Clinical Reference / Therapeutic Strategies / Leukocytoclastic Vasculitis

Leukocytoclastic Vasculitis


Key Points

  • Leukocytoclastic vasculitis (LCV) represents inflammation of small vessels in the skin stemming from immune complex deposition.
  • Clinically, LCV presents with an eruption of palpable monomorphous purpuric papules, which may have central vesicles or pustules and may coalesce into larger plaques. It favors dependent areas of the body.
  • In approximately 50% of cases, cutaneous LCV is caused by medications, drugs (cocaine), infection (such as hepatitis, streptococcal infection), autoimmune disease, or malignancy.
  • The therapeutic strategy is to search for an etiology, then treat the underlying disorder.

Introduction

Leukocytoclastic vasculitis (LCV) represents inflammation of small vessels in the skin stemming from immune complex deposition. It is sometimes associated with skin pruritus or tenderness. Clinically, LCV presents with an eruption of palpable monomorphous purpuric papules, which may have central vesicles or pustules and may coalesce into larger plaques. The presentation is typically symmetric and affects dependent areas of the body (lower extremities in an ambulatory patient, sacrum and buttocks in a hospitalized patient). In approximately 50% of cases, cutaneous LCV is caused by medications, drugs (cocaine), infection (such as hepatitis, streptococcal infection), autoimmune disease, or malignancy. In rare cases, LCV will present in the setting of a syndrome such as Henoch-Schönlein purpura (HSP), typified by the triad of cutaneous LCV (IgA deposition), arthritis, and gastrointestinal involvement in which the skin lesions predominantly affect the lower extremities and buttocks.

The therapeutic strategy is to treat the underlying disorder. Most cases of cutaneous LCV will require no or only brief courses of immunosuppressive therapy so, if symptoms persist, the concern for a systemic vasculitis should be diagnostically evaluated. Cutaneous LCV may be associated with internal organ involvement, especially of small vessels of the brain, lungs, kidney, and gastrointestinal system, and the morphology and severity of the skin disease do not predict such involvement. Therefore, systemic small vessel vasculitis must be evaluated in every patient presenting with LCV. Treatment may be required for systemic disease even when skin lesions are mild.

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