Professional Documents
Culture Documents
BRIAN V. REAMY, COL, USAF, MC, and PAMELA M. WILLIAMS, LT COL, USAF, MC
Uniformed Services University of the Health Sciences, Bethesda, Maryland
TAMMY J. LINDSAY, LT COL, USAF, MC, Saint Louis University Family Medicine Residency Program,
Belleville, Illinois
H
Patient information: Pathophysiology
▲
enoch-Schönlein purpura is
A handout on Henoch-
Schönlein purpura, writ- an acute, systemic, immune In patients with Henoch-Schönlein purpura,
ten by the authors of complex–mediated, leukocyto- immunoglobulin A (IgA) immune com-
this article, is available clastic vasculitis. It is character- plexes are deposited in small vessels, which
at http://www.aafp. ized by a clinical triad of palpable purpura causes petechiae and palpable purpura.
org/afp/20091001/697-
s1.html. (without thrombocytopenia), abdominal When immune complexes occur in small
pain, and arthritis. Glomerulonephritis vessels of the intestinal wall, gastrointesti-
and gastrointestinal bleeding are common nal hemorrhage may develop. If the immune
complications. complexes affect the renal mesangium, it
may produce mild proliferative to severe
Epidemiology crescentic glomerulonephritis.10
Henoch-Schönlein purpura occurs in Exposure to an antigen from an infection,
approximately 10 to 22 persons in 100,000 medication, or other environmental factor
each year.1-4 It is most commonly seen from may trigger antibody and immune complex
late autumn to early spring, but it may occur formation. Group A streptococcus has been
at any time.5 More than 90 percent of patients found in cultures of more than 30 percent of
are children younger than 10 years, with a children with Henoch-Schönlein nephritis,
peak incidence at six years of age.2-5 How- and serum antistreptolysin-O titers are more
ever, it is also seen in infants, adolescents, likely to be positive in patients with Henoch-
and adults. Henoch-Schönlein purpura is Schönlein nephritis.4,11 Other postulated viral
milder in infants and children younger than and bacterial triggers of Henoch-Schönlein
two years.6 It is more severe and more likely purpura include parvovirus B19, Bartonella
to cause long-term renal disease in adults.7,8 henselae, Helicobacter pylori, Haemophilus
Henoch-Schönlein purpura is the most com- parainfluenza, Coxsackie virus, adenovi-
mon vasculitis in children and has a slight rus, hepatitis A and B viruses, mycoplasma,
predominance in males.3-5,9 Epstein-Barr virus, varicella, campylobacter,
Downloaded from the American Family Physician Web site at www.aafp.org/afp. Copyright © 2009 American Academy of Family Physicians. For the private, noncommercial
use of one individual user of the Web site. All other rights reserved. Contact copyrights@aafp.org for copyright questions and/or permission requests.
SORT: KEY RECOMMENDATIONS FOR PRACTICE
Evidence
Clinical recommendation rating References
698 American Family Physician www.aafp.org/afp Volume 80, Number 7 ◆ October 1, 2009
Henoch-Schönlein Purpura
October 1, 2009 ◆ Volume 80, Number 7 www.aafp.org/afp American Family Physician 699
Henoch-Schönlein Purpura
purpose of laboratory evaluation is to iden- enema may be used safely to diagnose and
tify complications or exclude other diseases treat suspected intussusception, although
(Table 2).3,11,16 Useful baseline studies include the location is usually ileoileal and less ame-
renal function tests (e.g., electrolytes, blood nable to nonsurgical correction by barium
urea nitrogen, creatinine, urinalysis), com- enema.25
plete blood count with platelet count, coagu-
lation profile (e.g., prothrombin time, partial Differential Diagnosis
thromboplastin time, fibrinogen, fibrin deg- Key differential diagnostic considerations
radation products), and IgA levels.3,11,16 of Henoch-Schönlein purpura are shown
A normal platelet count, leukocytosis, eosin- in Table 3.26,27 These include polyarteri-
ophilia, azotemia, elevated serum IgA levels, tis nodosa, juvenile rheumatoid arthri-
hematuria, proteinuria, and red blood cell tis, acute hemorrhagic edema of infancy,
casts are commonly seen. The need for blood Wegener granulomatosis, meningococce-
