Updated: 12/7/2018

Leukocytoclastic Vasculitis

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Snapshot
  • A 56-year-old woman presents to her dermatologist’s office for a rash on both of her shins. She reports that this rash started a day ago and that her legs ache also. She has had low-grade fevers and some recent weight loss. Her past medical history includes diabetes, hypertension, and systemic lupus erythematosus, which was diagnosed a year ago but has not had a flare in over 6 months. On physical exam, she has bilateral symmetric non-blanching palpable purpura. Several lesions on her right shin have coalesced into violaceous plaques. She is started on steroids.
Introduction
  • Clinical definition
    • leukocytoclastic vasculitis (LCV)
      • an inflammatory small vessel vasculitis that affects the skin (cutaneous) and/or other organs (systemic)
  • Epidemiology
    • incidence
      • cutaneous LCV > systemic LCV
    • demographics
      • female > male
    • location
      • skin
  • Etiology
    • idiopathic (50% of cases)
    • autoimmune disease (15-20% of cases)
    • drugs (i.e., levamisole-tainted cocaine)
    • infections (i.e., group A streptococcus, hepatitis B, or hepatitis C)
    • malignancy
  • Pathogenesis
    • immune complex deposition in small vessels causes complement activation
    • this releases neutrophils and vasoactive amines, causing inflammation, vessel destruction, and extravasation of red blood cells
      • the “violaceous” purpura is caused by this extravasation of red blood cells
  • Associated conditions
    • Henoch-Schönlein purpura
      • occurs in children
  • Prognosis
    • recurrence occurs in up to 10% of patients
Presentation
  • Symptoms
    • rash
      • occurs 7-10 days after infection, exposure to a medication, or 6+ months after onset of autoimmune disease
      • occurs on dependent areas of the body, such as lower extremities
      • maybe burning, painful, or pruritic
    • systemic symptoms
      • fever
      • weight loss
      • fatigue
      • myalgias
      • abdominal pain
  • Physical exam
    • symmetric, non-blanching, violaceous palpable purpura 
      • 1-3 mm in size
      • lesions may coalesce and lead to plaques, nodules, and necrosis
      • occurs on lower extremities and areas of pressure/trauma (i.e., sock lines and under compression devices)
Studies
  • Labs
    • in cases in which the cause is unknown, patients often undergo autoimmune workup, including antinuclear antibody (ANA), rheumatoid factor (RF), antineutrophil cytoplasmic antibodies (ANCA), cryoglobulins, complement levels, and hepatitis serologies
  • Skin biopsy of fresh lesions (24-48 hours old)
    • histology
      • leukocytoclasis” describes neutrophilic infiltrate, along with granulocytic debris, fibrinoid necrosis of vessel walls, endothelial swelling, and extravasation of red blood cells
    • direct immunofluorescence helps narrow down the differential diagnosis
      • immune complex deposition in small vessel walls
        • immunoglobulin (Ig) A suggests Henoch-Schonlein purpura
        • IgM may suggest cryoglobulinemia
        • C3 or IgG suggests systemic lupus erythematosus
        • negative results may suggest pauci-immune vasculitides such as ANCA-vasculitis
  • Making the diagnosis
    • based on clinical presentation and laboratory studies
Differential
  • Polyarteritis nodosa
    • distinguishing factor
      • a medium vessel vasculitis like polyarteritis nodosa presents with more subcutaneous nodules, livedo reticularis, retiform purpura, and significant ulcers
Treatment
  • Medical
    • topical corticosteroids
      • indication
        • mild disease or single episode
    • systemic corticosteroids
      • indication
        • severe or recurrent disease
    • colchicine or dapsone
      • indication
        • patients who cannot receive corticosteroids, or if the disease recurs after steroid taper
Complications
  • Involvement of internal organs

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