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Rheumatology > Vasculitis
Leukocytoclastic Vasculitis
Article Last Updated: Feb 14, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Editors: Bryan L Martin, DO, Chief, Allergy Immunology Department, Walter Reed Army Medical Center, Washington, DC; Associate Professor of Medicine and Pediatrics, Uniformed Services University of the Health Sciences, Bethesda, MD; United States Army Consultant in Allergy Immunology and Immunizations; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Author and Editor Disclosure
Synonyms and related keywords:
leukocytoclastic vasculitis, LCV, hypersensitivity vasculitis, allergic angiitis, small vessel vasculitis, small-vessel vasculitis, Henoch-Schönlein purpura, Henoch-Schönlein purpura, serum sickness, serum sickness syndrome, urticarial vasculitis, upper respiratory tract infections, beta-hemolytic streptococci, viral hepatitis, HIV, bacterial endocarditis, hepatitis C, hepatitis B, collagen vascular disease, rheumatoid arthritis, Sjögren syndrome, lupus erythematosus, inflammatory bowel disease, ulcerative colitis, Crohn disease, hairy cell leukemia, cutaneous vasculitis, Wegener granulomatosis, polyarteritis nodosa, microscopic polyarteritis, Churg-Strauss syndrome, erythema elevatum diutinum
Background
Leukocytoclastic vasculitis (LCV) is a histopathologic term commonly used to denote a small-vessel vasculitis. Many possible causes exist for this condition, but a cause is not found in as many as 50% of patients.
The disorder may be localized to the skin, or it may manifest in other organs. The internal organs affected most commonly include the joints, the gastrointestinal tract, and the kidneys. When internal involvement is not present, the prognosis is good.
The disorder may be acute or chronic. Chronic disease that primarily involves the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids, immunosuppressive agents, or both.
Pathophysiology
In the past, circulating immune complexes were believed to be the cause of LCV. Although immune complexes are involved in the pathogenesis of LCV, other autoantibodies such as antineutrophil cytoplasmic antibody (ANCA), other inflammatory mediators, and local factors that involve the endothelial cells and adhesion molecules play an important role. The exact mechanisms remain unknown.
Frequency
United States
The incidence of LCV is not known, but the disorder is presumed to be uncommon.
International
Several studies from Spain have been conducted. Hypersensitivity vasculitis occurs in 10-30 persons per million persons per year. Fourteen cases of Henoch-Schönlein purpura per million persons per year have been reported.
Mortality/Morbidity
- The prognosis of LCV generally is good, but if the kidneys, gastrointestinal tract, lungs, heart, or central nervous system is involved, mortality is possible.
- Chronic cutaneous disease may involve ulceration or painful bouts of purpura. Some patients alter their lives because of recurrent purpuric eruptions.
Race
- LCV appears to be reported more often in the white population than in other races.
Sex
- LCV affects men and women in approximately equal proportions. Some of the studies from Spain suggest that LCV may be slightly more common in men than in women.
Age
- LCV may occur at any age.
- In children, it may be called Henoch-Schönlein purpura.
History
- Patients with vasculitis of their skin may experience itching, burning, or pain, or they may have asymptomatic lesions.
- Vasculitis of the skin may occur in the absence of any systemic disease.
- It may manifest as an eruption only, or it may occur in conjunction with collagen vascular disorders, paraproteinemia, certain foods, oral or parenteral medications, infections, or, rarely, malignancy.
- The physician should elicit information from the patient about possible systemic manifestations. Questions should determine the presence or absence of fever, arthralgia, arthritis, myalgia, abdominal pain, diarrhea, hematochezia, cough, hemoptysis, sinusitis, paresthesia, weakness, and hematuria.
- Information should be obtained about symptoms of an associated disorder. Questions should determine the history of intravenous drug use, history of hepatitis, history of a transfusion, travel history, symptoms or history of inflammatory bowel disease, and history or symptoms of a collagen vascular disorder, particularly rheumatoid arthritis, lupus erythematosus, or Sjögren syndrome.