cultures, antistreptolysin-O titers, renal mia, Kawasaki disease, and thrombocytope-
biopsy, and tests to identify other infectious nic purpura.26,27 All causes of acute surgical
agents depends on the presence of clinical abdomen are diagnostic considerations in
indicators of specific infections or progres- patients with severe abdominal pain. Promi-
sive renal disease. nent purpuric ecchymoses in the absence of
Imaging studies are not performed rou- other symptoms may be mistaken for child
tinely in the evaluation of Henoch-Schönlein abuse. Other causes of palpable purpura
purpura.17 Arteriography may be required to include Rocky Mountain spotted fever and
localize hemorrhage, and gastrointestinal bacterial endocarditis. Henoch-Schönlein
endoscopy may be needed to evaluate gas- purpura may occur with other forms of
trointestinal bleeding.17 Abdominal ultraso- autoimmune disease, such as familial
nography or computed tomography may be Mediterranean fever or inflammatory bowel
needed to assess abdominal pain.17,25 Barium disease. One study reported that 5 percent of
700 American Family Physician www.aafp.org/afp Volume 80, Number 7 ◆ October 1, 2009
Table 3. Differential Diagnosis for Henoch-Schönlein Purpura
Acute abdomen Acute or subacute onset of abdominal pain or rigidity CBC, hCG, amylase, liver function
tests, urinalysis, abdominal CT
scan
Acute hemorrhagic Small vessel leukocytoclastic vasculitis affecting children four to Skin biopsy, CBC, coagulation
edema 24 months of age; rapid onset of purpuric lesions on face, ears, studies, urinalysis
and extremities; associated edema of limbs and face
Bacterial endocarditis Acute or subacute onset of fever; vascular phenomena Blood cultures drawn more than
(e.g., arterial emboli, septic pulmonary infection); immunologic two hours apart, CBC, ESR,
phenomena (e.g., glomerulonephritis, Osler nodes, Roth spots); echocardiography
new valvular regurgitation
Child abuse Worrisome skin marks (e.g., ecchymosis, shaped contusions, Detailed history and physical
burns, bites, lacerations); blunt abdominal or other trauma; examination; appropriate
fractures; immersion injuries laboratory tests and imaging
Familial Mediterranean Paroxysms of fever and polyserositis (e.g., abdominal pain, Mutation analysis for the MEFV
fever* peritonitis, pleurisy, pericarditis, synovitis) gene mutation
Hypersensitivity Small-vessel vasculitis associated with abrupt onset of a rash Skin biopsy, CBC, ESR
(leukocytoclastic) (palpable purpura most common); fever, malaise, myalgia,
vasculitis and anorexia after exposure to triggering antigen (e.g., drug,
infectious agent)
Inflammatory bowel Diarrhea (bloody or nonbloody), abdominal pain, cramping, CBC, ESR, electrolytes, stool
disease* fever, and weight loss studies, colonoscopy with biopsy
Juvenile rheumatoid Insidious or abrupt onset of morning stiffness or arthralgia that ESR, CBC, antinuclear antibody
arthritis may be associated with spiking fevers and an evanescent
salmon-pink rash
Kawasaki disease Child with fever of 102.9°F to 104.9°F (39.4°C to 40.5°C) for five CBC, CRP, ESR, echocardiography,
days or longer; associated with extensive polymorphous rash, electrocardiography
bilateral conjunctival injection, changes of the lip and oral cavity,
unilateral cervical lymphadenopathy, and extremity changes
Leukemia Nonspecific symptoms of fever, easy bleeding, skin findings CBC, ESR, coagulation profile,
(e.g., petechiae, purpura), bone pain, fatigue, bone marrow biopsy
hepatosplenomegaly, lymphadenopathy
Meningococcemia Malaise, fever, rash (e.g., maculopapular rash, petechiae, CBC, PT/aPTT, fibrinogen, fibrin
ecchymosis), and hypotension with possible associated degradation productions, blood
symptoms of meningitis or tenosynovitis culture, CSF analysis and culture
Polyarteritis nodosa Multisystem involvement from segmental inflammatory, Biopsy of involved organ,
necrotizing vasculitis of the small- and medium-sized muscular urinalysis, CBC, ESR, CRP,
arteries leading to general symptoms of fever, weakness, angiography
weight loss, malaise, myalgia, rash (e.g., livedo reticularis,
