eMedicine Specialties > Dermatology > Diseases of the Vessels

Henoch-Schonlein Purpura (Anaphylactoid Purpura)

Andrew D Montemarano, DO, Consulting Staff, The Skin Cancer Surgery Center

Updated: Aug 12, 2009

Introduction

Background

Henoch-Schönlein purpura (HSP, or anaphylactoid purpura) is an immunoglobulin (Ig) A-mediated small-vessel vasculitis that predominantly affects children but also is seen in adults. HSP is a subset of necrotizing vasculitis characterized by fibrinoid destruction of blood vessels and leukocytoclasis. Clinical manifestations primarily include palpable purpura, arthralgia or arthritis, abdominal pain, gastrointestinal (GI) bleeding, and nephritis. The most serious long-term complication from HSP is progressive renal failure, which occurs in 1-2% of patients.
Heberden first described the disease in 1801 in a 5-year-old child with abdominal pain, hematuria, hematochezia, and purpura of the legs. In 1837, Johann Schönlein described a syndrome of purpura associated with joint pain and urinary precipitates in children. Eduard Henoch, a student of Schönlein's, further associated abdominal pain and renal involvement with the syndrome. Frank proposed the term "anaphylactoid purpura" in 1915. This followed from the reasoning that the pathogenesis likely involved a hypersensitivity reaction to an inciting agent.

Two major classification systems are used to make a diagnosis of HSP. The first, from the American College of Rheumatology, requires 2 or more of the following to be present:

• Patient age younger than 20 years
• Palpable purpura
• Abdominal pain or GI bleeding
• Extravascular or perivascular granulocytes on biopsy

• Direct immunofluorescence (DIF) results consistent with vascular IgA deposition
• Patient age younger than 20 years
• GI involvement
• Upper respiratory tract infection tract (URI) prodrome
• Mesangioproliferative glomerulonephritis with or without IgA deposition

• Palpable purpura
• Bowel angina
• GI bleeding
• Hematuria
• Patient age of onset younger than 20 years
• No medications as a precipitating agent

Pathophysiology

The etiology of HSP (anaphylactoid purpura) is unknown but involves the vascular deposition of IgA immune complexes. More specifically, the immune complexes are composed of IgA1 and IgA2 and are produced by peripheral B lymphocytes. These complexes likely are formed in response to an inciting factor. The circulating complexes become insoluble, are deposited in the walls of small vessels (arteries, capillaries, venules), and activate complement, most likely by the alternative pathway (based on the presence of C3 and properdin and the absence of the first component of complement in most biopsies).

Polymorphonuclear leukocytes are recruited by chemotactic factors and cause inflammation and necrosis of vessel walls with concomitant thrombosis. This leads to extravasation of erythrocytes from hemorrhage in the affected organs and is manifested histologically as leukocytoclastic vasculitis.

Histology of involved skin reveals polymorphonuclear cells or cell fragments around small dermal blood vessels. Immune complexes containing IgA and C3 have been found in skin, kidneys, intestinal mucosa, and joints, which are the major organ sites involved in HSP.

Clinical manifestations of HSP reflect small-vessel injury. Abdominal pain, present in as many as 65% of patients, is secondary to vasculitis-induced submucosal and subserosal hemorrhage and edema, with thrombosis of the microvasculature in the gut. Hematuria and proteinuria occur in HSP-associated nephritis. Renal manifestations range from minimal change to severe crescentic glomerulonephritis.

Etiology is secondary to the mesangial deposition of IgA predominantly, but IgG, IgM, C3, and properdin deposition also may occur. These deposits also can occur in the subendothelial and subepithelial glomerular spaces. Many believe that both HSP nephritis and IgA nephropathy (Berger disease), which are the most common causes of glomerulonephritis in the world, are different clinical presentations of the same disease process. Dermatologic manifestations occur secondary to immune complex deposition (IgA, C3) in vessels of the papillary dermis, resulting in vessel injury, extravasation of RBCs, and clinically observable palpable purpura. This tends to occur in dependent body regions, such as the lower legs, buttocks, back, and abdomen.

