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Cutaneous leukocytoclastic angiitis

Orpha number ORPHA889
Synonym(s) Cutaneous hypersensitivity vasculitis
Cutaneous small vessel vasculitis
Prevalence Unknown
Inheritance Not applicable
Age of onset All ages
ICD-10
  • M31.0
ICD-O -
OMIM -
UMLS -
MeSH -
MedDRA -

Summary

Cutaneous vasculitis is a histopathologic entity characterized by neutrophilic transmural inflammation of the blood vessel wall associated with fibrinoid necrosis, termed leukocytoclastic vasculitis (LCV). Prevalence is unknown. Clinical manifestations of cutaneous vasculitis occur when small and/or medium vessels are involved. Small vessel vasculitis can present as palpable purpura, urticaria, pustules, vesicles, petechiae, or erythema multiforme-like lesions. Signs of medium vessel vasculitis include livedo reticularis, ulcers, subcutaneous nodules, and digital necrosis. The prevalence of vasculitis with skin involvement is unknown. Vasculitis can involve any organ system in the body, ranging from skin-limited to systemic disease. Although vasculitis is idiopathic in 50% of cases, common associations include infections, inflammatory diseases, drugs, and malignancy. The management of cutaneous vasculitis is based on four sequential steps: confirming the diagnosis with a skin biopsy, evaluating for systemic disease, determining the cause or association, and treating based on the severity of disease.

Expert reviewer(s)

  • Dr L CHUNG
  • Pr David FIORENTINO

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Detailed information

Review article
  • EN (2005,pdf)
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