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Ntatsaki E, Carruthers D, Chakravarty K, et al; BSR and BHPR Standards, Guidelines and Audit Working Group. BSR and BHPR guideline for the management of adults with ANCA-associated vasculitis. Rheumatology (Oxford). 2014 Dec;53(12):2306-9. doi: 10.1093/rheumatology/ket445. Epub 2014 Apr 11. PubMed PMID: 24729399.
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan;65(1):1-11. doi: 10.1002/art.37715. PubMed PMID: 23045170.
Mukhtyar C, Guillevin L, Cid MC, et al; European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2009 Mar;68(3):310-7. doi: 10.1136/ard.2008.088096. Epub 2008 Apr 15. PubMed PMID: 18413444.
DefinitionTop
Cutaneous leukocytoclastic angiitis is an isolated cutaneous vasculitis with no internal organ involvement (also known as cutaneous small-vessel vasculitis; formerly hypersensitivity vasculitis). Secondary cutaneous leukocytoclastic vasculitis may be, among other causes, due to medications, infections, and malignancies.
Clinical FeaturesTop
Typically the disease manifests as fever, arthralgia, myalgia, and malaise. Cutaneous lesions usually cause no complaints but pruritus and burning sensation may occur. The lesions mainly involve the lower extremities, buttocks, and sites of prior trauma, as well as areas subject to pressure from tight clothing; they have a form of maculopapular rash, palpable purpura, urticaria, or ulcerating papules.
DiagnosisTop
Diagnosis is based on results of histologic examination of skin biopsy specimens and exclusion of internal organ involvement.
Differential diagnosis includes thromboembolic events (eg, in the course of sepsis or disseminated intravascular coagulation), thrombotic thrombocytopenic purpura, other small-vessel vasculitides, and primary or secondary rash unrelated to vasculitis.
TreatmentTop
1. Primary leukocytoclastic angiitis:
1) General measures (these are frequently sufficient): Keeping the involved area warm, avoiding exposure to cold and sunlight, limited physical activity.
2) Antihistamines and nonsteroidal anti-inflammatory drugs in the case of symptomatic cutaneous lesions or arthralgia may be helpful.
3) Colchicine 0.5 mg bid and/or dapsone 50 to 300 mg daily in patients with chronic, extensive, or painful skin lesions or persistent arthralgia may be effective.Evidence 1Weak recommendation (benefits likely outweigh downsides, but the balance is close or uncertain; an alternative course of action may be better for some patients). Low Quality of Evidence (low confidence that we know true effects of the intervention). Quality of Evidence lowered due to the lack of patient-important outcomes and imprecision. Sais G, Vidaller A, Jucglà A, Gallardo F, Peyrí J. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol. 1995 Dec;131(12):1399-402. PubMed PMID: 7492128. The effects of treatment are observed within 2 weeks.
4) Oral glucocorticoids in patients with severe cutaneous lesions may be helpful.
5) Azathioprine 2 mg/kg/d in case the above-mentioned agents are ineffective (eg, when lesions recur after tapering down the dose of glucocorticoids).
2. Secondary leukocytoclastic angiitis: Treatment of the underlying condition or discontinuation of the offending drug. In the case of life-threatening symptoms, treatment is the same as in primary disease.
The primary disease usually resolves spontaneously within several weeks or months. In ~10% of patients the disease relapses after several months or years. The course of secondary disease depends on the underlying condition and usually resolves once the causative factor has been successfully treated.
