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Presentation

History

Patients with leukocytoclastic vasculitis (LCV) may experience itching, burning, or pain, or they may have asymptomatic lesions. See the image below.

Erythema elevatum diutinum, a rare cutaneous vasculitis.

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Leukoctyoclastic vasculitis may manifest as a cutaneous eruption only, or it may occur in conjunction with any of the following:

Collagen-vascular disorders
Paraproteinemia
Certain foods
Oral or parenteral medications
Infections
Malignancy

A thorough history with complete review of systems should be obtained, with specific evaluation for the following:

Preceding fever
Arthralgias
Arthritis
Myalgias
Abdominal pain
Diarrhea
Melena
Hematochezia
Cough
Hemoptysis
Sinusitis
Parasthesias
Weakness
Hematuria

Additional questions should be asked regarding new prescription, over-the-counter, and herbal medications, as well as dose changes in pre-existing medications. Consideration should also be given for an underlying disorder, such as collagen vascular disease, including rheumatoid arthritis, systemic and cutaneous lupus erythematosus, and Sjögren syndrome, hepatitis, inflammatory bowel disease, and history of blood transfusion. A thorough social history should be obtained regarding recent travel, new environmental exposures, and illicit drug use.

Physical

The most common manifestation of leukocytoclastic vasculitis (LCV) is palpable purpura, but other manifestations may develop. Features of palpable purpura in LCV are as follows:

Lesions are usually round and 1-3 mm
They may coalesce to form plaques; in some instances, they may form bullae and ulcerate
Palpable purpura is most common on dependent areas, such as the lower legs; areas of tight-fitting clothing; and areas of trauma (Koebner phenomenon ^[30]); however, any surface can be involved
In some cases, the purpuric lesions are barely palpable

Urticarial lesions may develop in some patients with LCV. In rare cases, this type of lesion predates the purpuric lesions.

The urticarial lesions associated with LCV differ from those of classic urticaria. The following three characteristics of these lesions, although not always present in every patient, are typical of urticarial vasculitis:

Longer duration (often >24 h)
Residual pigmentation or ecchymosis after resolution (see the image below)
Patients experience more burning than itching

Urticarial vasculitis. Lesions differ from routine urticaria (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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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 syndrome may develop arthritis, chronic obstructive pulmonary disease, uveitis, episcleritis, and glomerulonephritis. Consequently, careful examination of the joints, heart, lungs, eyes, and kidneys is warranted.

Other distinguishing features are as follows:

Livedo reticularis or racemosa are rarely manifestations of small-vessel vasculitis; these are more common in patients with occlusive or inflammatory disease of medium-sized vessels
Nodular lesions may develop in some patients with small-vessel vasculitis, although these are more common in patients with medium-vessel vasculitis
Ulceration is more common in vasculitis that affects larger vessels, but it may complicate intense LCV and is often referred to as bullous LCV
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^[31]

Differential Diagnoses

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Media Gallery

Hypersensitivity vasculitis.
Henoch-Schönlein purpura.
Histopathology of leukocytoclastic vasculitis.
Urticarial vasculitis. Lesions differ from routine urticaria (hives) in that they last longer (often >24 h), are less pruritic, and often resolve with a bruise or residual pigmentation.
Erythema elevatum diutinum, a rare cutaneous vasculitis.

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Author

A Brooke W Eastham, MD Board Certified Dermatologist, Nashville Skin and Cancer

A Brooke W Eastham, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Medical Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Bryan L Martin, DO Associate Dean for Graduate Medical Education, Designated Institutional Official, Associate Medical Director, Director, Allergy Immunology Program, Professor of Medicine and Pediatrics, Ohio State University College of Medicine

Bryan L Martin, DO is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Osteopathic Internists, American College of Physicians, American Medical Association, American Osteopathic Association

Disclosure: Nothing to disclose.

Leukocytoclastic Vasculitis Clinical Presentation

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