As many as 50% of occurrences in pediatric patients are preceded by a URI, and a recent study in adults demonstrated that 40% of patients had an antecedent URI. Several agents have been implicated, including group A streptococci, varicella, hepatitis B, Epstein-Barr virus, parvovirus B19, Mycoplasma, Campylobacter, and Yersinia. Less commonly, other factors have been associated as inciting agents in the development of HSP. These include drugs, malignancy, foods, pregnancy, familial Mediterranean fever, and exposure to cold. HSP also has been reported following vaccinations for typhoid, measles, yellow fever, and cholera.

Frequency

United States

In the US, 75% of HSP (anaphylactoid purpura) occurrences are in children aged 2-14 years. The incidence in this age group is 14 cases per 100,000 population.

International

Although no reports cite differences in the incidence of HSP among countries, one source states that the occurrence of glomerulonephritis resulting from HSP varies among countries. HSP accounts for 18-40% of glomerular diseases in Japan, France, Italy, and Australia while responsible for only 2-10% of glomerular lesions in the US, Canada, and the United Kingdom. No explanation for these differences was offered, but they may be secondary to differences in associated provocative or inducing factors among locations.

Mortality/Morbidity

Most morbidity and mortality in HSP (anaphylactoid purpura) results from glomerulonephritis and its associated acute and chronic renal manifestations. At a minimum, transient hematuria occurs in 90% of patients. Renal insufficiency occurs in less than 2% of patients, and end-stage renal failure occurs in less than 1%. HSP accounts for 3-15% of children entering dialysis programs.

Although rare, pulmonary hemorrhage is an often fatal complication of HSP.³

Race

HSP is uncommon in black persons, both in the United States and in Africa.

Season: HSP incidence is greatest in the spring, fall, and winter months.

Sex

Male-to-female predominance ranges from 1.5-2:1.

Age

• Most patients (75%) are children aged 2-14 years. The median age of onset is 4-5 years. Although one of the criteria for the diagnosis of HSP as published by the American College of Rheumatology is "age less than 20 years," the disease can occur from infancy to the ninth decade.
• A study by Allen et al shows that the clinical manifestations of HSP vary with age. Children younger than 2 years have less renal, GI, and joint involvement but more subcutaneous edema.⁴

Clinical

History

The presenting history varies with each patient who has Henoch-Schönlein purpura (HSP, or anaphylactoid purpura). The hallmark of the disease is the characteristic palpable purpura, which is seen in almost 100% of patients. HSP tends to occur on the buttocks and upper thighs in younger children and on the feet, ankles, and lower legs of older children and adults. Patients often present with low-grade fever and malaise in addition to more specific symptoms. Purpura may be the presenting sign. As many as 50% of children present with symptoms other than purpura. The eruption often is preceded by arthralgia or arthritis, abdominal pain, or testicular swelling. Although it may be present initially, renal disease often develops up to 3 months after initial presentation.

