Recurrent Cutaneous Eosinophilic Vasculitis: A Systematic Review of Clinicopathologic Features and Treatment Outcomes

Authors

  • Shin Iinuma Department of Dermatology, Japanese Red Cross Kitami Hospital, Kitami, Japan; Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan https://orcid.org/0000-0002-3673-0932
  • Takahiro Kobayashi Department of Dermatology, Japanese Red Cross Kitami Hospital, Kitami, Japan
  • Yasuyuki Fujita Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan https://orcid.org/0000-0001-7934-9261

DOI:

https://doi.org/10.2340/actadv.v106.adv-2026-0612

Keywords:

eosinophilia, eosinophilic granulomatosis with polyangiitis, glucocorticoids, systematic review, vasculitis

Abstract

Recurrent cutaneous eosinophilic vasculitis is a rare, predominantly cutaneous vasculitis described mainly in isolated reports. We aimed to systematically synthesize published evidence on its clinico-pathological features, treatment and outcomes. We reviewed case reports and case series of recurrent cutaneous eosinophilic vasculitis and related entities identified in PubMed, Scopus and reference lists (last searched 16 February 2026), using structured report and case-level extraction, duplicate handling, Joanna Briggs Institute critical appraisal and descriptive synthesis. Twenty reports comprising 24 unique cases were included. Most patients presented with relapsing pruritic purpura of the lower extremities. Peripheral eosinophilia was frequent, antineutrophil cytoplasmic antibodies were negative in all reported cases and histopathology consistently showed eosinophil-rich vasculitis, usually with fibrinoid and/or necrotizing change. Systemic corticosteroids often induced improvement, but relapse during tapering or after withdrawal was common. The evidence was limited by small, heterogeneous reports and incomplete follow-up. Overall, the findings support a recognizable cutaneous-predominant, relapse-prone pattern, highlight the importance of assessing extracutaneous involvement in differential diagnosis and support provisional refinement of the previously proposed diagnostic framework.

Downloads

Download data is not yet available.

References

Quijano-Gomero EG, Rodríguez-Zúñiga MJM, Sanz-Montero ME, Durand-Buse D. Clinical, dermoscopic and histologic features of recurrent cutaneous eosinophilic vasculitis cases. Actas Dermosifiliogr 2019; 110: 590–596. DOI: https://doi.org/10.1016/j.adengl.2018.07.017

Li W, Cao W, Song H, Ciu Y, Lu X, Zhang F. Recurrent cutaneous necrotizing eosinophilic vasculitis: a case report and review of the literature. Diagn Pathol 2013; 8: 185. DOI: https://doi.org/10.1186/1746-1596-8-185

Chen KR, Pittelkow MR, Su D, Gleich J, Newman W, Leiferman KM. Recurrent cutaneous necrotizing eosinophilic vasculitis. A novel eosinophil-mediated syndrome. Arch Dermatol 1994; 130: 1159–1166. DOI: https://doi.org/10.1001/archderm.1994.01690090083012

Ishibashi M, Kawahara Y, Chen KR. Spectrum of cutaneous vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): a case series. Am J Dermatopathol 2015; 37: 214–221. DOI: https://doi.org/10.1097/DAD.0000000000000192

Lefèvre G, Leurs A, Gibier JB, Copin MC, Staumont-Sallé D, Dezoteux F, et al. “Idiopathic eosinophilic vasculitis”: another side of hypereosinophilic syndrome? A comprehensive analysis of 117 cases in asthma-free patients. J Allergy Clin Immunol Pract 2020; 8: 1329–1340. DOI: https://doi.org/10.1016/j.jaip.2019.12.011

Chen KR, Su WP, Pittelkow MR, Conn DL, George T, Leiferman KM. Eosinophilic vasculitis in connective tissue disease. J Am Acad Dermatol 1996; 35: 173–182. DOI: https://doi.org/10.1016/S0190-9622(96)90318-7

Lekić B, Gajić-Veljić M, Bonači-Nikolić B, Nikolić M. Penicillin-induced cutaneous necrotizing eosinophilic vasculitis with cryofibrinogenemia. Acta Dermatovenerol Croat 2019; 27: 28–32.

Lin TL, Yang CS, Chen YJ. Recurrent cutaneous necrotising eosinophilic vasculitis. Australas J Dermatol 2021; 62: e102–e106. DOI: https://doi.org/10.1111/ajd.13451

Haddaway NR, Page MJ, Pritchard CC, McGuinness LA. PRISMA2020: an R package and Shiny app for producing PRISMA 2020-compliant flow diagrams, with interactivity for optimised digital transparency and Open Synthesis. Campbell Syst Rev 2022; 18: e1230. DOI: https://doi.org/10.1002/cl2.1230

Nakajima H, Nakamura T. Thrombotic eosinophilic vasculitis. Clin Exp Dermatol 2009; 34: e69–71. DOI: https://doi.org/10.1111/j.1365-2230.2008.03178.x

Chen KR, Su WP, Pittelkow MR, Leiferman KM. Eosinophilic vasculitis syndrome: recurrent cutaneous eosinophilic necrotizing vasculitis. Semin Dermatol 1995; 14: 106–110. DOI: https://doi.org/10.1016/S1085-5629(05)80005-7

