4.4 Review

Diagnosis and management of leukocytoclastic vasculitis

Journal

INTERNAL AND EMERGENCY MEDICINE
Volume 16, Issue 4, Pages 831-841

Publisher

SPRINGER-VERLAG ITALIA SRL
DOI: 10.1007/s11739-021-02688-x

Keywords

Leukocytoclastic vasculitis; Small vessel vasculitis; Cryoglobulinemic vasculitis; IgA vasculitis; Hypocomplementemic urticarial vasculitis

Funding

  1. Universita Politecnica delle Marche within the CRUI-CARE Agreement

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Leukocytoclastic vasculitis is a type of small vessel vasculitis characterized by palpable purpura, which can be caused by various factors such as medications, infections, or malignancy. Diagnosis and treatment involve a comprehensive workup and symptom management, with appropriate therapy based on the underlying cause and severity of the condition.
Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (leukocytoclasia). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch-Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

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