Physical
- The most common manifestation of cutaneous vasculitis is palpable purpura, but other manifestations may be present.
- Palpable purpura is the most frequent presentation of small-vessel vasculitis.
- Lesions usually are round and 1-3 mm.
- They may coalesce to form plaques, and in some instances, they may ulcerate.
- Palpable purpura is observed most often on the legs, but any surface can be involved. Sometimes, the purpuric lesions are barely palpable.
- Urticarial lesions may occur in some patients; rarely, this type of lesion predates purpuric lesions.
- The urticarial lesions are of a different character than routine urticaria. They tend to be of longer duration, often longer than 24 hours, and tend to resolve with some residual pigmentation or ecchymosis. Patients experience more burning rather than itching.
- To determine the duration of individual lesions, the examiner may encircle several lesions and ask the patient to periodically observe them and note when they resolve or move to another site.
- Patients with hypocomplementemic urticarial vasculitis may develop chronic obstructive pulmonary disease, and a careful examination of the heart and lungs is warranted.
- Livedo reticularis is a rare manifestation of small-vessel vasculitis. It is more common in patients with occlusive or inflammatory disease of medium-sized vessels.
- Nodular lesions may occur in some patients with small-vessel vasculitis.
- Ulceration is more common in vasculitis that affects larger vessels, but it may complicate intense purpura.
- A careful physical examination is warranted in patients with vasculitis and should include specific observation of the cardiopulmonary, musculoskeletal, and gastrointestinal systems.
- Retiform purpura has been described by Piette and Stone and has been linked to immunoglobulin A (IgA)–associated disease.1
Causes
- Between one third and one half of the cases of cutaneous vasculitis are idiopathic, and the remainder have various causes.
- The most common drugs that can cause cutaneous vasculitis are antibiotics, particularly beta-lactam drugs, nonsteroidal anti-inflammatory drugs, and diuretics. However, almost all drugs are potential causes. Foreign proteins such as streptokinase, those found in vaccines, and those used in monoclonal antibody therapy can be associated with a serum sickness syndrome with leukocytoclastic vasculitis (LCV).
- Various infections may be associated with vasculitis.
- Upper respiratory tract infections, particularly with beta-hemolytic streptococci, and viral hepatitis are implicated most often.
- HIV infection also may be associated with some cases of cutaneous vasculitis.
- LCV also may be seen with bacterial endocarditis.
- Occasionally, ascertaining whether a drug (eg, antibiotic) or an infection (eg, upper respiratory infection) is responsible for the disease is impossible because the occurrence of the vasculitis postdates the infection and the drug used to treat the infection.
- Foods or food additives may cause vasculitis.
- Hepatitis C is a regularly recognized cause of vasculitis, probably through the presence of cryoglobulins; however, in the past, hepatitis B was implicated in some cases of vasculitis.
- Collagen vascular diseases account for 10-15% of vasculitis cases.
- In particular, rheumatoid arthritis, Sjögren syndrome, and lupus erythematosus may have an associated vasculitis.
- Often, the presence of vasculitis denotes active disease.
- Inflammatory bowel disease, ulcerative colitis, or Crohn disease may be associated with cutaneous vasculitis.
- Malignancy accounts for less than 1% of cases of cutaneous vasculitis.
- Perhaps lymphoproliferative diseases are more common, particularly hairy cell leukemia; however, any type of tumor at any site may possibly be related to cutaneous vasculitis.
- Effective tumor therapy has led to an apparent cure of the vasculitis in some patients.
- Cutaneous vasculitis may be part of a larger-vessel vasculitis such as Wegener granulomatosis, polyarteritis nodosa, microscopic polyarteritis, or Churg-Strauss syndrome.
Amyloidosis, AA (Inflammatory)
Antiphospholipid Syndrome
Atrial Myxoma
Behcet Disease
Immune Thrombocytopenic Purpura
Meningococcemia
Scurvy
Wegener Granulomatosis
Other Problems to be Considered
Hypersensitivity vasculitis
Idiopathic thrombocytopenia purpura
Polyangiitis overlap syndrome
Thrombocytopenia
Lab Studies
- The evaluation of the patient with vasculitis serves 2 purposes. The first is determining the presence of systemic disease. The second is identifying an associated disorder, which aids in the prediction of the patient's prognosis.
