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Leukocytoclastic vasculitis (cutaneous small vessel vasculitis)
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Leukocytoclastic vasculitis (LCV) is a histological term that denotes vasculitis of small vessels (capillaries and postcapillary venules). LCV is the histological presentation of cryoglobulinemic vasculitis, IgA vasculitis, normocomplementemic and hypocomplementemic urticarial vasculitis, vasculitis that may be associated with systemic disease (systemic lupus erythematosus, rheumatoid arthritis, sarcoidosis, inflammatory bowel disease, etc.), vasculitis associated with a probable etiology (drugs, infections), IgM/IgG vasculitis, nodular vasculitis, erythema elevatum diutinum, hypergammaglobulinemic macular vasculitis, and sometimes with ANCA-associated vasculitides (AAV) including microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis. Clinically, LCV may present as palpable purpura, urticarial papules or plaques, nodules, hemorrhagic bullae, or superficial ulceration. Given the broad range of diagnoses that present with LCV, careful evaluation for systemic involvement is necessary. Although histopathologically patients with AAV most commonly present with LCV, the management of AAV is beyond the scope of this chapter.
Nomenclature of cutaneous: dermatologic addendum to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides
Sunderkotter CH, Zegler B, Chen KR, et al. Arthritis Rheumatol 2018; 70(2): 171–84.
Management Strategy
Management of LCV requires evaluation for systemic involvement, removal of potential causative agents or treatment directed at associated diseases, management of symptoms in those patients with self-limiting disease, and targeted or empiric therapy for patients with recurrent or recalcitrant disease. Patients with systemic vasculitis, particularly when involving the kidneys, lungs, or central nervous system, require immediate referral to rheumatology, nephrology, or other specialists and frequently require hospitalization for treatment with systemic corticosteroids and immunosuppressive/immunomodulatory agents .
Patients with LCV in whom there is an identifiable cause, such as a drug, are treated symptomatically in addition to removing the presumed causative agent. Symptomatic measures include rest , elevation , gradient support stockings , and antihistamines.
The challenge is to treat the patient who has chronic LCV without a defined etiology and without systemic involvement. In patients with asymptomatic disease who are not bothered by the appearance of their vasculitic lesions, no treatment may be needed. For those patients who develop pain, ulcerations, or psychological distress, the risks and benefits of therapy should be discussed. Treatment recommendations for LCV are largely based on case reports, case series, and expert opinion. If systemic therapy is considered for disease ‘confined to the skin’, colchicine and dapsone are first-line agents, given their relative safety. Immunosuppressive/immunomodulatory agents including methotrexate and azathioprine have been used in patients who are refractory to colchicine and dapsone. Systemic corticosteroids should be avoided when possible due to the narrow window between therapeutic effect and toxicity.
Specific Investigations
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The purpose of evaluating the patient with cutaneous vasculitis is to identify a cause of the process and assess for the presence of systemic involvement. The evaluation begins with a careful history and physical examination, followed by selected testing based on the acuteness of the process and the findings from the history and physical examination. Direct immunofluorescence (DIF) is required to differentiate IgA vasculitis, which might be associated with a higher risk of renal involvement, from other causes of LCV.
Cutaneous vasculitis in children and adults: associated diseases and etiologic factors in 303 patients
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. Medicine 1998; 77: 403–18.
Diagnoses and associated diseases of 172 adults and 131 children who presented to a medical center over a 19-year period. Of the 131 children, only one had a secondary vasculitis. In contrast, approximately 30% of the adults had systemic involvement or secondary vasculitis. The authors therefore recommend less intensive investigations in children than in adults.
A practical approach to the diagnosis, evaluation, and management of cutaneous small-vessel vasculitis
Goeser MR, Laniosz V, Wetter DA. Am J Clin Dermatol 2014; 15: 299–306.
An approach to the patient with small vessel vasculitis and a very useful algorithm for evaluation and treatment.