• Joint symptoms: Arthralgia or arthritis is the presenting complaint in 25% of patients and occurs at some point in 60-75% of patients. Ankles and knees are affected most commonly, although any joint may be involved.
• Abdominal pain: Abdominal pain with concomitant hematochezia is the second most frequent symptom, is observed in 50-65% of patients, and is the presenting complaint in 10-15%. Pain tends to be sharp or colicky, and surgical consultation may be indicated to exclude a surgical abdomen. Indeed, as many as 6% of patients require surgery as a result of complications such as intussusception, bowel wall perforation, or infarction. Gastric hemorrhage with hematemesis, hydrops of the gallbladder, appendicitis, and pancreatitis also has been reported.
• Renal involvement
  • An incidence of renal involvement of 10-60% has been reported, and the extent of glomerular injury mostly determines the long-term morbidity and mortality of HSP. The presence of glomerular crescents on renal biopsy correlates with a poor prognosis. One study of 57 adults with HSP showed that a recent URI, purpura of the upper part of the trunk, fever, and presence of serum markers of inflammation (eg, erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]; see Lab Studies) predicted renal involvement.
  • HSP nephritis usually presents as macroscopic hematuria and proteinuria lasting days to weeks. These may be accompanied by increased plasma creatinine and/or hypertension, followed by microscopic hematuria, which may last months to years. Gross hematuria may occur years after the initial illness with relapses of purpura, often following a URI. Of those patients with renal involvement, as many as 10% may develop chronic renal failure and end-stage renal disease. However, less than 1% of patients with HSP have this poor prognosis.
• Disease recurrence: Disease recurrence occurs throughout weeks to months in adults and children. In the large pediatric study by Allen et al, children older than 2 years had a recurrence rate of 50%, while those younger than 2 years had a less than 25% chance of recurrence.⁴ The primary differences between children and adults, according to one study of 57 adults with HSP, are the chronicity and severity of the eruption in the latter population. Bullae and ulcers are more common in adults, and cutaneous exacerbations may be seen for 6 months or longer.
• Other signs and symptoms: Less common manifestations of HSP include testicular pain and swelling, hepatosplenomegaly, central or peripheral nervous system involvement (seizures or mononeuropathies, respectively), headache, and rarely, myocardial infarction or pulmonary hemorrhage.
• Other pertinent history: Ascertain the history of recent drug ingestion and food consumption, since reports exist of medication-associated and food-associated HSP.

Physical

A full physical examination is indicated, since Henoch-Schönlein purpura (HSP, or anaphylactoid purpura) can affect many organ systems.

• Skin - Primary lesion
  • An eruption may begin as erythematous macular or urticarial lesions, progressing to blanching papules, and later, to palpable purpura, usually 2-10 mm in diameter.
  • Bullae, vesicles, petechiae, and ecchymotic, necrotic, ulcerative, or targetlike lesions also may occur.
  • Subcutaneous edema is common in children younger than 3 years.

Purpuric papules and plaques of the lower extremity characteristic of Henoch-Schönlein purpura.

Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura.

• Skin - Distribution
  • Lesions typically are symmetric and tend to be distributed in dependent body areas, such as the ankles and lower legs in older children and adults, and the back, buttocks, upper extremities, and upper thighs in young children, since these regions tend to be dependent in the latter group. The face, palms, soles, and mucous membranes usually are spared, except in infants, in whom facial involvement may not be uncommon. The subcutaneous edema prominent in young children involves the scalp, periorbital regions, hands, feet, and scrotal area.
  • Lesions usually occur in crops and may fade over several days. Recurrences tend to occur in the same sites as previous lesions.

Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation with ecchymoses on the dorsal foot and ankle.

• Skin: Color as seen in the areas of purpura progresses from red to purple, then becomes rust-colored or brown before fading.
• Heart: Cardiac tamponade and myocardial infarction rarely have been reported with HSP.
• Lungs: Although seldom a manifestation of HSP, pulmonary hemorrhage has been reported. When present, it is a poor prognostic sign with a 50% mortality rate. One pediatric study showed that 95% of patients with active disease had impaired diffusion capacity of carbon monoxide, which was readily reversible once the syndrome resolved.
• Abdomen: Pain secondary to vasculitic involvement of small mesenteric or bowel mucosal vessels is common. Examine the abdomen for a palpable mass, which may indicate intussusception. Pancreatitis, gallbladder hydrops, appendicitis, and massive gastric hemorrhage also have been reported.⁵
• Scrotum/testicles: Testicular involvement has varied in reports from 4-38%. Testicular pain may be so intense that it mimics torsion.
• Extremities: Arthralgia and arthritis are common, primarily affecting the ankles and knees, although any joint may be involved. Periarticular inflammation is common.
• Neurologic: Headaches, seizures, and mononeuropathies rarely have been reported with HSP. Perform a careful neurologic examination for focal deficits.