- Although no routine has been established, testing for all adult patients includes complete blood cell count, erythrocyte sedimentation rate, urinalysis, and blood chemistry panel.
- Although stool guaiac testing and Hematest are not reliable, such tests should be obtained in patients with cutaneous vasculitis.
- Serologic studies, such as antinuclear antibody, ANCA (cytoplasmic ANCA [cANCA], perinuclear ANCA [pANCA], atypical ANCA), and rheumatoid factor, should be obtained in patients without an obvious cause of their disease.
- In patients suggested to have lupus erythematosus or patients who have urticarial vasculitis, complement levels may be obtained, including total hemolytic complement (CH100 or CH50), C3 levels, and C4 levels.
- In patients without otherwise identified disease, tests for paraproteins should include serum protein electrophoresis, cryoglobulins, and hepatitis C antibody.
- Hepatitis B was associated with vasculitis in the past; however, it appears that the association may have occurred by virtue of co-infection with hepatitis C (previously termed non-A/non-B hepatitis).
- Cryoglobulins may be present, especially in association with infections (hepatitis C, bacterial endocarditis). A positive result for rheumatoid factor often occurs in patients with cryoglobulinemia.
- Patients with fever and/or a heart murmur should undergo cardiac ultrasonography and blood cultures.
- HIV testing should be performed in patients at high risk and in patients with an otherwise unidentifiable cause of their disease.
- If the peripheral smear result is abnormal, then obtaining bone marrow may be useful.
Imaging Studies
- Chest radiograph is part of the routine evaluation.
- Visceral angiography may be requested in patients with a severe vasculitic syndrome.
Other Tests
- Pulmonary function tests should be obtained in patients with hypocomplementemic urticarial vasculitis.
Procedures
- A skin biopsy of a relatively fresh lesion should be performed in most, if not all, adult patients. Children suggested to have vasculitis often are not forced to endure biopsy for humanitarian reasons.
- Muscle or nerve biopsy or biopsy of visceral organs may be requested in patients with severe vasculitic syndromes; however, most patients with leukocytoclastic vasculitis (LCV) of the skin do not require such tests.
- Direct immunofluorescence microscopy may be obtained in selected patients. IgA is present in Henoch-Schönlein purpura.
Histologic Findings
Skin biopsy reveals the presence of vascular and perivascular infiltration of polymorphonuclear leukocytes with formation of nuclear dust (leukocytoclasis), extravasation of erythrocytes, and fibrinoid necrosis of the vessel walls. This process is dynamic, and a biopsy of a lesion taken too early or too late in its evolution may not reveal these findings. The presence of eosinophils has been correlated with drug-associated disease.
The picture of LCV is a pattern that can occur in any vasculitic syndrome but also may occur in nonvasculitic diseases such as neutrophilic dermatoses, at the base of leg ulceration, or in some insect bite reactions. Careful clinical-pathologic correlation is necessary.
Immunofluorescent staining may reveal immunoglobulins (eg, immunoglobulin G, immunoglobulin M) and complement components (eg, C3, C4) deposited on the skin basement membrane, which are suggestive of immune complex deposition. In Henoch-Schönlein purpura, IgA deposits may be found.
Medical Care
- Once a diagnosis of cutaneous small-vessel vasculitis is established and the patient is fully evaluated, specific or nonspecific management options may be used.
- The disease often affects dependent areas; thus, elevation of the legs or compression stockings may be useful.
- Patients with an identifiable cause should receive treatment for that cause. Removal of a drug thought to be causing the vasculitis may result in rapid clearing of the process in as short as 2 weeks.
- For patients with disease of the skin, with or without joint manifestations, colchicine or dapsone may be administered.
- Patients with urticarial lesions may be treated with antihistamines, including both soporific agents and less-sedating agents.
