Case Discussion

By
Chinnasamy Selvaraj
-Anaphylactoid purpura.
-common vasculitis of small vessels with
cutaneous and systemic complications.
-most common cause of
nonthrombocytopenic purpura in
children.

Henoch-Schönlein Purpura(HSP)
-Most frequent in children than in adults
(2-8 yrs old), males are affected 2x than
female

-Most frequently in the winter months

Epidemiology
-Unknown, but HSP often follows an
upper respiratory tract infection

Etiology
-Not known
-Characterized by increased serum concentrations of
the cytokines tumor necrosis factor-α (TNF-α) and
interleukin (IL)–6.
-Almost half of the patients had elevated
antistreptolysin O (ASO) antibodies, implicating
group A streptococcus

Pathogenesis
-IgA-mediated vasculitis of small vessels

-Immunofluorescence techniques show deposition of

IgA and C3 in the small vessels of the skin and the
renal glomeruli

-The role of complement activation is controversial

Pathogenesis
-Acute
-With the appearance of several
manifestations simultaneously, or insidious,
with sequential occurrence of symptoms
over a period of weeks or months.

-Low-grade fever and fatigue

Clinical Manifestation
-The typical rash and the clinical
symptoms of HSP are a consequence of
the usual location of the acute small
vessel damage primarily in the skin,
gastrointestinal tract, and kidneys.

Continues….
-Rash, beginning as pinkish maculopapules that
initially blanch on pressure and progress to purpura,
which are characterized clinically as palpable
purpura that evolve from red to purple to rusty brown
before they eventually fade.

-The lesions tend to occur in crops, last from 3–10

days, and may appear at intervals that vary from a
few days to as long as 3–4 months

Continues…
-In <10% of children, recurrences of the
rash may not end until as late as a yr,
and, rarely, several yr, after the initial
episode.
-Damage to cutaneous vessels also results
in local angioedema, which may precede
the palpable purpura.

Continues…
-Edema independent of purpura occurs primarily in
dependent areas such as below the waist, over the
buttocks (or on the back and posterior scalp in the
infant), or in areas of greater tissue distensibility such
as the eyelids, lips, scrotum, or dorsum of the hands
and feet.

Continues…
HSP
-Arthritis, present in more than ⅔ of
children with HSP
-The effusions are serous, not
hemorrhagic, in nature and resolve after a
few days without residual deformity or
articular damage

Continues…
-Edema and damage to the vasculature of the
gastrointestinal tract may also lead to intermittent
abdominal pain that is often colicky in nature.
-There may be peritoneal exudate, enlarged mesenteric
lymph nodes, segmental edema, and hemorrhage into
the bowel.
-More than half of patients have occult heme-positive
stools, diarrhea (with or without visible blood), or
hematemesis.

Continues…
-Intussusception may occur, which is
suggested by an empty right lower
abdominal quadrant on physical
examination or by currant jelly stools,
which may rarely be followed by
complete obstruction or infarction with
bowel perforation.

Continues…
-Renal involvement occurs in 25–50% of children and
may manifest with hematuria, proteinuria, or both;
nephritis or nephrosis; or acute renal failure.

-Renal involvement at presentation may lead to chronic

hypertension or end-stage renal disease in the future.

Continues…
-Hepatosplenomegaly and
lymphadenopathy may also be present
during active disease

-A rare but potentially serious outcome of

neurological involvement is the
development of seizures, paresis, or
coma

Continues…
-Other rare complications include
rheumatoid-like nodules, cardiac and eye
involvement, mononeuropathies,
pancreatitis, and pulmonary or
intramuscular hemorrhage.

Continues…
-The pattern of crops of palpable purpuric lesions of
similar hue in dependent areas of the body is
characteristic of HSP.
-The presentation of unremitting fever, a
maculopapular rash that does not reappear in crops
but is prominent on the lower extremities, and
peripheral arthritis may suggest Kawasaki disease.

Diagnosis
-HSP must be distinguished from
systemic-onset juvenile rheumatoid
arthritis, in which the salmon-pink rash is
evanescent and maculopapular, with
swelling that does not extend beyond the
joint.

Continues…
-Acute cutaneous benign leukocytoclastic vasculitis seen in
children ≤2 yr of age that may be confused with HSP.

