HENOCH-SCHÖNLEIN

PURPURA
ETIOLOGY
Henoch-Schönlein purpura (HSP) is a vasculitis of unknown etiology
characterized by inflammation of small blood vessels with leukocytic
infiltration of tissue, hemorrhage, and ischemia. The immune complexes
associated with HSP are predominantly composed of IgA.

EPIDEMIOLOGY
HSP is the most common systemic vasculitis of childhood and cause of
nonthrombocytopenic purpura, with an incidence of 13 per 100,000
children. It occurs primarily in children 3 to 15 years of age, although it
has been described in adults. HSP is slightly more common in boys than
girls and occurs more frequently in the winter than in the summer months.

CLINICAL MANIFESTATIONS
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 Red Urine and Hematuria
 Proteinuria
 Scrotal Pain
 Fever and Rash
 Petechiae/Purpura
HSP is characterized by rash, arthritis, and, less frequently,
gastrointestinal or renal vasculitis. The hallmark of HSP is palpable
purpura, caused by small vessel inflammation in the skin leading to
extravasation of blood into the surrounding tissues, frequently with IgA
deposition. The rash is classically found in dependent areas: below the
waist, on the buttocks, and lower extremities (Fig. 87-1). The rash can
begin as small macules or urticarial lesions but rapidly progresses to
purpura with areas of ecchymosis. The rash also can be accompanied
by edema, particularly of the calves and dorsum of the feet, scalp, and
scrotum or labia. HSP occasionally is associated with encephalopathy,
pancreatitis, and orchitis. Arthritis occurs in 80% of patients with HSP and
is most common in the lower extremities, particularly the ankles and
knees. The arthritis is acute and very painful with refusal to bear weight.
Joint swelling can be confused with peripheral edema seen with the rash
of HSP. Gastrointestinal involvement occurs in about one half of affected
children and most typically presents as mild to moderate crampy
abdominal pain, thought to be due to small vessel involvement of the
gastrointestinal tract leading to ischemia. Less commonly, significant
abdominal distention, bloody diarrhea, intussusception, or abdominal
perforation occurs and requires emergent intervention. Gastrointestinal
involvement is typically seen during the acute phase of the illness. It may
precede the onset of rash.
One third of children with HSP develop renal involvement, which can be
acute or chronic. Although renal involvement is mild in most cases, acute
glomerulonephritis manifested by hematuria, hypertension, or acute renal
failure can occur. Most cases of glomerulonephritis occur within the first
few months of presentation, but rarely patients develop late renal disease,
which ultimately can lead to chronic renal disease, including renal failure.

Figure 87-1 Rash of Henoch-Schönlein purpura on the lower

extremities of a child. Note evidence of both purpura and petechiae

LABORATORY AND IMAGING STUDIES

Erythrocyte sedimentation rate, C-reactive protein, and white blood cell
count are elevated in patients with HSP. The platelet count is the most
important test, because HSP is characterized by nonthrombocytopenic
purpura with a normal, or even high, platelet count, differentiating HSP
from other causes of purpura that are associated with thrombocytopenia
such as autoimmune thrombocytopenia, systemic lupus erythematosus, or
leukemia. A urinalysis screens for evidence of hematuria. A serum blood
urea nitrogen and creatinine should be obtained to evaluate renal
function. Testing the stool for blood may identify evidence of gut ischemia.
Any question of gut perforation requires radiologic investigation.

DIFFERENTIAL DIAGNOSIS
The diagnosis of HSP is based on the presence of two of four criteria (Table
87-1), which provides 87.1% sensitivity and 87.7% specificity for the
disease. The differential diagnosis includes other systemic vasculitides
(Table 87-2) and diseases associated with thrombocytopenic purpura, such
as idiopathic thrombocytopenic purpura and leukemia.

TREATMENT
Therapy for HSP is supportive. A short-term course of nonsteroidal anti-
inflammatory drugs can be administered for the acute arthritis. Systemic
corticosteroids usually are reserved for children with gastrointestinal
disease and provide significant relief of abdominal pain. A typical dosing
regimen is prednisone, 1 mg/kg/day for 1 to 2 weeks, followed by a taper
schedule. Recurrence of abdominal pain as corticosteroids are weaned
may necessitate a longer course of treatment. Acute nephritis typically is
treated with corticosteroids but may require more aggressive
immunosuppressive therapy.

COMPLICATIONS
Most cases of HSP are monophasic, lasting 3 to 4 weeks and resolving
completely. The rash can wax and wane, however, for 1 year after HSP.
Parents should be warned regarding possible recurrences. The arthritis of
HSP does not leave any permanent joint damage; it does not typically
recur. Gastrointestinal involvement can lead to temporary abnormal
peristalsis that poses a risk of intussusception, which may be followed by
complete obstruction or infarction with bowel perforation. Any child with a
recent history of HSP who presents with acute abdominal pain,
obstipation, or diarrhea should be evaluated for intussusception. Renal
involvement rarely may lead to renal failure.

PROGNOSIS
The prognosis of HSP is excellent. Most children have complete resolution
of the illness without any significant sequelae. Patients with HSP renal
disease (elevated blood urea nitrogen, persistent high-grade proteinuria)
are at highest risk for long-term complications, such as hypertension or
renal insufficiency, particularly if the initial course was marked by
significant nephritis. There is a long-term risk of progression to end-stage
renal disease in less than 1% of children with HSP. The rare patients who
develop end-stage renal disease may require renal transplantation. HSP
may recur in the transplanted kidney

Table 87-2 Classification of Vasculitides

ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED
VASCULITIS

Granulomatosis with polyangiitis (formerly known as Wegener

granulomatosis)
Polyarteritis nodosa
Churg-Strauss syndrome
Microscopic polyangiitis

HYPERSENSITIVITY SYNDROMES
Henoch-Schönlein purpura
Serum sickness (e.g., drug-related)
Vasculitis associated with infections

CONNECTIVE TISSUE DISEASES

Systemic lupus erythematosus
Dermatomyositis
Juvenile idiopathic arthritis

GIANT CELL ARTERITIS

Temporal arteritis
Takayasu arteritis

OTHERS
Behçet syndrome
Kawasaki disease
Hypocomplementemic urticarial vasculitis