- Author: A Brooke W Eastham, MD; Chief Editor: Herbert S Diamond, MD more...
Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis and hypersensitivity angiitis, is a histopathologic term commonly used to denote a small-vessel vasculitis (see the image below). Histologically, LCV is characterized by leukocytoclasis, which refers to vascular damage caused by nuclear debris from infiltrating neutrophils. LCV classically presents as palpable purpura. Less common clinical findings include urticarial plaques, vesicles, bullae, and pustules.
LCV may be secondary to medications, underlying infection, collagen-vascular disorders, or malignancy. However, approximately half of cases are idiopathic.[2, 3]
LCV may be localized to the skin or may be associated with systemic involvement. Internal disease most often manifests in the joints, the gastrointestinal (GI) tract, and the kidneys.
In the absence of internal involvement, the prognosis is excellent, with the majority of cases resolving within weeks to months. Approximately 10% of patients will have chronic or recurrent disease.
LCV may be acute or chronic. Patients with chronic disease may experience persistent lesions or intermittent recurrence. Cases that primarily involve the skin should be treated with nontoxic modalities whenever possible, avoiding the use of systemic corticosteroids and immunosuppressive agents.
Henoch-Schönlein purpura (HSP), a specific subtype of LCV warranting separate discussion, is characterized by predominant IgA-mediated vessel injury. The classic clinical findings of palpable purpura in HSP are often preceded by viral respiratory illness. HSP is more common in children, but can also occur in adults. Children may develop systemic disease with GI, joint, and/or kidney involvement. In adults, arthritis and kidney disease occur more frequently. HSP in adults, especially older men, may be associated with malignancy.
For additional information on HSP, see Henoch-Schönlein purpura.
For additional information on cutaneous manifestations of leukocytoclastic vasculitis, see Hypersensitivity Vasculitis (Leukocytoclastic Vasculitis).
Immune complex deposition, with resultant neutrophil chemotaxis and release of proteolytic enzymes and free oxygen radicals, is a key component in the pathophysiology of LCV.[8, 9] In addition, other autoantibodies, such as antineutrophil cytoplasmic antibody (ANCA); inflammatory mediators, including tumor necrosis factor alpha; and enhanced expression of vascular adhesion molecules may play a role. However, the exact mechanisms remain unknown.
The incidence of leukocytoclastic vasculitis is unknown, but the disorder is presumed to be uncommon.
Several studies on leukocytoclastic vasculitis have been conducted in Spain.[11, 12, 13] Hypersensitivity vasculitis (see first image below) occurs in 10-30 persons per million persons per year. Fourteen cases of Henoch-Schönlein purpura (see second image below) per million persons per year have been reported.
Patients with LCV generally have a good prognosis, but if the kidneys, GI tract, lungs, heart, or central nervous system are involved, morbidity may increase and mortality can occur.
Cutaneous lesions of LCV are often asymptomatic, but may be associated with pruritus or pain.
Bullous lesions, as well as chronic cutaneous disease, may involve ulceration or painful episodes of purpura, which may cause physical limitations.
Race-, Sex-, and Age-related Demographics
Leukocytoclastic vasculitis is reported more often in whites than in other races.
Leukocytoclastic vasculitis affects men and women in approximately equal proportions. Some studies from Spain suggest that LCV may be slightly more common in men than in women.
Leukocytoclastic vasculitis may occur at any age. Henoch-Schönlein purpura is more common in children under 10 years of age.
Lie JT. Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose. Arthritis Rheum. 1994 Feb. 37(2):181-6. [Medline].
Lotti T, Ghersetich I, Comacchi C, Jorizzo JL. Cutaneous small-vessel vasculitis. J Am Acad Dermatol. 1998 Nov. 39(5 Pt 1):667-87; quiz 688-90. [Medline].
Tai YJ, Chong AH, Williams RA, Cumming S, Kelly RI. Retrospective analysis of adult patients with cutaneous leukocytoclastic vasculitis. Australas J Dermatol. 2006 May. 47(2):92-6. [Medline].
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997 Nov 20. 337(21):1512-23. [Medline].
Loricera J, Calvo-Rio V, Ortiz-Sanjuan F, Gonzalez-Lopez MA, Fernandez-Llaca H, Rueda-Gotor J, et al. The spectrum of paraneoplastic cutaneous vasculitis in a defined population: incidence and clinical features. Medicine (Baltimore). 2013 Nov. 92(6):331-43. [Medline].
Tancrede-Bohin E, Ochonisky S, Vignon-Pennamen MD, et al. Schonlein-Henoch purpura in adult patients. Predictive factors for IgA glomerulonephritis in a retrospective study of 57 cases. Arch Dermatol. 1997 Apr. 133(4):438-42. [Medline].
Zurada JM, Ward KM, Grossman ME. Henoch-Schönlein purpura associated with malignancy in adults. J Am Acad Dermatol. 2006 Nov. 55(5 Suppl):S65-70. [Medline].
Mackel SE, Jordon RE. Leukocytoclastic vasculitis. A cutaneous expression of immune complex disease. Arch Dermatol. 1982 May. 118(5):296-301. [Medline].
Sams WM Jr. Hypersensitivity angiitis. J Invest Dermatol. 1989 Aug. 93(2 Suppl):78S-81S. [Medline].
Kevil CG, Bullard DC. Roles of leukocyte/endothelial cell adhesion molecules in the pathogenesis of vasculitis. Am J Med. 1999 Jun. 106(6):677-87. [Medline].
Garcia-Porrua C, Gonzalez-Gay MA, Lopez-Lazaro L. Drug associated cutaneous vasculitis in adults in northwestern Spain. J Rheumatol. 1999 Sep. 26(9):1942-4. [Medline].
