Tuberculosis
Mumps
Streptococcus
Morganella morganii

PRECIPITATING ANTIGENS

Drugs
Vancomycin
Streptokinase
Ranitidine
Cefuroxime
Diclofenac
Enalapril
Captopril

PRECIPITATING ANTIGENS

Other:
Food hypersensitivity
Cold exposure
Autosomal recessive Chronic granulomatous
disease
Myelodysplastic syndrome
Small cell lung cancer
Breast cancer

PATHOLOGIC FEATURES

DERMATOLOGIC FINDINGS:
Leukocytoclastic vasculitis with IgA
deposition

Direct Immunofluorescence of skin biopsy. Granular IgA and C3 staining of cutaneous

vasculature.
http://www.medscape.com/viewarticle/459714

H & E stain of skin biopsy showing leukocytoclastic vasculitis with infiltration of

neutrophils.
http://www.medscape.com/viewarticle/459714

Skin biopsy: Leukocytoclastic vasculitis with mononuclear and

polymorphonuclear cell infiltrates in the perivascular space
www.kjronline.org/abstract/view_articletext.asp?year=2004&page=178

PATHOLOGIC FEATURES

RENAL FINDINGS: Granular deposits of

IgA, mesangioproliferative
glomerulonephritis and crescent formation

Renal biopsy: sclerosis and fibrous crescents in the glomerulus.

http://www.ndt-educational.org/nagycase.asp

Immunofluorescence: Glomerular deposits of IgA

http://www.ndt-educational.org/nagycase.asp

CLINICAL FEATURES

Tetrad of symptoms
Abdominal pain
Renal disease
Palpable purpura
Arthritis/arthralgias more common in adults
and most common in knees and ankles.
Generally self-limiting

CLINICAL FEATURES

PALPABLE PURPURA: most commonly

seen on lower extremities and buttocks,
however can also been seen on the trunk
and arms.
Lesions begin as erythematous macules and
progress to purpuric, non-blanching,
nonpruritic lesions that may become confluent

CLINICAL FINDINGS

GI INVOLVEMENT: more common in

children. Symptoms include abdominal
pain, nausea, vomiting, diarrhea,
constipation or bowel intussusception.
May present with GI bleeding.

CLINICAL FEATURES

RENAL INVOLVEMENT:
in up to 50% of patients
Usually more rapidly progressive in adults.
Rare in children
May present with hematuria
Can have mild glomerulonephritis leading to
microscopic hematuria and can lead to a
rapidly progressive glomerulonephritis with
RBC casts
Usually resolve spontaneously.

DIAGNOSTIC EVALUATION
May have mild leukocytosis
Normal platelet count
Normal serum complement levels
Elevated IgA in 50%

DIAGNOSIS
Generally a clinical diagnosis
Skin Biopsy: can be helpful and used to
confirm IgA and C3 deposits and
leukocytoclastic vasculitis.
Renal Biopsy: not usually needed for
diagnosis. Will show mesangial IgA
deposits and segmental glomerulonephritis

MANAGEMENT
Usually self-limiting (1-6 weeks)
Steroids:

may decrease tissue edema, may aid in

arthralgias and some abdominal pain
Has not been shown to be beneficial in kidney
disease or dermal manifestations
Does not lessen chance of recurrence
Does not shorten duration of disease

MANAGEMENT

if rapidly progressive glomerulonephritis

Multidrug regimens with cytotoxic drugs
however not many reports with treatment in
adults.
Plasmaphoresis
IVIG

Symptomatic management of GI
symptoms and surgical intervention if
warranted.

PROGNOSIS

Prognostic factors:
generally a milder course in children with
shorter duration and fewer recurrences
Proteinuria >1gm/day with worse prognosis if
develop nephrotic syndrome

1-5% children progress to ESRD

Recurrence in up to 40% of patients

REFERENCES
Kasper, Dennis, and Eugene Braunwald, 16th edition,
eds. Harrisons Principles of Internal Medicine. New
York: McGraw-Hill, 2005.
Tierney Jr, Lawrence, and Stephen McPhee, 45th edition,
eds. Current Medical Diagnosis and Treatment. New
York: McGraw-Hill, 2006.
Uptodate: Clinical manifestations and diagnosis of
Henoch-Schonlein Purpura
Anup Rai, et al., Henoch-Schonlein Purpura Nephritis.
American Society of Nephrology. Volume 10, pages
2637-2644, 1999
Espositio et al., Henoch-Schonlein Purpura in Chronic
Hemodialys patients. Journal of Nephrology. Volume 12:
197-200, 1999