Causes

Knowledge concerning the exact mechanisms by which the immune complexes implicated in the pathogenesis of Henoch-Schönlein purpura (HSP, or anaphylactoid purpura) are formed is lacking. Similarly, factors that predispose certain patients to development of the disease are poorly understood. The most common associated factor reported is an antecedent URI in approximately 50% of patients. Other reported associated factors are noted as follows:

• Infections
  • Bacteria - Group A beta hemolytic streptococci, Campylobacter jejuni, Yersinia species, Mycoplasma pneumoniae, and Helicobacter pylori (reported in one patient)
  • Viruses - Varicella, hepatitis B, Epstein-Barr virus, and parvovirus B19
• Drugs
  • Medication-associated HSP - More frequent in adults than children (although reported in both populations)
  • Drugs associated with HSP - Ampicillin penicillin, erythromycin, quinines, and chlorpromazine
• Neoplasms
  • Leukemia
  • Lymphoma
• Solid tumors
  • Ductal carcinoma of the breast
  • Bronchogenic carcinoma
  • Adenocarcinoma of the prostate
  • Adenocarcinoma of the colon
  • Renal cell carcinoma
  • Cervical carcinoma
  • Melanoma
• Foods - Sensitivity to foods containing salicylates and azo dyes
• Other - Pregnancy, familial Mediterranean fever, and cryoglobulinemia

Differential Diagnoses

Other Problems to Be Considered

Essential mixed cryoglobulinemia 
Microscopic polyangiitis
Polyarteritis nodosa 
Rheumatoid arthritis
Schamberg disease
Acute abdomen - Intussusception, bowel perforation, infarction
Child abuse
Bacterial endocarditis
Meningococcal septicemia 
Rocky Mountain spotted fever
Orchitis and testicular torsion
Diagnoses compatible with signs and symptoms of Henoch-Schönlein purpura (HSP) (may manifest at any point during disease course)

Workup

Laboratory Studies

• Urinalysis: Since renal failure and end-stage renal disease are the most serious long-term sequelae of this disease, initial and repeated urinalyses (UA) are crucial for appropriate monitoring of disease progression and resolution. Proteinuria and microscopic hematuria are the most common abnormalities on UA. Since renal involvement may follow the appearance of purpura for up to 3 months, perform UA monthly for several months after presentation.
• Serum electrolytes: Creatinine and blood urea nitrogen measurements indicate HSP-associated acute or chronic renal failure. Electrolyte imbalance may exist if significant diarrhea, GI bleeding, or hematemesis is seen.
• Antistreptolysin-O antibody titer: URIs with streptococcal species have been implicated as predisposing factors in as many as 50% of patients.
• Serum IgA level: The level often is elevated in HSP (anaphylactoid purpura), although this is not a specific test for the disease.
• Antinuclear antibody test: Systemic lupus erythematosus is in the differential diagnosis for HSP.
• Rheumatoid factor: IgA rheumatoid factor has been reported in patients with HSP. In addition, rheumatoid arthritis is in the differential diagnosis for patients presenting with significant joint complaints.
• CBC count with differential: Perform a CBC count to determine etiology when a suggestion of underlying infection exists (eg, bandemia with bacterial infection) and to exclude thrombocytopenia as a cause of purpura.
• Coagulation studies: Perform prothrombin time (PT) and partial thromboplastin time (aPTT) to exclude a bleeding diathesis.
• Liver function tests and hepatitis serologies: Hepatitis B has been reported in association with HSP.
• Direct immunofluorescence (DIF): Perform DIF for IgA on biopsy sections to demonstrate the predominance of IgA deposited in vessel walls of affected tissues. Perilesional skin adjacent to cutaneous lesions also may demonstrate IgA deposits. Renal biopsy specimens demonstrate mesangial IgA deposition in a granular pattern, often with C3, IgG, or IgM. This test is both sensitive and specific for HSP.
• Stool culture and guaiac: With appropriate history of GI complaints, a stool culture may be useful to search for Yersinia or Campylobacter species. Always search for occult GI bleeding when HSP is suspected.
• Serum markers of inflammation: ESR and CRP often are elevated in HSP.
• Renal biopsy: Renal insufficiency with nephrotic range proteinuria (>3.5 g protein/24 h) justifies renal biopsy. DIF for IgA reveals granular mesangial IgA deposition.