purpura), headache, and abdominal pain
Rocky Mountain Headache; fever associated with a centripetal rash (involving Immunofluorescence staining of
spotted fever palms and soles, spreading to arms, legs, and trunk) that is tissue specimen or serologic
often petechial; report of recent tick bite or outdoor activity analysis for Rickettsia rickettsii,
in endemic area CBC, electrolytes
Thrombocytopenic Thrombocytopenia associated with petechia or purpura; bleeding CBC with peripheral smear,
purpura (e.g., gingival, gastrointestinal, mucocutaneous); symptoms of coagulation studies,
end-organ ischemia (in patients with thrombotic disease) platelet-associated antibody
(immunoglobulin G), electrolytes,
BUN, creatinine, liver function
tests, LDH, urinalysis
Wegener Granulomatous vasculitis with a characteristic triad of involvement Biopsy of involved organ, c-ANCA,
granulomatosis of the upper airway (e.g., otitis media, sinusitis, rhinitis), CBC, ESR, electrolytes, BUN,
lungs (e.g., pulmonary infiltrates), and kidneys; rash, arthritis/ creatinine, urinalysis, chest
arthralgias, and nervous system (peripheral and central) radiography
patients with familial Mediterranean fever affected extremities during the active phase
will get Henoch-Schönlein purpura.28 of the illness may help prevent purpura.
Patients should be advised that they may
Potential Complications experience recurrent purpura as they increase
Orchitis and scrotal swelling may occur in up their activity level.
to 35 percent of boys with Henoch-Schönlein Hospitalization may be required when ade-
purpura.5 Severe scrotal edema may cause quate outpatient monitoring is unavailable or
testicular torsion, which requires emergent if dehydration, hemorrhage, or pain control
surgical exploration. Fewer than 10 percent require inpatient management. Nephrology
of patients with Henoch-Schönlein purpura referral is recommended with significant renal
experience myocardial infarction, pulmo- involvement.29 In patients with severe renal
nary hemorrhage, or central nervous system disease, renal biopsy is needed to provide a
involvement with seizures and hemorrhage definitive diagnosis and guide therapy.29
(Table 45,10,13,16).13,16 Early steroid treatment is most appropri-
ate for children with renal involvement or
Management severe extrarenal symptoms.30 It may also
Because Henoch-Schönlein purpura sponta- help relieve scrotal swelling.5 Oral predni-
neously resolves in 94 percent of children and sone at 1 to 2 mg per kg daily for two weeks
89 percent of adults, supportive treatment is has been used to treat moderate to severe
the primary intervention.5,22,29 Acetamino- abdominal and joint symptoms, and to
phen or nonsteroidal anti-inflammatory hasten the resolution of Henoch-Schönlein
drugs (NSAIDS) may be used to alleviate purpura in children.30 A double-blind ran-
arthralgia, although NSAIDS may aggra- domized trial found that early treatment
vate gastrointestinal symptoms and should with prednisone reduced abdominal and
be avoided in patients with known renal joint pain severity in children.30 Although
involvement. Relative rest and elevation of prednisone did not prevent renal disease, it
was useful in treating renal disease after it
started.30 A meta-analysis found that cor-
Table 4. Potential Complications of Henoch-Schönlein ticosteroid use in children with Henoch-
Purpura Schönlein purpura reduced the mean
time to resolution of abdominal pain and
Renal Central nervous system decreased the odds of developing persistent
Glomerulonephritis Aphasia renal disease.31 Steroids did not affect the
Hemorrhagic cystitis Ataxia resolution of the purpura, and no harms
Nephrotic syndrome Cerebral hemorrhage were demonstrated.31
Renal failure Chorea Early aggressive therapy is recommended
Ureteral obstruction Cortical blindness for children and adults with severe renal
Gastrointestinal Neuropathy involvement.30-32 Treatment options include
Bowel infarction Paresis high-dose steroids with immunosuppres-
Bowel perforation Seizure sants, high-dose intravenous immunoglob-
Duodenal obstruction Other ulin, plasmapheresis, and renal transplant.
