Abstract
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.
Highlights
The term leukocytoclastic vasculitis (LCV) refers to an histopathologic description of a common form of small vessel vasculitis (SVV), involving arterioles, capillaries and postcapillary venules, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”) [1]
Briefly: (1) a single simultaneous course of vasculitis is more frequently due to a drug or infection, (2) recurrent bouts of purpuric rash with periods of remission are suggestive of Henoch–Schönlein purpura (HSP), Cryoglobulinemic Vasculitis (CV) or Immunecomplex vasculitis and (3) chronic persistent occurrence of LCV lesions may be observed in patients with systemic SVV, connective tissue diseases or paraneoplastic syndromes
LCV is a histopathologic term that defines vasculitis of the small vessels in which the inflammatory infiltrate is composed of neutrophils with leukocytoclasia phenomenon
Summary
The term leukocytoclastic vasculitis (LCV) refers to an histopathologic description of a common form of small vessel vasculitis (SVV), involving arterioles, capillaries and postcapillary venules, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”) [1]. Briefly: (1) a single simultaneous course of vasculitis is more frequently due to a drug or infection, (2) recurrent bouts of purpuric rash with periods of remission are suggestive of HSP, CV or Immunecomplex vasculitis and (3) chronic persistent occurrence of LCV lesions may be observed in patients with systemic SVV, connective tissue diseases or paraneoplastic syndromes (hematological or solid malignancies). Non-palpable purpura, on the other hand, is usually due to non-inflammatory vessel wall abnormalities with increased capillary fragility (scurvy, Ehlers–Danlos Syndrome, amyloidosis, steroid purpura, solar purpura, exercise purpura) or hematological or clotting disorders (e.g., thrombocytopenia, clotting defects) Pseudovasculitis, presenting both as palpable or nonpalpable purpura, include several potentially severe diseases, such as infectious emboli (e.g., from endocarditis), acute meningococcemia, disseminated gonococcal infection, Rocky mountain spotted fever, disseminated intravascular coagulation, monoclonal paraproteinemias or Waldenstrom’s disease, thrombotic thrombocytopenic purpura (Moskowitz’s disease), emboli due to cardiac myxoma and cholesterol emboli. In systemic forms, the prognosis largely depends on the severity of organ involvement as well as the extent of underlying disorder
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