Leukocytoclastic Vasculitis

Excerpt

Vasculitis refers to inflammation of the blood vessels leading to tissue destruction with or without organ damage. Vasculitis is classified as small vessel, medium vessel or large vessel vasculitis and maybe either idiopathic or associated with an underlying pathology/disease. Small vessel vasculitis can be seen secondary to systemic vasculitides such as Anti-neutrophil Cytoplasmic Antibody (ANCA) associated vasculitis (Microscopic polyangiitis, Granulomatosis with polyangiitis or Eosinophilic granulomatosis with polyangiitis), Behçet’s disease, and Cogan’s syndrome. Immune complex-mediated small vessel vasculitis can be seen in rheumatoid arthritis, systemic lupus erythematosus, Sjogren syndrome, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, Hypocomplementemic urticarial vasculitis, Erythema elevatum diutinum, and cutaneous leukocytoclastic angiitis, formerly known as hypersensitivity vasculitis. Other causes of small vessel vasculitis or leukocytoclastic vasculitis include drug-induced vasculitis, paraneoplastic vasculitis, and infection associated vasculitis (hepatitis B, hepatitis C, syphilis).

Leukocytoclastic vasculitis is a small vessel vasculitis characterized histopathologically by immune complex-mediated vasculitis of the dermal capillaries and venules. Cutaneous leukocytoclastic vasculitis is usually confined to skin with rare extracutaneous manifestations in less than 30% of the cases. Key clinical features of cutaneous leukocytoclastic angiitis include palpable purpura, lower extremity location, small vessel involvement. If leukocytoclastic vasculitis is suspected, a punch biopsy should be performed with direct immunofluorescence studies. If no systemic symptoms are present, laboratory testing including C-reactive protein, complete blood count (CBC), basic metabolic panel, liver function tests, and urinalysis should be done as well. If there is a concern for systemic involvement, a more extensive workup needs to be performed. Most cases of idiopathic cutaneous small-vessel vasculitis cases are self-limited with 90% resolving in weeks to months of onset. In persistent vasculitis, treatment depends on the severity of disease and can range from oral corticosteroids to various steroid-sparing agents.