Schnlein-Henoch purpura

Author: Professor Seza Ozen1

Creation Date: March 2003
Scientific Editor: Professor Loc Guillevin
1

Department of Paediatrics, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey.
sezaozen@hacettepe.edu.tr
Abstract
Keywords
Disease name and synonyms
Diagnosis
Differential Diagnosis
Incidence
Laboratory investigations
Etiology
Genetics
Diagnostic methods
Management and treatment
Unresolved questions
Key words
References
Abstract
Schnlein-Henoch purpura (HSP) is a systemic vasculitis that affects vessels of a small calibre. Diagnosis
is clinically established when at least 2 of the following 4 criteria are present: palpable purpura, age less
20 years at disease onset, bowel angina, and wall granulocytes on biopsy. HSP incidence in children is at
least 135 per million, about 100 times more than in adults. The characteristic presentation of this
syndrome is a triad of purpura, abdominal pain and arthritis. The typical skin feature is nonthrombocytopenic palpable purpura, the cutaneous lesion is a leukocytoclastic angiitis of the small
vessels. Inflammation of visceral blood vessels and gut ischemia result in abdominal pain and bloody
stools, possible intussusceptions, and very rarely perforation. Arthritis is especially common in children.
Renal involvement occurs in almost half of the patients and usually presents as microscopic hematuria
with varying degrees of proteinuria. Despite being one of the most common vasculitides, definite data on
etiology and pathogenesis are still missing. Several different bacterial organisms have been involved as
the initiating factors of the disease. HSP is not a monogenic disease. Certain genetic polymorphisms have
been suspected to affect disease course. Therapy is administered on a case-to-case basis and treatment
of the clinical manifestations generally consists in non steroidal anti-inflammatory agents and
immunosuppressants.
Keywords
Systemic vasculitis, palpable purpura, bowel angina, wall granulocytes, onset in the childhood

Disease name and synonyms

Henoch- Schnlein purpura (HSP)
Schnlein-Henoch purpura,
Anaphylactoid purpura
Rhumatoid purpura

Diagnosis
Diagnosis of HSP is established when at least 2
of the following 4 criteria are present:
Palpable purpura
Age <20 years at disease onset
Bowel angina
Wall granulocytes on biopsy

Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003.

http://www.orpha.net/data/patho/GB/uk-HSP1.pdf

These criteria were issued by the American

College of Rheumatology in 1990. However,
diagnosis of HSP is usually made by
pediatricians only when recognizing typical
purpura (Figure 1).
The disease may also present in adults.

other areas as well. The color of the lesions

tends to change from red to purple and brown.
Edema of the extremities and scalp is also
typical.
Gastrointestinal tract
Inflammation of visceral blood vessels and gut
ischemia result in abdominal pain and bloody
stools, possible intussusceptions, and very rarely
perforation.
Musculo-skeletal system
Arthritis is especially common in children.
However, in a recent study only 23% of the
adults had arthritis. Arthralgia may also occur.

Differential Diagnosis
Other vasculitides need to be excluded.
Patients with polyarteritis nodosa and Wegeners
granulomatosis (WG) may present with purpura
and leukocytoclastic vasculitis of the skin.
Therefore these two entities should not be
mistaken for HSP.
Other clinical signs, such as severe kidney and
other organs involvement, can also be found in
microscopic polyangiitis (polyarteritis), WG, as
Churg-Strauss
syndrome
and
well
as
cryoglobulinemic vasculitis in adults. Diagnosis
of these different entities will only be made
relying on other disease-specific features and
biopsy of affected organs.
In rare atypical cases -without palpable lesions-,
a complete blood count may be required to
exclude thrombocytopenia.
Incidence
HSP occurs mainly in children: incidence in
children is at least 135 per million, about 100
times more than in adults. Ninety percent of
patients are less than 10 years.
Clinical features
Gastrointestinal involvement is an early
symptom of the disease whereas renal disease
is a late symptom. Disease course is more
severe in adults. The characteristic presentation
of HSP in children is a triad of purpura,
abdominal pain and arthritis
Skin
The typical feature of the disease is nonthrombocytopenic
palpable
purpura,
the
cutaneous lesion is a leukocytoclastic angiitis of
the small vessels. This rash is most prominent
on pressure-bearing areas, especially in the
lower extremities and buttocks, but may occur in