Gonzalez-Gay MA, Garcia-Porrua C. Systemic vasculitis in adults in northwestern Spain, 1988-1997. Clinical and epidemiologic aspects. Medicine (Baltimore). 1999 Sep. 78(5):292-308. [Medline].
Martinez-Taboada VM, Blanco R, Garcia-Fuentes M, Rodriguez-Valverde V. Clinical features and outcome of 95 patients with hypersensitivity vasculitis. Am J Med. 1997 Feb. 102(2):186-91. [Medline].
Piette WW, Stone MS. A cutaneous sign of IgA-associated small dermal vessel leukocytoclastic vasculitis in adults (Henoch-Schonlein purpura). Arch Dermatol. 1989 Jan. 125(1):53-6. [Medline].
Jose SK, Marfatia YS. Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome?. Indian J Sex Transm Dis. 2016 Jan-Jun. 37 (1):81-4. [Medline].
Cacoub P, Poynard T, Ghillani P, et al. Extrahepatic manifestations of chronic hepatitis C. MULTIVIRC Group. Multidepartment Virus C. Arthritis Rheum. 1999 Oct. 42(10):2204-12. [Medline].
Sanchez-Guerrero J, Gutierrez-Urena S, Vidaller A, et al. Vasculitis as a paraneoplastic syndrome. Report of 11 cases and review of the literature. J Rheumatol. 1990 Nov. 17(11):1458-62. [Medline].
Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013 Jan. 65(1):1-11. [Medline].
Garcia-Porrua C, Gonzalez-Gay MA. Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch-Schonlein purpura in adults. Semin Arthritis Rheum. 1999 Jun. 28(6):404-12. [Medline].
Davis MD, Daoud MS, Kirby B, et al. Clinicopathologic correlation of hypocomplementemic and normocomplementemic urticarial vasculitis. J Am Acad Dermatol. 1998 Jun. 38(6 Pt 1):899-905. [Medline].
Wisnieski JJ, Baer AN, Christensen J, et al. Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patients. Medicine (Baltimore). 1995 Jan. 74(1):24-41. [Medline].
Gibson LE, Su WP. Cutaneous vasculitis. Rheum Dis Clin North Am. 1995 Nov. 21(4):1097-113. [Medline].
Gonzalez-Gay MA, Garcia-Porrua C, Salvarani C, et al. Cutaneous vasculitis: a diagnostic approach. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S85-8. [Medline].
Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of serum antiphospholipid antibody levels in adult Henoch-Schönlein purpura and cutaneous leukocytoclastic angiitis. Arthritis Rheum. 2008 Apr 15. 59(4):561-7. [Medline].
Jara LJ, Navarro C, Medina G, Vera-Lastra O, Saavedra MA. Hypocomplementemic urticarial vasculitis syndrome. Curr Rheumatol Rep. 2009 Dec. 11(6):410-5. [Medline].
de Hollanda A, Beucher A, Henrion D, Ghali A, Lavigne C, Lévesque H, et al. Systemic and immune manifestations in myelodysplasia: a multicenter retrospective study. Arthritis Care Res (Hoboken). 2011 Aug. 63(8):1188-94. [Medline].
Solans-Laqué R, Bosch-Gil JA, Pérez-Bocanegra C, Selva-O'Callaghan A, Simeón-Aznar CP, Vilardell-Tarres M. Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases. J Rheumatol. 2008 Feb. 35(2):294-304. [Medline].
Loricera J, González-Vela C, Blanco R, Hernández JL, Armesto S, González-López MA, et al. Histopathologic differences between cutaneous vasculitis associated with severe bacterial infection and cutaneous vasculitis secondary to other causes: study of 52 patients. Clin Exp Rheumatol. 2016 May-Jun. 34 (3 Suppl 97):93-7. [Medline].
Carlson JA, Cavaliere LF, Grant-Kels JM. Cutaneous vasculitis: diagnosis and management. Clin Dermatol. 2006 Sep-Oct. 24(5):414-29. [Medline].
Russell JP, Weenig RH. Primary Cutaneous Small Vessel Vasculitis. Curr Treat Options Cardiovasc Med. 2004 Apr. 6(2):139-149. [Medline].
Fiorentino DF. Cutaneous vasculitis. J Am Acad Dermatol. 2003 Mar. 48(3):311-40. [Medline].
Sais G, Vidaller A, Jucgla A, et al. Colchicine in the treatment of cutaneous leukocytoclastic vasculitis. Results of a prospective, randomized controlled trial. Arch Dermatol. 1995 Dec. 131(12):1399-402. [Medline].
Callen JP. Colchicine is effective in controlling chronic cutaneous leukocytoclastic vasculitis. J Am Acad Dermatol. 1985 Aug. 13(2 Pt 1):193-200. [Medline].
Callen JP. Cutaneous vasculitis: Relationship to systemic disease and therapy. Curr Probl Dermatol. 1993. 5:45-80.
Lunardi C, Bambara LM, Biasi D, et al. Elimination diet in the treatment of selected patients with hypersensitivity vasculitis. Clin Exp Rheumatol. 1992 Mar-Apr. 10(2):131-5. [Medline].
Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010 Jul 15. 363(3):221-32. [Medline].
Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. Cutaneous vasculitis in children and adults. Associated diseases and etiologic factors in 303 patients. Medicine (Baltimore). 1998 Nov. 77(6):403-18. [Medline].
Ekenstam Eaf, Callen JP. Cutaneous leukocytoclastic vasculitis. Clinical and laboratory features of 82 patients seen in private practice. Arch Dermatol. 1984 Apr. 120(4):484-9. [Medline].