(4)DEBRA M. KRAFT, M.D., DENISE MCKEE, M.D., and CAROL SCOTT, M.D.

, University of
Nevada School of Medicine, Reno, Nevada
Am Fam Physician. 1998 Aug 1;58(2):405-408.
Henoch-Schnlein purpura is an IgA-mediated, autoimmune, hypersensitivity vasculitis of
childhood that results in a triad of symptoms, including a purpuric rash occurring on the lower
extremities, abdominal pain or renal involvement, and arthritis. However, any of the triad may
be absent, which often leads to confusion in diagnosing the condition. Although the cause is
unknown, Henoch-Schnlein purpura is often associated with infectious agents such as group A
streptococci and Mycoplasma. It has also been associated with food reactions, exposure to cold,
insect bites and drug allergies. Treatment is supportive, and children affected by this disorder
need close follow-up of renal status.
Henoch-Schnlein purpura is a nonthrombocytopenic, purpuric and systemic vasculitis of
childhood1 that occurs twice as often in males as in females. It has an incidence of 14 cases per
100,000 people and occurs most frequently in the spring and fall. 2 Henoch-Schnlein purpura may
present as a triad of symptoms: a palpable purpuric rash on the lower extremities, abdominal pain or
renal involvement, and arthritis. It can masquerade as many different conditions, depending on the
symptoms. Purpura may be defined as visible, unblanching hemorrhages in the skin or mucous
membranes that are 5 to 10 mm in diameter and often palpable. Knowledge of the classifications of
purpura may be helpful to the physician in constructing a differential diagnosis of purpura. 3
Henoch-Schnlein purpura is an inflammatory disorder of unknown cause characterized by IgAdominant immune complexes in smaller venules, capillaries and arterioles. It represents a diffuse
vasculitis that is secondary to hypersensitivity. The disorder appears to represent a variety of
leukocytoclastic angiitis initiated by deposition of immune complexes and can occur in response to
infectious agents such as group A streptococci, Mycoplasma, Epstein-Barr virus and Varicella virus.
Parvovirus B19 and Campylobacter enteritis have also been associated with Henoch-Schnlein
purpura.4,5 Cases have been reported following vaccinations for typhoid, measles, cholera and yellow
fever.6 In addition, exposure to allergens in drugs or food, exposure to cold, and insect bites have been
linked to the development of Henoch-Schnlein purpura. 6 However, the precise etiology of the
disorder is unknown. It is thought to be an IgA-mediated vasculitis, with renal lesions that are
histopathologically indistinguishable from IgA nephropathy (Berger's disease). Both diseases can
progress to renal insufficiency.7
Clinical Presentation
RASH
Henoch-Schnlein purpura is a disease of children and young adults, with 75 percent of cases
occurring between two and 11 years of age 1,8; peak incidence is five years of age. Children younger
than two years of age tend to have a milder course. A rash of erythematous papules is typically
followed by purpura, abdominal pain, arthritis and nephritis. The rash occurs in 100 percent of cases.
Lesions typically appear on the lower extremities and buttocks, but may also involve the upper
extremities, face and trunk, and are accentuated in areas of pressure (such as sock lines and the
waistline). Classic lesions consist of urticarial wheals, erythematous maculopapules and larger,

palpable ecchymosis-like lesions. Petechiae and target lesions may be present as well. These lesions
may initially blanch on pressure but later lose this feature. The purpuric areas evolve from red to
purple, become rust-colored with a brownish hue and then fade. 9 In more severe cases, hemorrhagic,
purpuric or necrotic lesions may be prominent. It is mandatory to differentiate these lesions from
those of meningococcal septicemia or other septic emboli or toxic vasculitides, such as those seen
with drug reactions, iodides and arsenicals.10
ABDOMINAL PAIN
The second most frequent symptom of Henoch-Schnlein purpura is abdominal pain, which occurs in
up to 65 percent of cases. The most common complaint is colicky abdominal pain, which may be
severe and associated with vomiting. Stools may show gross or occult blood; hematemesis may also
occur. The pain may mimic that of an acute abdomen. Severe cases may proceed to intussusception,
hemorrhage and shock. Younger children are less likely to exhibit gastrointestinal
symptoms.11 Endoscopic evaluation often shows mucosal erosions and swelling. 3
JOINT INVOLVEMENT
The third symptom of the triad is arthritis characterized by warmth, tenderness and swelling of the
joints, particularly the large joints. The ankles and knees are most frequently affected; however, the
elbows, hands and feet may also be involved. Joint symptoms occur in 70 percent of cases, are
transient and leave no permanent deformity.12 Joint symptoms may precede the rash in 25 percent of
cases.3
RENAL DISEASE
The most serious sequela of Henoch-Schnlein purpura is renal involvement. This complication
occurs in 50 percent of older children but in only 25 percent of children younger than two years.
Fewer than 1 percent of cases progress to end-stage renal disease. 13 Patients who develop renal
involvement generally do so within three months of the onset of rash. The most common
manifestation of renal disease is hematuria. Apparently, development of bloody stools with HenochSchnlein purpura is also a risk factor for renal disease. 13 Rash persistence is also associated with
nephropathy. The presence of proteinuria and hematuria is also associated with progression to renal
insufficiency. In 50 percent of patients who display a combination of nephritis-nephrotic symptoms,
end-stage disease develops after 10 years. On renal biopsy, those with glomerular crescents have a
100 percent chance of developing end-stage disease. 13 Renal histopathology may include minimal
change to severe glomerulonephritis that is indistinguishable from IgA nephropathy.
OTHER CLINICAL MANIFESTATIONS
Other rare systemic manifestations of Henoch-Schnlein purpura are listed in Table 1 and may include
hepatosplenomegaly, myocardial infarction, pulmonary hemorrhage and pleural effusion. Central
nervous system involvement may manifest as behavioral changes, seizures, headaches and focal
deficit. Peripheral nervous system lesions may appear as mononeuropathies. Extrarenal genital
involvement such as scrotal swelling and testicular torsion has also been reported. An illustration of
Henoch-Schnlein purpura can be seen in Figure 1.
View/Print Table

