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

Clinical Snapshot

Key Findings

• P Value: Not reported — descriptive synthesis without inferential statistics

• Effect Size: Not applicable — descriptive synthesis only; no pooled effect size calculable from 24 case-level observations

• Primary Outcome: Clinicopathological characterisation of RCEV: most patients presented with relapsing pruritic purpura of the lower extremities; histopathology consistently showed eosinophil-rich vasculitis, usually with fibrinoid and/or necrotizing change; ANCA was negative in all reported cases; peripheral eosinophilia was frequent

• Nnt Or Sensitivity: Not calculable; systemic corticosteroids frequently induced clinical improvement but relapse during tapering or after withdrawal was described as common across cases — no formal response rate with confidence intervals provided

• Confidence Interval: Not reported — no quantitative pooling performed

Clinical Application

Diagnosis requires skin biopsy with histopathological expertise in vasculitis and eosinophilic disorders. ANCA testing is essential to exclude EGPA and other ANCA-associated vasculitides. Systemic corticosteroids are accessible in all healthcare settings. Steroid-sparing agents may be required given the relapsing course, but evidence for specific second-line agents is absent from this review. Long-term dermatological and potentially rheumatological follow-up is warranted. RCEV is not currently listed as a distinct diagnostic entity in RACGP or Australian Rheumatology Association guidelines, reflecting its rarity and recent characterisation. Dermatologists and rheumatologists in Australia should be aware of this condition in the differential diagnosis of recurrent eosinophilic vasculitis. Systemic corticosteroids (prednisolone) are PBS-listed for vasculitic conditions. Second-line steroid-sparing agents such as dapsone, colchicine, or hydroxychloroquine, which may be considered based on individual case reports, have variable PBS availability for this indication. TGA approval for specific agents in RCEV does not exist given the absence of clinical trial data. Referral to a tertiary dermatology or vasculitis centre is advisable for diagnostic confirmation and management planning. The predominantly Japanese authorship and case base warrants caution regarding direct applicability to the ethnically diverse Australian population. Patients presenting with recurrent, relapsing pruritic purpura predominantly affecting the lower extremities, with peripheral eosinophilia and negative ANCA, in whom skin biopsy demonstrates eosinophil-rich vasculitis with fibrinoid or necrotising change. Clinicians should consider RCEV in the differential diagnosis after excluding secondary causes of eosinophilic vasculitis including drug reactions, parasitic infection, hypereosinophilic syndrome, eosinophilic granulomatosis with polyangiitis (EGPA), and connective tissue disease.

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.

References

1. 1.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-2026-0612. https://doi.org/10.2340/actadv.v106.adv-2026-0612