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Update on Cutaneous Small Vessel Vasculitis: Terminology, Morphology, Diagnostic Evaluation, and Management

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Abstract

Purpose of the Review

Cutaneous small vessel vasculitis (CSVV) is the clinical presentation of the histologic entity of leukocytoclastic vasculitis (LCV); the vasculitis may be skin-limited, or also affect internal organ systems. Even when skin-limited, it may be a manifestation of a systemic illness. The etiologies and disease associations are broad, ranging from an uncomplicated, self-limited, immunological phenomenon to a harbinger of autoimmune disease, infection, or malignancy. It is a challenging entity for clinicians to provide a thoughtful and comprehensive evaluation for patients presenting with small vessel vasculitis.

Recent Findings

A dermatologic addendum to the Chapel Hill Consensus Criteria proposed a logical framework to categorize cutaneous small vessel vasculitis into both skin-limited and systemic variants. Recent publications have demonstrated that branching morphology and systemic symptoms suggest a greater need for systemic work up, while cases presenting with non-branching, venule involvement with scant systemic symptoms may not require extensive evaluation. Moreover, radiological studies have demonstrated limited value.

Summary

As cutaneous small vessel vasculitis is a heterogenous disease entity, an updated understanding of terminology, pathogenesis and lesion morphology will help to inform clinicians when to suspect systemic versus skin-limited disease and guide their evaluation accordingly.

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Author information

Author notes

  1. Sophia Reyes-Hadsall and Lara Drake have equal contribution to authorship.

Authors and Affiliations

  1. University of Miami Miller School of Medicine, Miami, FL, USA

    Sophia Reyes-Hadsall

  2. Department of Dermatology, Brigham and Women’s Hospital, 41 Avenue Louis Pasteur, Emmanuel Hall, Boston, MA, 02115, USA

    Sophia Reyes-Hadsall, Lara Drake & Andrew C. Walls

  3. Tufts University School of Medicine, Boston, MA, USA

    Lara Drake

  4. Harvard Medical School, Boston, MA, USA

    Andrew C. Walls

Authors
  1. Sophia Reyes-Hadsall
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  2. Lara Drake
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  3. Andrew C. Walls
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Correspondence to Andrew C. Walls.

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None of the authors (SRH, LD, ACW) have any conflicts of interest or competing interests to disclose.

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Reyes-Hadsall, S., Drake, L. & Walls, A.C. Update on Cutaneous Small Vessel Vasculitis: Terminology, Morphology, Diagnostic Evaluation, and Management. Curr Derm Rep 12, 233–244 (2023). https://doi.org/10.1007/s13671-023-00402-y

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