Gastrointestinal hemorrhage Anterior uveitis A recent trial found that cyclophospha-
Intestinal stricture Myocarditis mide (Cytoxan) was effective in patients
Intussusception Myositis with overt nephritis, although cyclo-
Pulmonary Orchitis sporine (Sandimmune) was not helpful
Alveolar hemorrhage Scrotal edema (Table 5 ).
5,16,30,31 32
702 American Family Physician www.aafp.org/afp Volume 80, Number 7 ◆ October 1, 2009
Henoch-Schönlein Purpura
*—Recommended pediatric dosage is prednisone 1 to 2 mg per kg daily for one to two weeks, followed by a taper.
†—Nephrology, gastroenterology, surgery, rheumatology, or other subspecialty as determined by presenting symp-
toms or organ systems involved.
Information from references 5, 16, 30, and 31.
Most patients recover fully within four and facilitate early nephrology consultation
weeks.16 Recurrences of Henoch-Schönlein and the initiation of steroids.33
purpura occur in up to one third of patients
within the first six months after onset and The Authors
are more common in patients with renal BRIAN V. REAMY, COL, USAF, MC, is the chair of the
involvement. Department of Family Medicine at Uniformed Services
Long-term prognosis depends on the University of the Health Sciences in Bethesda, Md.
severity of renal involvement; end-stage PAMELA M. WILLIAMS, LT COL, USAF, MC, is an assistant
renal disease occurs in 1 to 5 percent of professor in the Department of Family Medicine at the Uni-
patients.12,29 One systematic review revealed formed Services University of the Health Sciences.
that the onset of renal disease in patients TAMMY J. LINDSAY, LT COL, USAF, MC, is the interim pro-
with Henoch-Schönlein purpura developed gram director at Saint Louis University Family Medicine
Residency Program in Belleville, Ill., which is affiliated with
within four weeks in 85 percent of patients, Scott Air Force Base.
six weeks in 91 percent of patients, and six
Address correspondence to Brian V. Reamy, COL, USAF,
months in 97 percent of patients.33 Perma- MC, Uniformed Services University, 4301 Jones Bridge
nent renal impairment did not develop in Rd., Bethesda, MD 20814 (e-mail: breamy@usuhs.mil).
patients with a normal urinalysis, although Reprints are not available from the authors.
it occurred in 19.5 percent of patients with Author disclosure: Nothing to disclose.
nephritic or nephrotic syndrome.33 The opinions and assertions contained herein are the
A blood pressure measurement and uri- private views of the authors and not to be construed as
nalysis should be performed at the time official or reflecting the views of the Uniformed Services
Henoch-Schönlein purpura is diagnosed and University, the U.S. Air Force. or the U.S. Department of
Defense.
at each return physician office visit.33 Serum
blood urea nitrogen and creatinine determi-
REFERENCES
nation are needed if hematuria or protein-
uria are identified.33 If the initial urinalysis 1. Gardner-Medwin JM, Dolezalova P, Cummins C, South-
wood TR. Incidence of Henoch-Schönlein purpura, Kawa-
is normal, or if there is isolated hematuria
saki disease, and rare vasculitides in children of different
(without nephritic or nephrotic syndrome), ethnic origins. Lancet. 2002;360(9341):1197-1202.
a monthly urinalysis should be performed 2. Calviño MC, Llorca J, García-Porrúa C, Fernández-Iglesias
for the first six months after the diagnosis JL, Rodriguez-Ledo P, González-Gay MA. Henoch-
Schönlein purpura in children from northwestern Spain:
of Henoch-Schönlein purpura.33 This will a 20-year epidemiologic and clinical study. Medicine.
detect the first signs of renal involvement, 2001;80(5):279-290.
October 1, 2009 ◆ Volume 80, Number 7 www.aafp.org/afp American Family Physician 703
Henoch-Schönlein Purpura
704 American Family Physician www.aafp.org/afp Volume 80, Number 7 ◆ October 1, 2009