- Sometimes, a combination of these agents is needed to control the disease manifestations.
- Some patients respond to nonsteroidal anti-inflammatory agents.
- Patients with severe visceral involvement may require high doses of corticosteroids (1-2 mg/kg/d) with or without an immunosuppressive agent (eg, cyclophosphamide, azathioprine, methotrexate).
- Patients with chronic cutaneous vasculitis may be put on a restrictive diet, possibly leading to identification of the cause and control of the disease.
- Patients with either severe or debilitating disease might also be treated with biologic agents such as rituximab or intravenous immune globulin.
Surgical Care
- Surgical care rarely is needed for patients with vasculitis.
- Surgical care may be appropriate if a tumor is identified as a cause of the process.
- Surgical care also may be appropriate if a recalcitrant ulceration is present after control of the active disease has been achieved.
- Surgery may be needed for appropriate organ biopsy.
Consultations
- Rheumatologist
- Dermatologist
- Nephrologist
- Gastroenterologist or hepatologist
- Immunologist or allergist
- Pulmonologist
Diet
- No specific diet is required.
- A restrictive diet may be used for as long as 2 weeks for diagnostic and therapeutic purposes.
Activity
- No specific restrictions on activity are necessary.
No effective therapy has been established for all patients. A few of the therapies have been tested in controlled trials.
Drug Category: Anti-inflammatory agents
Inhibit key events involved in the inflammatory process.
| Drug Name | Colchicine |
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| Description | Has effects against neutrophils, which probably are involved in the pathogenesis of cutaneous vasculitis. Has been demonstrated to be steroid sparing in open-label studies. The only double-blind, placebo-controlled trial failed to demonstrate efficacy; however, the study had several methodological errors. |
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| Adult Dose | 0.5-0.6 mg PO bid/tid |
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| Pediatric Dose | 0.5 mg PO bid |
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| Contraindications | Documented hypersensitivity; severe renal, hepatic, GI, or cardiac disorders; blood dyscrasias |
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| Interactions | Sympathomimetic agent toxicity and effect of CNS depressants are significantly increased |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Pregnancy category D when used parenterally; risk of renal failure, hepatic failure, permanent hair loss, bone marrow suppression, numbness or tingling in hands and feet, disseminated intravascular coagulopathy, and decreased sperm count may occur; dose-dependent GI upset is common |
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Drug Category: Antibiotics
Some types of antimicrobials (eg, dapsone) have anti-inflammatory properties.
| Drug Name | Dapsone (Avlosulfon) |
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| Description | Small, open-label studies or single case reports have suggested that dapsone is effective in some patients with cutaneous vasculitis. |
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| Adult Dose | 150-200 mg PO qd |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; G-6-PD deficiency |
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| Interactions | May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during the second and third mo of therapy); probenecid increases toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased in renal clearance, levels may significantly decrease when administered concurrently with rifampin |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Perform weekly blood counts (first mo), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets, WBC, or RBC counts is seen; caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M due to high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light |
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Drug Category: Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Prednisone (Deltasone, Orasone, Meticorten) |
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| Description | Indicated for vasculitis affecting internal organs such as kidneys, lungs, or CNS. Skin involvement resulting in ulceration may require corticosteroid therapy. |
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| Adult Dose | 1 mg/kg/d PO; can be administered IV in severe cases |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | No absolute contraindication; severe bacterial, viral, or fungal infection; peptic ulcer disease; diabetes mellitus |
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| Interactions | Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
|
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| Precautions | Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, avascular necrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, growth suppression, and infections may occur |
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Drug Category: Cytotoxic/immunosuppressive agents
Inhibit cell growth and proliferation, causing a decrease in immune reactions.
