Leukocytoclastic vasculitis

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Leukocytoclastic vasculitis

Nicole Fett and Jeffrey P. Callen

Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports

Vasculitis is best classified based on the size of the vessels involved. Large vessel vasculitides, giant cell arteritis and Takayasu arteritis, involve large arteries and rarely have associated cutaneous findings. Pure medium vessel vasculitides such as polyarteritis nodosa, cutaneous polyarteritis nodosa and Kasawaki disease affect vessels with muscular walls. Cutaneous features of medium vessel vasculitides include livedo reticularis, retiform purpura, nodules, ulcers, and infarcts. Vasculitides that involve both medium and small vessels include granulomatosis with polyangiitis (previously Wegener granulomatosis), eosinophilic granulomatous angiitis (previously Churg–Strauss Syndrome), microscopic polyangiitis, and cryoglobulinemic vasculitis. Overlap medium and small vessel vasculitides may present with features of medium vessel involvement and features classic for small vessel involvement (urticaria, palpable purpura). Lastly, pure small vessel vasculitides (cutaneous small vessel vasculitis) include IgA vasculities (previously Henoch–Schönlein purpura (HSP)), erythema elevatum diutinum, urticarial vasculitis, and ‘leukocytoclastic vasculitis’ (LCV). LCV is a term that defines a histopathological pattern, not cutaneous findings. The majority of patients labeled with LCV have cutaneous small vessel vasculitis (CSVV); however, the most common cutaneous manifestation of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and connective tissue disease associated vasculitis is LCV and therefore careful evaluation for systemic involvement is necessary in these patients. CSVV is secondary to exposure to medications and viruses over 60% of the time and in general tends to be self-limited.

Management strategy

Management of small vessel vasculitis requires evaluation for systemic involvement, removal of potential causative agents, management of symptoms in those patients with self-limited disease, and targeted therapy for patients with recurrent or recalcitrant disease. Patients with systemic vasculitis require immediate referral to rheumatology, pulmonology, or nephrology and generally require treatment with systemic corticosteroids and systemic immunosuppressive agents.

Patients with CSVV 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 CSVV without a defined etiology and without systemic involvement. In patients with asymptomatic disease who are not bothered by the appearance of their vasculitis, no treatment is necessary. For those patients who develop pain, ulcerations, or psychological distress, the risks and benefits of therapy should be discussed. Treatment recommendations for CSVV 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. Systemic corticosteroids, methotrexate, and azathioprine have been used in patients who are refractory to colchicine and dapsone.

Specific investigations

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 is required to differentiate IgA vasculitis from other causes of CSVV.

Cutaneous vasculitis in children and adults: associated diseases and etiologic factors in 303 patients.

Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M. Medicine 1998; 77: 403–18.

With an ultimate goal of proposing an algorithm for the evaluation of patients who present with cutaneous lesions consistent with vasculitis, these authors review the 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. Based on these findings, the authors recommend less intensive investigations in children than in adults.

Cutaneous vasculitis update: classification, epidemiology, etiology, pathogenesis, evaluation and prognosis.

Carlson JA, Chen KR. Am J Dermatopathol 2005; 27: 504–28.

This exhaustive review covers classification based on vessel size, biopsy timing, importance of direct immunofluorescence, and makes further recommendations on evaluation.

Clinical approaches to cutaneous vasculitis.

Gonzalez-Gay MA, Garcia-Porrua C, Pujol RM. Curr Opin Rheumatol 2005; 17: 56–61.

This review details an approach to the patient with small vessel vasculitis and provides a very useful algorithm.

First-line therapies

Observation	D
Removal or withdrawal of the causative agent (e.g., drug)	D
Colchicine	C
Dapsone	C
Non-steroidal anti-inflammatory drugs	C

Colchicine use in CSVV is supported by four cases series. An inadvertently imbalanced randomized controlled trial of colchicine in CSVV failed to show statistically significant improvement.

Colchicine in the treatment of cutaneous leukocytoclastic vasculitis: results of a prospective, randomized controlled trial.

Sais G, Vidaller A, Jucgla A, Gallardo F, Peyri J. Arch Dermatol 1995; 131: 1399–402.

Forty subjects with 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 amount of subjects who failed other therapies. Thus the colchicine arm included patients with more recalcitrant disease, which likely contributed to the negative results. 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.

There are several single cases reporting efficacy of dapsone in CSVV and four small case series supporting its use.

Second-line therapies

Systemic corticosteroids	A
Immunosuppressive/cytotoxic agents: azathioprine (B for negative, C for positive), methotrexate (C), cyclosporine (C), mycophenolate mofetil (C)
Rituximab (A for cryoglobulinemic vasculitis and ANCA-associated vasculitis, E for other vasculitides)