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Updated: Dec 28 2021

Leukocytoclastic Vasculitis

  • 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
  • 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
  • Differential
    • Polyarteritis nodosa
      • distinguishing factor
        • a medium vessel vasculitis like polyarteritis nodosa presents with more subcutaneous nodules, livedo reticularis, retiform purpura, and significant ulcers
    • Making the diagnosis
      • based on clinical presentation and laboratory studies
  • 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
  • Prognosis
    • Recurrence occurs in up to 10% of patients
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