Cutaneous small-vessel vasculitis

Last updated: June 9, 2023

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Summarytoggle arrow icon

Cutaneous small-vessel vasculitis is a non-ANCA-associated vasculitis of small vessels that affects the skin; involvement of other organs must be absent. Cutaneous small-vessel vasculitis is often idiopathic, but all patients should be evaluated for potential underlying causes, including infections (e.g., HIV, HCV), the use of certain drugs (e.g., propylthiouracil, hydralazine, allopurinol), and other vasculitides (e.g., eosinophilic granulomatosis with polyangiitis). Patients usually present with tender, symmetrical palpable purpura on the lower limbs. Skin biopsy is required to confirm the diagnosis; findings include leukocytoclastic vasculitis. Management depends on the severity and chronicity of the lesions, but NSAIDs and bed rest are usually recommended. Glucocorticoids may be indicated in patients with severe, recurrent, or chronic disease.

Definitiontoggle arrow icon

A necrotizing vasculitis of cutaneous small vessels caused by immune complex deposition; involvement of organs other than the skin must be absent.

Etiologytoggle arrow icon

Patients may report flare triggers such as prolonged sitting or standing, alcohol consumption, or URTIs. [2]

Clinical featurestoggle arrow icon

  • Painful, symmetric nonblanching palpable purpura on the lower limbs [2]
  • Other lesions: subcutaneous nodules, urticaria, ulcers, vesicles
  • Arthralgias may be present.

If drug-induced, lesions usually appear 7–10 days after exposure.

Diagnosticstoggle arrow icon

Approach [2][3]

  • Consult dermatology and/or rheumatology.
  • Request additional diagnostics to:
    • Investigate the underlying etiology
    • Rule out systemic vasculitis
  • A skin biopsy is needed to confirm the diagnosis.

If lesions are confined to a single location, consider a local factor (e.g., trauma, an insect bite) as the underlying etiology. [2]

Cutaneous biopsy

The pathological mechanism that causes cutaneous small-vessel vasculitis can also cause vasculitides in organs other than the skin, which are categorized as distinct diseases. A skin biopsy is essential to establish a definite diagnosis. [2]

Treatmenttoggle arrow icon

Consult a dermatologist and/or rheumatologist for all patients.

Referencestoggle arrow icon

  1. Micheletti RG, Pagnoux C. Management of cutaneous vasculitis. Presse Med. 2020; 49 (3): p.104033.doi: 10.1016/j.lpm.2020.104033 . | Open in Read by QxMD
  2. Goeser MR, Laniosz V, Wetter DA. A Practical Approach to the Diagnosis, Evaluation, and Management of Cutaneous Small-Vessel Vasculitis. Am J Clin Dermatol. 2014; 15 (4): p.299-306.doi: 10.1007/s40257-014-0076-6 . | Open in Read by QxMD
  3. Kasper DL, Fauci AS, Hauser SL, Longo DL, Lameson JL, Loscalzo J. Harrison's Principles of Internal Medicine. McGraw-Hill Education ; 2015
  4. $Contributor Disclosures - Cutaneous small-vessel vasculitis. All of the relevant financial relationships listed for the following individuals have been mitigated: Alexandra Willis (copyeditor, was previously employed by OPEN Health Communications). None of the other individuals in control of the content for this article reported relevant financial relationships with ineligible companies. For details, please review our full conflict of interest (COI) policy:.

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