Single-organ cutaneous small-vessel vasculitis according to the 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides: a study of 60 patients from a series of 766 cutaneous vasculitis cases
Loricera J, Blanco R, Ortiz-Sanjuán F, et al. Rheumatology 2015; 54: 77–82.
This retrospective review spanning 35 years assessed 766 patients with a diagnosis of cutaneous vasculitis. Of these patients, 60 (7.8%) met the 2012 CHCC definition of single-organ cutaneous small vessel vasculitis (SoCSVV). The etiology of the SoCSVV was medication-related in 52% and infection-related in 34%. Approximately a quarter of patients with SoCSVV were treated with systemic corticosteroids or non-steroidal antiinflammatory drugs (NSAIDs). Complete recovery was noted in all patients within 4 months; however, 8% of them developed recurrence.
Colchicine in the treatment of cutaneous leukocytoclastic vasculitis: results of a prospective, randomized controlled trial
Sais G, Vidaller A, Jucgla A, et al. Arch Dermatol 1995; 131: 1399–402.
Forty subjects with cutaneous small-vessel vasculitis (CSVV) were randomized to receive colchicine or topical emollients. The study was powered to detect a 40% difference between groups. The study failed to detect a statistically significant difference between groups; however, the colchicine-treated group included all patients who had failed to respond to dapsone and a disproportionate number of subjects who had failed other therapies. Thus, the colchicine arm included patients with more recalcitrant disease, and this may have contributed to the negative result. It was also noted that the subjects’ disease flared with withdrawal of the colchicine.
Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis
Callen JP. J Am Acad Dermatol 1985; 13: 193–200.
This open-label study involved 13 patients. This is the largest case series of colchicine use in CSVV.
Colchicine use in CSVV is supported by several case reports and case series. An inadvertently imbalanced randomized controlled trial (RCT) of colchicine in CSVV failed to show statistically significant improvement.
Sulfone therapy in the treatment of leukocytoclastic vasculitis. Report of three cases
Fredenberg MF, Malkinson FD. J Am Acad Dermatol 1987; 16: 772–8.
Three adult patients with CSVV were successfully treated with moderate doses of dapsone (100–150 mg daily).
Dapsone in Henoch-Schönlein purpura
Sarma PS. Postgrad Med J 1994; 70: 464–5.
Case series of six adults with IgA vasculitis treated with dapsone 100 mg daily. Four of six had clearance of arthritis and purpura within 1 week.
Indications and efficiency of dapsone in IgA vasculitis (Henoch-Schonlein purpura): case series and a review of the literature
Roman C, Dima B, Muyshont L, et al. Eur J Pediatr 2019; 178(8): 1275–81.
Case series including two children of IgA vasculitis and literature review to include 15 additional cases. All 17 children with refractory cutaneous IgA vasculitis responded to 1 to 2 mg/kg/day of dapsone for 7 days. One patient developed methemoglobinemia. No improvements were noted in systemic manifestations of IgA vasculitis.
The therapeutic response of urticarial vasculitis to indomethacin
Millns JL, Randle HW, Solley GO, et al. J Am Acad Dermatol 1980; 3: 349–55.
Open-label trial of 10 patients with urticarial vasculitis (UV) treated with indomethacin 25 mg three times daily to 50 mg four times daily. Six cleared within 17 days. Three had partial improvement.
Characteristics and management of IgA vasculitis (Henoch–Schönlein) in adults: data from 260 patients included in a French multicenter retrospective survey
Audemard-Verger A, Terrier B, Dechartres A, et al. Arthritis Rheumatol 2017; 69(9): 1862–70.
In total, 260 adult patients with IgA vasculitis were treated with combinations of corticosteroids, cyclophosphamide, and colchicine. Patients treated with corticosteroids alone had the highest response rate at 80%. Statistical modeling did not show clear support for the addition of cyclophosphamide to corticosteroids.
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