Imaging Studies

• Ultrasound: Ultrasound is indicated if abdominal pain is present to exclude intussusception, bowel wall edema, thickening, or perforation. This modality also is useful to evaluate acute testicular pain to exclude torsion.
• Chest radiograph: Chest radiograph excludes pulmonary nodules or hilar adenopathy suggestive of malignancy (primary or metastatic) or lymphoma, which have been associated with HSP (anaphylactoid purpura).

Procedures

• Skin biopsy: Biopsy early skin lesions for routine histology and DIF to confirm diagnosis or when diagnosis is in question.
• Renal biopsy: Perform renal biopsy when renal insufficiency with nephrotic range proteinuria is seen. Perform DIF for IgA deposits.
• Esophagogastroduodenoscopy: Upper GI endoscopy is indicated in patients with HSP when epigastric pain, melena, or hematemesis is present. Esophagogastroduodenoscopy (EGD) often demonstrates intensely red raised lesions, multiple ulcers, or diffuse erosive lesions. The duodenum frequently is involved.
• Colonoscopy: Colonoscopy is indicated when severe rectal bleeding exists. The appearance of the lesions is similar to that described for EGD.

Histologic Findings

Leukocytoclastic vasculitis is the predominant finding in affected tissues. Skin biopsy demonstrates fibrinoid necrosis of arteriolar and venular walls in the superficial dermis, with neutrophilic infiltration of the walls and perivascular regions. Associated fragments of inflammatory cells with nuclear debris are seen. Products of lysosomal enzyme digestion, as well as erythrocytes from hemorrhage, are extravasated. DIF shows IgA deposition in affected blood vessels. IgM, C3, and properdin also may be seen. Note that immune complex deposition occurs before chemotactants attract neutrophils, which then causes vascular injury. Once necrosis occurs, immune complexes disappear.

Mucosal biopsy from affected GI tissue shows histopathology identical to that seen in the skin. Biopsy of affected renal tissue shows a spectrum of glomerular disease from minimal change to severe crescentic glomerulonephritis. IgA, C3, fibrin, properdin and to some extent IgG and IgM, are seen as granular mesangial deposits on DIF. If severe disease is present, deposits also may be seen in the subendothelial and subepithelial spaces.

Treatment

Medical Care

Henoch-Schönlein purpura (HSP, or anaphylactoid purpura) is usually self-limited, and treatment is primarily supportive to ensure adequate hydration and replacement of excessive blood loss. Search for and treat underlying or predisposing factors. Controversies concerning the use of corticosteroids in the treatment of HSP exist with regard to whether or not they can (1) reduce severity or duration of disease, (2) decrease the risk of glomerulonephritis, and (3) increase the rate of relapses of the disease. An excellent editorial that reviews these issues has recently been published,⁶ and it is briefly summarized here.

Rosenblum and Winter⁷  reported more rapid improvement in abdominal pain (within 24 h) in 50% of patients when corticosteroids were initiated, compared with 14% in those not receiving corticosteroids. Niaudet and Habib,⁸  in a prospective uncontrolled study of 38 children with glomerulonephritis, found that corticosteroid use should be limited to patients at risk for progression of renal disease. Another study of 12 children with rapidly progressive crescentic glomerulonephritis treated with methylprednisolone followed by prednisone, cyclophosphamide, and dipyridamole showed that most had normal renal function at the end of 3 months. A similar study of 14 patients treated with prednisolone, cyclophosphamide, heparin, and dipyridamole showed that 9 had normal renal function after an average of 7.5 years of follow-up.

To address the issue of whether corticosteroid use can prevent glomerulonephritis in patients with HSP, 168 children were randomized to receive prednisone or no steroids. Zero of 84 patients treated with prednisone developed nephropathy, whereas 12 of 84 from the control group developed nephropathy.

A randomized placebo-controlled study of 40 children with HSP was conducted in which 21 received prednisone within 7 days of disease onset and 19 received placebo. Early treatment did not reduce the risk of renal or GI involvement.