TABLE 1
Complications of Henoch-Schnlein Purpura

Hepatosplenomegaly
Myocardial infarction
Pulmonary hemorrhage
Pleural effusion
Unnecessary abdominal surgery
Intussusception
Hemorrhage
Shock
Gastrointestinal bleeding
Bowel infarction
Renal failure
Hematuria
Proteinuria
Seizures
Mononeuropathies
View/Print Figure

FIGURE 1.
Henoch-Schnlein purpura in a nine-month-old male Hispanic patient following a febrile
upper respiratory infection.
Diagnosis
Diagnosis is not difficult if the classic triad of rash, gastrointestinal complaints or hematuria, and
arthritis is present. The American College of Rheumatology 12 provided criteria for differentiating
Henoch-Schnlein purpura from hypersensitivity vasculitis, with the major differences being the
presence of elevated blood urea nitrogen and creatinine levels andmore importantlyglobal organ
involvement in hypersensitivity vasculitis. Ultrasound is probably the imaging modality of choice for
patients with gastrointestinal-related Henoch-Schnlein purpura. 14 However, when symptoms are not
typical, the differential diagnosis can become extensive (Table 2). Abdominal pain alone can mimic an
acute abdomen, and children have occasionally undergone laparotomy with negative findings. Joint
involvement raises the question of many rheumatic illnesses of childhood such as rheumatic fever,
rheumatoid arthritis or systemic lupus erythematosus. The rash alone may be mistaken for child
abuse, trauma, drug reactions, hemorrhagic diathesis or a septicemia such as meningococcemia. Other
conditions that present with palpable purpura include subacute bacterial endocarditis and Rocky
Mountain spotted fever.15
View/Print Table
TABLE 2
Selected Differential Diagnosis of Henoch-Schnlein Purpura

Acute abdomen
Meningococcal meningitis or septicemia
Rheumatoid arthritis
Rheumatic fever
Idiopathic thrombocytopenic purpura

Systemic lupus erythematosus

Child abuse
Drug reactions
Bacterial endocarditis
Rocky Mountain spotted fever
The diagnosis of Henoch-Schnlein purpura depends on clinical findings and history. There is not a
specific laboratory test for the disorder, although an elevated serum IgA level is suggestive. The
complete blood count may reveal a normal or elevated white blood cell count and possible
eosinophilia. Sedimentation rate and platelet count may be elevated. Electrolytes may be affected
secondary to gastrointestinal involvement. Urinalysis may show hematuria. Renal manifestations may
follow the development of the rash by up to three months; therefore, urinalysis should be performed
monthly, as well as measurements of blood urea nitrogen and creatinine levels in the presence of
continued hematuria. A stool guaiac test may be positive. An underlying infectious etiologic agent
should be excluded when clinically indicated. A normal platelet count differentiates HenochSchnlein purpura from thrombocytopenic purpura. Skin biopsy may show a leukocytoclastic
vasculitis.
Treatment
There is no specific treatment for Henoch-Schnlein purpura. Bed rest and supportive care, such as
assuring adequate hydration, are helpful. Nonsteroidal anti-inflammatory drugs can relieve joint and
soft tissue discomfort. Corticosteroids have some use in patients with severe abdominal pain.
However, corticosteroids are not recommended for treatment of rash, joint pain or renal disease alone.
Treatment with cyclophosphamide (Cytoxan, Neosar), plasmapheresis, cyclosporine (Neoral) and
azathioprine (Imuran) is controversial. In the absence of renal disease and central nervous system
involvement, the prognosis for patients with Henoch-Schnlein purpura is excellent. The illness lasts
four to six weeks in most patients. One half of patients have a reccurrence. Long-term follow-up is
necessary for patients with renal disease. The renal disease may not arise for several years. Renal
biopsy may be performed to establish the diagnosis and determine the prognosis. Prognosis overall is
excellent. The primary long-term complication is renal disease, which develops in 5 percent of
patients. One study suggests that corticosteroids and azathioprine may be helpful in treating renal
disease once it develops.16
Final Comment
In summary, Henoch-Schnlein purpura is generally a benign, self-limited condition but bears close
follow-up with repeated urinalysis as a small percentage of patients progress to renal failure.

Pharmacologic treatments are controversial, and more research needs to be done to clarify therapeutic
regimens.