Launay D, Delaporte E, Gillot JM, Janin A, Hachulla E. An unusual cause of vascular purpura: recurrent cutaneous eosinophilic necrotizing vasculitis. Acta Derm Venereol 2000; 80: 394–395. DOI: https://doi.org/10.2340/0001555580394395

Sakuma-Oyama Y, Nishibu A, Oyama N, Saito M, Nakamura K, Kaneko F. A case of recurrent cutaneous eosinophilic vasculitis: successful adjuvant therapy with suplatast tosilate. Br J Dermatol 2003; 149: 901–903. DOI: https://doi.org/10.1046/j.1365-2133.2003.05568.x

Tsunemi Y, Saeki H, Ihn H, Tamaki K. Recurrent cutaneous eosinophilic vasculitis presenting as annular urticarial plaques. Acta Derm Venereol 2005; 85: 380–381. DOI: https://doi.org/10.1080/00015550510030113

Tanglertsampan C, Tantikun N, Noppakun N, Pinyopornpanit V. Indomethacin for recurrent cutaneous necrotizing eosinophilic vasculitis. J Med Assoc Thai 2007; 90: 1180–1182.

Kiorpelidou D, Gaitanis G, Zioga A, Tsili AC, Bassukas ID. Chronic periaortitis (retroperitoneal fibrosis) concurrent with recurrent cutaneous eosinophilic vasculitis. Case Rep Dermatol Med 2011; 2011: 548634. DOI: https://doi.org/10.1155/2011/548634

Sugiyama M, Nozaki Y, Ikoma S, Kinoshita K, Funauchi M. Successful treatment with tacrolimus in a case of the glucocorticoid-dependent recurrent cutaneous eosinophilic vasculitis. Ann Dermatol 2013; 25: 252–254. DOI: https://doi.org/10.5021/ad.2013.25.2.252

Sawada C, Taniai M, Kawashima M, Ishiguro N. Recurrent cutaneous eosinophilic vasculitis. Eur J Dermatol 2016; 26: 108–109. DOI: https://doi.org/10.1684/ejd.2015.2680

Riyaz N, Sasidharanpillai S, Hazeena C, Aravindan KP, Bindu CS, Silpa KN. Recurrent cutaneous eosinophilic vasculitis: a rare entity. Indian J Dermatol 2016; 61: 235. DOI: https://doi.org/10.4103/0019-5154.177794

Uehara A, Endo Y, Kosaka K, Oka A, Ishikawa O, Motegi SI. Case of recurrent cutaneous eosinophilic vasculitis with subcutaneous nodules in the early stage of the disease. J Dermatol 2022; 49: e217–e218. DOI: https://doi.org/10.1111/1346-8138.16342

Gisondi P, Geat D, Colato C, Girolomoni G. Recurrent cutaneous eosinophilic vasculitis characterized by annular purpuric lesions: a case report. SAGE Open Med Case Rep 2023; 11: 2050313X231163636. DOI: https://doi.org/10.1177/2050313X231163636

Iinuma S, Kobayashi T, Ishida-Yamamoto A. Pruritic palpable purpura on the lower legs: a quiz. Acta Derm Venereol 2023; 103: adv18366. DOI: https://doi.org/10.2340/actadv.v103.18366

Li J, Lin J, Ma L. Azathioprine combined with corticosteroids for recurrent cutaneous necrotizing eosinophilic vasculitis secondary to eosinophilic dermatitis. Minerva Gastroenterol 2023; 69: 596–597. DOI: https://doi.org/10.23736/S2724-5985.23.03424-1

Rodriguez-Troncoso M, Ruiz-Villaverde R, Pegalajar-García MD. sQuiz your knowledge! recurrent ulcerated papuloplaques on the legs: a diagnosis not to be missed. Eur J Dermatol 2025; 35: 162–164. DOI: https://doi.org/10.1684/ejd.2025.4897

Ohnishi Y, Zenke Y, Arai S, Suyama Y. Recurrent cutaneous eosinophilic vasculitis. J Allergy Clin Immunol Pract 2025; 13: 3420–3421. DOI: https://doi.org/10.1016/j.jaip.2025.09.033

Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, et al. Evidence-based guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol 2023; 19: 378–393. DOI: https://doi.org/10.1038/s41584-023-00958-w

Frumholtz L, Laurent-Roussel S, Lipsker D, Terrier B. Cutaneous vasculitis: review on diagnosis and clinicopathologic correlations. Clin Rev Allergy Immunol 2021; 61: 181–193. DOI: https://doi.org/10.1007/s12016-020-08788-4

Additional Files

Published

2026-06-24

How to Cite

Iinuma, S., Kobayashi, T., & Fujita, Y. (2026). Recurrent Cutaneous Eosinophilic Vasculitis: A Systematic Review of Clinicopathologic Features and Treatment Outcomes. Acta Dermato-Venereologica, 106, adv–2026. https://doi.org/10.2340/actadv.v106.adv-2026-0612