Abstract
The skin is a common target organ in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Skin lesions may present as the initial manifestation or as the associated cutaneous manifestations in ANCA-associated vasculitis. Visually assessing the cutaneous manifestations and performing a biopsy of skin lesions to facilitate histopathologic confirmation are highly valuable diagnostically. Vasculitis (which affects dermal small vessels and subcutaneous muscular vessels) and nonvasculitic inflammation cause various types of skin lesions. The coexistence of different levels of vasculitis and nonvasculitis can occur in one or in several lesions or on different occasions. These puzzling clinical and histopathologic features may be closely related to the disease activity. Understanding the complicated clinical and histopathologic spectrum of skin lesions will contribute to the early diagnosis of ANCA-associated vasculitis with cutaneous complications.
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References
Chen KR, Carlson JA. Clinical approach to cutaneous vasculitis. Am J Clin Dermatol. 2008;9:71–92.
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med. 1997;337:1512–23.
Guillevin L, Cohen P, Gayraud M, Lhote F, et al. Churg Strauss syndrome: clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78:26–37.
Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42:421–30.
Duna GF, Galperin C, Hoffman GS. Wegener’s granulomatosis. Rheum Dis Clin North Am. 1995;21:949–86.
Chen KR, Sakamoto M, Ikemoto K, et al. Granulomatous arteritis in cutaneous lesions of Churg–Strauss syndrome. J Cutan Pathol. 2007;34:330–7.
Chen KR. Histopathology of cutaneous vasculitis. In: Amezcua-Guerra LM, editor. Advances in the diagnosis and treatment of vasculitis; 2011. p. 19–56 (ISBN: 978-953-307-786-4).
Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg–Strauss syndrome. Arthritis Rheum. 2005;52:2926–35.
Grau RG. Churg–Strauss syndrome: 2005–2008 update. Curr Rheumatol Rep. 2008;10:453–8.
Ishibashi M, Kudo S, Yamamoto K, et al. Churg–Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid-like blisters. J Cutan Pathol. 2011;38:290–4.
Wilmonth GJ, Perniciaro C. Cutaneous extravascular necrotizing granuloma (Winkelmann granuloma): confirmation of the association with systemic disease. J Am Acad Dermatol. 1996;34:753–9.
Daoud MS, Gibson LE, DeRemee RA, et al. Cutaneous Wegener’s granulomatosis: clinical, histopathologic and immunopathologic features of thirty patients. J Am Acad Dermatol. 1994;31:605–12.
Reed WB, Jensen AK, Konwaler BE, et al. The cutaneous manifestation in Wegener’s granulomatosis. Acta Derm Venereol (Stockh). 1963;43:250–64.
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Chen, KR. Skin involvement in ANCA-associated vasculitis. Clin Exp Nephrol 17, 676–682 (2013). https://doi.org/10.1007/s10157-012-0736-x
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DOI: https://doi.org/10.1007/s10157-012-0736-x