The issue of whether corticosteroids can increase the rate of disease relapse has recently been addressed. In an Italian cohort of 150 children reported by Trapani et al,⁹  disease relapse was significantly more frequent in those treated with corticosteroids. However, this may have been due to "confounding by indication," which means that it may have been the fact that these patients had more severe disease that led to their tendency to relapse, rather than treatment with corticosteroids (which were initiated because of the more severe disease).
The presence of nephrotic-range proteinuria and hematuria are associated with a 15% risk of renal failure, and the presence of more than 50% glomerular crescents in renal biopsy specimens is associated with 50% renal failure over a 10-year period. Because of the potentially severe sequelae in these patients, consider initiating corticosteroid therapy when nephrotic-range proteinuria with hematuria is seen or when biopsy results demonstrate glomerular crescents.

Therefore, in summary, it appears that if the patient is subject to glomerulonephritis, systemic steroids would be in order to protect the kidneys even though the occurrence of relapses may be more frequent. In contrast, if the patient has gastrointestinal pathology associated with his or her HSP, systemic steroids are not helpful and should be avoided.

Dapsone, azathioprine, and intravenous immunoglobulin therapies have been tried with varying success, as has plasmapheresis.

Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to treat arthralgia associated with HSP. Oral corticosteroids may be of benefit in treating painful subcutaneous edema.

Iwamoto et al reported successful treatment of HSP using pulse steroid therapy and tonsillectomy.¹⁰ Additionally, Donnithorne et al reported a series of 3 patients with refractory HSP successfully treated with 1-2 course of rituximab.¹¹

Consultations

• Dermatologist - For diagnosis when a cutaneous eruption is the presenting symptom of HSP (anaphylactoid purpura)
• Gastroenterologist - For possible EGD or colonoscopy when hematemesis, melena, or hematochezia is present
• Surgeon - For evaluation when abdominal pain is the presenting symptom and severity mimics that of an acute abdomen
• Nephrologist - For evaluation, diagnosis, and management of the renal complications of HSP

Medication

The goals of pharmacotherapy are to reduce morbidity and prevent complications.

Corticosteroids

Use for possible early treatment of abdominal pain and GI bleeding associated with Henoch-Schönlein purpura (HSP, or anaphylactoid purpura). Also use for possible prevention of delayed-onset HSP nephritis or in patients affected with nephritis as evidenced by nephrotic-range proteinuria or renal biopsy showing glomerular crescents.

Methylprednisolone (Solu-Medrol, Depo-Medrol)

Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability. Steroids ameliorate delayed effects of anaphylactoid reactions and may limit biphasic anaphylaxis.

Dosing

Adult

40 mg IV qd

Pediatric

1-2 mg/kg IV qd

Interactions

Coadministration with cyclosporine may exacerbate adverse effects normally associated with either drug alone; phenobarbital, phenytoin, and rifampin may increase clearance; ketoconazole and estrogens may decrease clearance; methylprednisolone may increase clearance of aspirin; steroid-induced hypokalemia may increase digitalis toxicity

Contraindications

Documented hypersensitivity; viral, fungal, or tubercular skin infections; premature infants

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

May mask signs of infection or acute abdomen; hyperglycemia, edema, osteonecrosis, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, myopathy, and infections are possible complications

Prednisone (Deltasone)

May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Dosing

Adult

40 mg PO qd

Pediatric

1-2 mg/kg PO qd

Interactions

Coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics

Contraindications

Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur

Nonsteroidal anti-inflammatory drugs

Use to treat the symptoms of arthralgia or arthritis associated with HSP.

Ibuprofen (Ibuprin, Advil, Motrin)

DOC for mild to moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.