| Drug Name | Cyclophosphamide (Cytoxan, Neosar) |
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| Description | Useful in life-threatening cases of vasculitis. Patients with only skin disease generally should not be treated with this agent. Useful in patients with polyarteritis nodosa, Wegener granulomatosis, and Churg-Strauss syndrome. Alkylating agent that depresses T-cell and B-cell function. |
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| Adult Dose | 1-2 mg/kg/d PO; alternatively, also may be given in pulsed doses of 500-1000 mg/m2 IV monthly
Dose adjusted according to clinical response, hematologic response, and toxicity |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; active infection, severely depressed bone marrow function, severe cytopenias |
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| Interactions | Allopurinol may increase risk of bleeding or infection and may enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia, and neuromuscular effects of succinylcholine may be potentiated by IV cyclophosphamide |
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| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
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| Precautions | Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; nausea, vomiting, infertility, teratogenicity, and risk of malignancy |
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| Drug Name | Azathioprine (Imuran) |
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| Description | Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. |
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| Adult Dose | 2-3 mg/kg/d PO |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; low levels of serum thiopurine methyl transferase (TPMT); active infection and severe cytopenias |
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| Interactions | Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine |
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| Pregnancy | B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
|
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| Precautions | Nausea, vomiting, leukopenia, thrombocytopenia, anemia, infection, and abnormal liver function test results may occur |
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| Drug Name | Methotrexate (Rheumatrex, Folex PFS) |
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| Description | Unknown mechanism of action in treatment of inflammatory reactions; may affect immune function. Ameliorates symptoms of inflammation (eg, pain, swelling, stiffness). Adjust dose gradually to attain satisfactory response. |
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| Adult Dose | 10-25 mg PO/SC qwk |
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| Pediatric Dose | 10-25 mg PO/SC qwk |
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| Contraindications | Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency |
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| Interactions | Oral aminoglycosides may decrease absorption and blood levels of concurrent oral methotrexate; charcoal lowers levels; coadministration with etretinate may increase hepatotoxicity; indomethacin and phenylbutazone can increase plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides (including TMP-SMZ) may increase effects and toxicity; may increase plasma levels of thiopurines |
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| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
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| Precautions | Monitor CBC counts monthly and liver and renal function every 1-3 mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated levels, eg, dehydration); has toxic effects on hematologic, renal, GI, pulmonary, and neurologic systems; discontinue if significant drop in blood counts occurs; aspirin, NSAIDs, or low-dose steroids may be administered concomitantly (possibility of increased toxicity with NSAIDs including salicylates has not been tested); oral folic acid therapy (1 mg/d) reduces adverse effects (especially GI) without reducing effectiveness |
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| Drug Name | Mycophenolate (CellCept) |
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| Description | Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
Two formulations are available and are not interchangeable. The original formulation, mycophenolate mofetil (MMF, CellCept) is a prodrug that, once hydrolyzed in vivo, releases the active moiety mycophenolic acid. A newer formulation, mycophenolic acid (MPA, Myfortic) is an enteric-coated product that delivers the active moiety. |
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| Adult Dose | Mycophenolate mofetil:
Renal transplantation: 1 g PO/IV q12h
Hepatic transplantation:
PO: 1.5 g PO q12h
IV: 1 g IV q12h infused over at least 2 h
Mycophenolic acid:
Renal transplantation: 720 mg PO bid; administer on empty stomach 1 h before or 2 h pc |
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| Pediatric Dose | Mycophenolate mofetil:
Renal transplantation: (Note: dose based on body surface area [BSA])
Oral susp: 600 mg/m2 PO q12h; not to exceed 2 g/d (ie, 10 mL/d)
Capsules:
BSA 1.25-1.5 m2: 750 mg PO q12h
BSA >1.5 m2: Administer as in adults
Mycophenolic acid:
Renal transplantation: Not established; limited data suggest 400 mg/m2 PO bid; not to exceed 720 mg bid
Note: Patients with BSA <1.19 m2 cannot be accurately administered Myfortic tab |
