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Cutaneous small-vessel vasculitis 

Cutaneous small-vessel vasculitis
Chapter:
Cutaneous small-vessel vasculitis
Author(s):

Anna Haemel

, Lindy Fox

, and M. Kari Connolly

DOI:
10.1093/med/9780199659869.003.0038
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date: 27 October 2021

Cutaneous small vessel vasculitis (CSVV) is an immune-complex-mediated disease targeting the postcapillary venules of the skin. Several classifications and synonyms have been proposed; however, simplified diagnostic criteria include palpable purpura with histopathology demonstrating leukocytoclastic vasculitis, in the absence of systemic small or medium-vessel vasculitis. CSVV is considered to be a reactive process, with underlying triggers (infection, drug, autoimmune disease, or neoplasm, though ≥40% of cases remain idiopathic). The triggers can be conceptualized as antigens to which antibodies are generated, with subsequent immune complex deposition, complement activation, neutrophil influx, and damage to the vessel wall. The inflammatory infiltrate contributes to the raised nature and erythema of the lesions, while vessel damage and haemorrhage result in non-blancheable purpura. The differential diagnosis includes macular purpura due to coagulation defects, other inflammatory skin diseases, and infections. While CSVV is generally limited to the skin, any site where immune complex could be filtered and deposited is potentially involved. Therefore, patients must be evaluated for both end-organ involvement/ systemic vasculitis syndromes and underlying triggers. Evaluation should also include punch biopsy for histopathology and direct immunofluorescence. Treatment includes removal of triggers and empiric anti-inflammatory agents for more severe disease.

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