Dosing

Adult

400-600 mg PO q6h

Pediatric

30-70 mg/kg/d PO divided tid/qid

Interactions

May increase levels of anticoagulants, cyclosporine, dipyridamole, hydantoins, lithium, methotrexate, penicillamine, and sympathomimetics; may decrease levels of ACE inhibitors, beta blockers, loop diuretics, and thiazide diuretics; salicylates may decrease NSAID levels; probenecid may increase NSAID levels

Contraindications

Documented hypersensitivity; hypersensitivity to other NSAIDs, aspirin, or iodides; patients with asthma, urticaria, or angioedema; active ulceration or inflammation of upper or lower GI tract; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding

Precautions

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Category D in third trimester of pregnancy (use during third trimester of pregnancy can increase risk of patent ductus arteriosus and other cardiac abnormalities); can prolong or cease labor; may cause GI irritation with ulceration and bleeding; inhibits platelet aggregation and prolongs bleeding time by 3-4 min; caution in congestive heart failure, hypertension, and decreased renal and hepatic function; caution in coagulation abnormalities or during anticoagulant therapy

Follow-up

Complications

• Renal involvement, chronic renal failure

Prognosis

• Prognosis for Henoch-Schönlein purpura (HSP, or anaphylactoid purpura) is excellent, except when progressive renal failure and end-stage renal disease develop. Perform monthly UA both in patients who have signs or symptoms of nephritis (to monitor progression versus resolution) and in patients who have no evidence of nephritis (may develop signs of renal involvement months after initial presentation).
• The syndrome resolves after 4-6 weeks in most patients, although as many as 50% have a recurrence.

Patient Education

• For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Blood in the Urine.

Miscellaneous

Special Concerns

• Exclude underlying diseases.

Multimedia

Media file 1: Purpuric papules and plaques of the lower extremity characteristic of Henoch-Schönlein purpura.

Media file 2: Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein purpura.

Media file 3: Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation with ecchymoses on the dorsal foot and ankle.

References

1. Helander SD, De Castro FR, Gibson LE. Henoch-Schonlein purpura: clinicopathologic correlation of cutaneous vascular IgA deposits and the relationship to leukocytoclastic vasculitis. Acta Derm Venereol. Mar 1995;75(2):125-9. [Medline].

2. Michel BA, Hunder GG, Bloch DA, Calabrese LH. Hypersensitivity vasculitis and Henoch-Schonlein purpura: a comparison between the 2 disorders. J Rheumatol. May 1992;19(5):721-8. [Medline].

3. Paller AS, Kelly K, Sethi R. Pulmonary hemorrhage: an often fatal complication of Henoch-Schoenlein purpura. Pediatr Dermatol. Jul-Aug 1997;14(4):299-302. [Medline].

4. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications. AMA J Dis Child. Jun 1960;99:833-54. [Medline].

5. Gunasekaran TS. Henoch-Schonlein purpura: what does the "rash" look like in the gastrointestinal mucosa?. Pediatr Dermatol. Nov-Dec 1997;14(6):437-40. [Medline].

6. Gonzalez-Gay MA, Llorca J. Controversies on the use of corticosteroid therapy in children with Henoch-Schönlein purpura. Semin Arthritis Rheum. Dec 2005;35(3):135-7. [Medline].

7. Rosenblum ND, Winter HS. Steroid effects on the course of abdominal pain in children with Henoch-Schonlein purpura. Pediatrics. Jun 1987;79(6):1018-21. [Medline].

8. Niaudet P, Habib R. Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis. Pediatr Nephrol. Apr 1998;12(3):238-43. [Medline].

9. Trapani S, Micheli A, Grisolia F, et al. Henoch Schonlein purpura in childhood: epidemiological and clinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. Dec 2005;35(3):143-53. [Medline].

10. Iwamoto M, Wakabayashi M, Hanada S, Kobayashi N, Hata T, Ando R. [A case of Henoch-Schonlein purpura nephritis successfully treated with tonsillectomy and steroid pulse therapy]. Nippon Jinzo Gakkai Shi. 2009;51(4):484-9. [Medline].

11. Donnithorne KJ, Atkinson TP, Hinze CH, et al. Rituximab therapy for severe refractory chronic Henoch-Schonlein purpura. J Pediatr. Jul 2009;155(1):136-9. [Medline].

12. Amitai Y, Gillis D, Wasserman D, Kochman RH. Henoch-Schonlein purpura in infants. Pediatrics. Dec 1993;92(6):865-7. [Medline].

13. Causey AL, Woodall BN, Wahl NG, Voelker CL, Pollack ES. Henoch-Schonlein purpura: four cases and a review. J Emerg Med. May-Jun 1994;12(3):331-41. [Medline].