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| Contraindications | Documented hypersensitivity |
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| Interactions | In combination with either acyclovir or ganciclovir, may result in higher levels for both interacting drugs because of competition for renal tubular excretion;
aluminum/magnesium present in some antacids and cholestyramine-containing products may decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates and azathioprine may increase toxicity; may decrease levonorgestrel AUC; may decrease live-virus vaccine immune response; when administered in combination with theophylline, may increase free fraction levels of theophylline |
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| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
|
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| Precautions | Increases risk for infection (monitor blood count); severe renal impairment (CrCl <25 mL/min) may have increased adverse effects due to increase free MPA; caution in active peptic ulcer disease; incidence of malignancies and lymphoma consistent with that reported for other immunosuppressants (0.9%); commonly causes constipation, nausea, diarrhea, urinary tract infection, and nasopharyngitis; rare reports include interstitial lung disorders, colitis, pancreatitis, intestinal perforation, GI hemorrhage, gastric ulcers, duodenal ulcers, and ileus; do not chew, crush, or cut Myfortic tab |
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| Drug Name | Rituximab (Rituxan) |
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| Description | Antibody genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of normal and malignant B lymphocytes. Antibody is an IgG1-kappa immunoglobulin that contains murine light- and heavy-chain variable region sequences and human constant region sequences. |
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| Adult Dose | 375 mg/m2 IV qwk for 4 doses (days 1, 8, 15, and 22) |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Hypotension, bronchospasm, and angioedema may occur; discontinue treatment if life-threatening cardiac arrhythmias occur |
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Further Inpatient Care
- Inpatient care is needed for patients who have severe vasculitic syndromes and severe organ dysfunction.
- Most patients with cutaneous vasculitis are treated in an outpatient setting.
Further Outpatient Care
- The design of a follow-up program depends on the vasculitic syndrome, its chronicity, and the organ systems affected.
- Once the process is inactive in a patient with hypersensitivity vasculitis, further follow-up care may not be needed.
- Patients with Henoch-Schönlein purpura may develop impairment of their renal function or hypertension; therefore, regular follow-up care, even after complete clearing of their disease, is needed.
In/Out Patient Meds
- Treating patients with chronic cutaneous vasculitis is a challenge.
- In the absence of an identifiable cause, dietary restriction may be attempted but usually is unsuccessful.
- Colchicine at 0.6 mg twice daily and/or dapsone at 100-200 mg/d may control the disease. Combination of these two agents seems to be complementary.
- In patients whose conditions do not respond or respond poorly, other agents, including immunosuppressive or cytotoxic agents, may be administered.
- Biologic therapies such as intravenous immune globulin and rituximab are useful in some patients.
Transfer
- Transfer to a tertiary care facility should be considered for patients with severe visceral disease.
- Patients with chronic cutaneous disease often are referred to a tertiary care center for specialty care.
Complications
- Vasculitis may be complicated by ulceration of the skin or by end-organ dysfunction.
Prognosis
- The prognosis of patients with cutaneous vasculitis depends on the underlying syndrome or the presence of end-organ dysfunction.
- Patients with disease that primarily affects the skin, joints, or both have a good prognosis.
- Patients with Wegener granulomatosis, polyarteritis nodosa, Churg-Strauss syndrome, or severe necrotizing vasculitis have a potentially fatal disease. Treatment with corticosteroids and/or immunosuppressive or cytotoxic agents often is lifesaving.
Medical/Legal Pitfalls
- Failure to accurately diagnosis a patient, eg, with vasculitis when the patient has a nonvasculitic disorder (eg, insect bite reaction)
- Failure to recognize serious systemic disease
- Failure to provide proper treatment (eg, overaggressive treatment of a patient with chronic but mild disease or undertreatment of a patient with severe systemic disease)
- Failure to inform the patient of the risks associated with therapy (eg, corticosteroid-induced avascular necrosis)
- Failure to recognize an infectious cause (eg, hepatitis C, bacterial endocarditis)
| Media file 4:
Urticarial vasculitis. Lesions differ from routine hives in that they last longer (often >24 h), are less pruritic, and often resolve with a bruise or residual pigmentation. |
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Media type: Photo
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| Media file 5:
Erythema elevatum diutinum, a rare cutaneous vasculitis. |
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Media type:
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Leukocytoclastic Vasculitis excerpt Article Last Updated: Feb 14, 2008
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