Kidney
Renal involvement occurs in almost half of the
patients and usually presents as microscopic
hematuria with varying degrees of proteinuria.
Nephrotic syndrome may occur. In children renal
failure is rare, whereas it is more common in
adults, as high as 10%.
Others
Central nervous system, pulmonary, cardiac and
testicular involvement has been very rarely
observed.
Laboratory investigations
The complete blood count is normal except for
mild leukocytosis in some cases. Urinalysis must
be performed, and when normal, followed for up
to 3 months, after which renal disease is unlikely
to occur. In patients with renal disease, the
degree of proteinuria should be determined and
renal function tests performed.
In patients with severe abdominal pain, an
ultrasound is helpful to delineate whether an
intussusception is present. Stools should be
examined for visible or occult blood.
Ig A levels may be elevated in 1/3-1/2 of the
patients.
Etiology
Despite being one of the most common
vasculitides, definite data on etiology and
pathogenesis are still missing.
Several different bacterial organisms have been
suspected to be involved as the initiating factors
of disease. For example, one 3-year prospective
study was carried out by Al-Sheyyab et al. in
order to examine the association of streptococci
with the disease. Antistreptolysin O titre positivity
was associated with a 10-fold increase in the risk
of HSP. IgA1 plays a critical role in the
pathogenesis of HSP and abnormalities in the
glycosylation of the hinge region of IgA1 may be
associated with the disease. IgA is the principal
antibody in the respiratory system for defense

Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003.

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against microbial agents. HSP is often preceded

by an upper respiratory tract infection.
Genetics
HSP is not a monogenic disease. Certain
genetic polymorphisms have been suspected to
affect disease course.
In a Spanish study, a certain polymorphism of
the IL-1RA (Interleukin 1 Receptor Antagonist)
gene was associated with severe renal
involvement. In another study HLA-B35 gene
has been suggested to encode renal
complications.
Diagnostic methods
HSP is diagnosed clinically. Skin biopsy is rarely
if ever-, indicated. When renal biopsy is
performed the characteristic finding is IgA
deposition mainly in the glomerular mesangium;
typically focal and segmental glomerulosclerosis
is observed.
Management and treatment
Joint pain responds well to non steroidal antiinflammatory agents. Cutaneous manifestations
are often self-limited, but may have a relapsing
pattern. For chronic cutaneous lesions different
treatment modalities have been tried such as
colchicine and aspirin, dapsone and even
systemic corticosteroids.
The optimal management of gastrointestinal and
renal involvement has not been determined yet.
Abdominal pain is common and usually selflimited. Parents should be informed in case the
child develops severe pain. For severe
abdominal pain uncontrolled studies support a
short course of corticosteroids. An oral dose of
prednisone of 1 mg/kg/day tapered after
resolution of symptoms is usually sufficient. The
major
complications
of
gastrointestinal
involvement are massive bleeding, perforation or
intussusception.
Patients with mild renal involvement such as
those with hematuria and mild proteinuria only,
require
neither
renal
biopsy
nor
immunsuppressive treatment. They should be
followed-up closely for the possible development
of more severe renal disease, however.
Corticosteroids have not been shown to alter the
prognosis in patients with mild renal disease. In
those with severe renal disease, with nephrotic
range proteinuria or impaired renal function, a
renal biopsy and high-dose corticosteroids with
or without immunosuppressants have been
suggested. If the crescent formation exceeds
50%, an aggressive approach is suggested with
3 pulses of methylprednisolone followed by at
least 3 months of oral corticosteroids (1.5 mg
prednisone/kg/day) and oral cyclophosphamide
(2 mg/kg/day).

Unresolved questions
No evidence-based data are available to show
whether the use of corticosteroids has an effect
on renal outcome or on the final outcome of the
gastrointestinal involvement. Definite data on the
pathogenesis, the factors leading to renal
involvement and preventive measures (if any)
are still missing. Data are also needed to define
factors predicting severe renal involvement.
Optimal therapy for mild and severe renal
involvement is yet to be determined.
References
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Ozen S. Henoch-Schonlein purpura. Orphanet encyclopedia, April 2003.

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