14. Crosby DL, Feldman SD. A pruritic vesicular eruption. Henoch-Schonlein purpura. Arch Dermatol. Nov 1990;126(11):1497-8, 1500. [Medline].

15. Cuttica RJ. Vasculitis in children: a diagnostic challenge. Curr Probl Pediatr. Sep 1997;27(8):309-18. [Medline].

16. Dillon MJ, Ansell BM. Vasculitis in children and adolescents. Rheum Dis Clin North Am. Nov 1995;21(4):1115-36. [Medline].

17. Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. Nov 1995;21(4):1097-113. [Medline].

18. Kraft DM, Mckee D, Scott C. Henoch-Schonlein purpura: a review. Am Fam Physician. Aug 1998;58(2):405-8, 411. [Medline].

19. Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. Nov 1995;21(4):883-909. [Medline].

20. Maestri A, Malacarne P, Santini A. Henoch-Schonlein syndrome associated with breast cancer. A case report. Angiology. Jul 1995;46(7):625-7. [Medline].

21. Patrignelli R, Sheikh SH, Shaw-Stiffel TA. Henoch-Schonlein purpura. A multisystem disease also seen in adults. Postgrad Med. May 1995;97(5):123-4, 127, 131-4. [Medline].

22. Piette WW. What is Schonlein-Henoch purpura, and why should we care?. Arch Dermatol. Apr 1997;133(4):515-8. [Medline].

23. Piette WW, Stone MS. A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schönlein purpura). Arch Dermatol. Jan 1989;125(1):53-6. [Medline].

24. Stevens GL, Adelman HM, Wallach PM. Palpable purpura: an algorithmic approach. Am Fam Physician. Oct 1995;52(5):1355-62. [Medline].

25. Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].

26. Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, Flageul B, Morel P, Rybojad M. Schönlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. Apr 1997;133(4):438-42. [Medline].

27. Tapson KM. Henoch-Schonlein purpura. Am Fam Physician. Feb 15 1993;47(3):633-8. [Medline].

28. Trujillo H, Gunasekaran TS, Eisenberg GM, Pojman D, Kallen R. Henoch-Schonlein purpura: a diagnosis not to be forgotten. J Fam Pract. Nov 1996;43(5):495-8. [Medline].

29. Uthman I, Kassak K, Nasr FW. Henoch-Schonlein purpura in adulthood and childhood: comment on the article by Blanco et al. Arthritis Rheum. Aug 1998;41(8):1518-20. [Medline].

30. Wananukul S, Pongprasit P, Korkij W. Henoch-Schonlein purpura presenting as hemorrhagic vesicles and bullae: case report and literature review. Pediatr Dermatol. Dec 1995;12(4):314-7. [Medline].

31. White RH. Henoch-Schonlein nephritis. A disease with significant late sequelae. Nephron. 1994;68(1):1-9. [Medline].

Keywords

Henoch-Schönlein purpura, Henoch-Schonlein purpura, anaphylactoid purpura, Schonlein-Henoch purpura, Schönlein-Henoch purpura, Henoch-Schoenlein purpura, vasculitis, HSP, immunoglobulin A, IgA-mediated disease, upper respiratory tract infection tract

Contributor Information and Disclosures

Author

Andrew D Montemarano, DO, Consulting Staff, The Skin Cancer Surgery Center
Andrew D Montemarano, DO is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Medical Editor

David Woodley, MD, Co-Chair, Professor, Department of Medicine, Division of Dermatology, University of Southern California
David Woodley, MD is a member of the following medical societies: American Academy of Dermatology, American Association for the Advancement of Science, American College of Emergency Physicians, American College of Physicians, American Federation for Medical Research, American Society for Clinical Investigation, New York Academy of Medicine, Society for Investigative Dermatology, and Southern Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Robert J Willard, MD, and previous Chief Editor, William D. James, MD, to the development and writing of this article.

© 1994- by Medscape.
All Rights Reserved
(http://www.medscape.com/public/copyright)