Leukocytoclastic Vasculitis

Updated: Oct 11, 2017
  • Author: A Brooke W Eastham, MD; Chief Editor: Herbert S Diamond, MD  more...
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Overview

Practice Essentials

Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see the image below). [1] Histologically, LCV is characterized by leukocytoclasis, which refers to vascular damage caused by nuclear debris from infiltrating neutrophils. LCV classically presents as palpable purpura. Less common clinical findings include urticarial plaques, vesicles, bullae, and pustules.

Histopathology of leukocytoclastic vasculitis. Histopathology of leukocytoclastic vasculitis.

LCV may be secondary to medications, underlying infection, collagen-vascular disorders, or malignancy. [2] However, approximately half of cases are idiopathic. [3, 4]

LCV may be localized to the skin or may be associated with systemic involvement. [5] Internal disease most often manifests in the joints, the gastrointestinal (GI) tract, and the kidneys.

In the absence of internal involvement, the prognosis is excellent, with the majority of cases resolving within weeks to months. Approximately 10% of patients will have chronic or recurrent disease. [6]

LCV may be acute or chronic. Patients with chronic disease may experience persistent lesions or intermittent recurrence. Cases that primarily involve the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.

Henoch-Schönlein purpura (HSP), a specific subtype of LCV warranting separate discussion, is characterized by predominant IgA-mediated vessel injury. [7] The classic clinical findings of palpable purpura in HSP are often preceded by viral respiratory illness. HSP is more common in children, but can also occur in adults. Children may develop systemic disease with GI, joint, and/or kidney involvement. In adults, arthritis and kidney disease occur more frequently. [8] HSP in adults, especially older men, may be associated with malignancy. [9]

For additional information on HSP, see Henoch-Schönlein purpura.

For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis).

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Pathophysiology

Immune complex deposition, with resultant neutrophil chemotaxis and release of proteolytic enzymes and free oxygen radicals, is a key component in the pathophysiology of LCV. [10, 11] In addition, other autoantibodies, such as antineutrophil cytoplasmic antibody (ANCA); inflammatory mediators, including tumor necrosis factor alpha; and enhanced expression of vascular adhesion molecules may play a role. [12] However, the exact mechanisms remain unknown.

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Epidemiology

Frequency

United States

The incidence of leukocytoclastic vasculitis is unknown, but the disorder is presumed to be uncommon.

International

Several studies on leukocytoclastic vasculitis have been conducted in Spain. [13, 14, 15] Hypersensitivity vasculitis (see first image below) occurs in 10-30 persons per million persons per year. Fourteen cases of Henoch-Schönlein purpura (see second image below) per million persons per year have been reported.

Hypersensitivity vasculitis. Hypersensitivity vasculitis.
Henoch-Schönlein purpura. Henoch-Schönlein purpura.

Mortality/Morbidity

Patients with LCV generally have a good prognosis, but if the kidneys, GI tract, lungs, heart, or central nervous system are involved, morbidity may increase and mortality can occur.

Cutaneous lesions of LCV are often asymptomatic, but may be associated with pruritus or pain.

Bullous lesions, as well as chronic cutaneous disease, may involve ulceration or painful episodes of purpura, which may cause physical limitations.

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Race-, Sex-, and Age-related Demographics

Leukocytoclastic vasculitis is reported more often in whites than in other races.

Leukocytoclastic vasculitis affects men and women in approximately equal proportions. Some studies from Spain suggest that LCV may be slightly more common in men than in women.

Leukocytoclastic vasculitis may occur at any age. Henoch-Schönlein purpura is more common in children under 10 years of age.

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