-AHE presents with fever; tender edema of the face, scrotum,

hands, and feet; and ecchymosis (usually larger than the
purpura of HSP) on the face and extremities.

-The trunk is spared, but petechiae may be seen in mucous

membranes.

Acute hemorrhagic edema (AHE)

-The patient usually appears well except for the rash.
The platelet count is normal or elevated; the
urinalysis is normal.
-The younger age, nature of the lesions, absence of
other organ involvement, and biopsy may help
distinguish AHE from HSP.

Continues…
AHE
-Routine laboratory tests are neither specific nor
diagnostic.
-Affected children often have a moderate
thrombocytosis and leukocytosis.
-The erythrocyte sedimentation rate (ESR) may be
elevated.

Laboratory Findings
Laboratory Findings
-Anemia may result from chronic or acute
gastrointestinal blood loss.

-Immune complexes are often present, and 50% of

patients have elevated concentrations of IgA as
well as IgM but are usually negative for
antinuclear antibodies (ANAs), antibodies to
nuclear cytoplasmic antigens (ANCAs), and
rheumatoid factor (even in the presence of
rheumatoid nodules).
-Anticardiolipin or antiphospholipid antibodies may be
present and contribute to the intravascular
coagulopathy.
-Intussusception is usually ileoileal in location; barium
enema may be used for both identification and
nonsurgical reduction.
-Renal involvement manifests in red blood cells, white
blood cells, casts, or albumin in the urine and
azotemia.

Laboratory Findings
-Definitive diagnosis of vasculitis is
confirmed by biopsy, which is
obtained when the clinical presentation
is atypical, of an involved cutaneous
site showing leukocytoclastic angiitis.

-Renal biopsy may show mesangial

deposition of IgA and occasionally
IgM, C3, and fibrin.

Laboratory Findings
Skin biopsy of one papule demonstrated
leukocytoclastic vasculitis on routine histology.

-Immunofluorescence micrograph of a glomerulus

from a patient with IgA nephropathy (IgAN) stained
for the presence of IgA.

Immunostaining for IgA in a patient with Henoch-

Schönlein nephritis.

Cont…
Symptomatic treatment:
-adequate hydration
-bland diet
-pain control with acetaminophen is
provided for self-limited complaints of
arthritis, edema, fever, and malaise

Treatment
 -Avoidance of competitive activities and avoidance
of maintaining the lower extremities in a
dependent position may decrease local edema.

-If edema involves the scrotum, elevation of the

scrotum and local cooling, as tolerated, may
decrease discomfort.

Treatment
-Intestinal complications (hemorrhage, obstruction,
intussusception) may be life-threatening and
managed with cortico-steroids and, when
necessary, resection of the intussusception

-Therapy with oral or intravenous corticosteroids

(1–2 mg/kg/day) is often associated with dramatic
improvement of both gastrointestinal and CNS
complications.

Treatment
-Rarely, children develop chronic or recurrent HSP,
which may respond to pulse doses of intravenous
methylprednisolone (30 mg/kg/day, maximum 1
g/day, daily for 3 days followed by 1–2 times weekly
and tapered in frequency depending on response).

Treatment
-Management of renal involvement is the same as
for other forms of acute glomerulonephritis.

-If anticardiolipin or antiphospholipid antibodies

are identified and thrombotic events have
occurred, aspirin given once may decrease the
risks associated with a hypercoagulable state.

-Rheumatoid nodules may respond to alternate-day

colchicine (0.6 mg every other day).
The major complications :
-renal involvement, including nephrotic
syndrome, and bowel perforation.

Complications
-An infrequent complication of scrotal
edema is testicular torsion, which is
quite painful and must be treated
promptly

Complications
 -self-limited vasculitic disease with an excellent
overall prognosis

-Chronic renal disease may result in morbidity

-A population-based study indicated that <1% of

patients with HSP develop persistent renal disease
and <0.1% develop serious renal disease

Prognosis
 -Rarely, death may occur during the
acute phase of the disease as a result of
bowel infarction, CNS involvement, or
renal disease.
-Occasionally, children who present
with an HSP-like syndrome acquire
characteristics of other connective
tissue diseases.

Prognosis
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