You are here

Article Text

Article menu
Download PDFPDF
Case Reports: Rare disease
Localised chronic fibrosing vasculitis versus erythema elevatum diutinum
  1. Mika M Tabata1,
  2. William M Lin2,
  3. Jay Goldstein3,
  4. Timothy R Quinn2 and
  5. Steven T Chen4
  1. 1Dermatology, UT Austin, Austin, Texas, USA
  2. 2Dermatology, Harvard University, Cambridge, Massachusetts, USA
  3. 3Goldstein Dermatology, Natick, MA, USA
  4. 4Dermatology, Internal Medicine, Harvard University, Cambridge, Massachusetts, USA
  1. Correspondence to Dr Steven T Chen; stchen{at}bics.bwh.Harvard.edu

Abstract

A woman in her 70s was referred for a painless plaque on the shin, present for 2 years and progressing in thickness. Examination revealed a large erythematous to violaceous indurated plaque with cobblestone appearance. Biopsy revealed an inflammatory infiltrate of neutrophils with scattered histiocytes, lymphocytes, eosinophils and plasma cells interspersed with areas of lamellar fibrosis and focal areas of vascular damage, suggestive of a localised chronic fibrosing vasculitis of the skin. Localised chronic fibrosing vasculitis is a rare dermatosis, typically presenting as ulcerated violet-red nodules, which can appear histologically similar to erythema elevatum diutinum (EED), which typically presents as red-brown annular plaques. EED may have a predominance of neutrophils and granulomas, while chronic fibrosing vasculitis may have a sparse infiltrate of mixed inflammatory cells without granulomas. While dapsone is a first-line treatment for EED, there are no formal guidelines on the treatment of localised chronic fibrosing vasculitis. Given the neutrophils in this sample and similarities with EED, this patient was treated with oral dapsone, resulting in plaque improvement.

  • Dermatology
  • Skin
  • Pathology

http://dx.doi.org/10.1136/bcr-2022-251515

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    View Full Text

    Footnotes

    • Contributors MMT wrote and edited the manuscript. WML and TRQ assisted in interpreting the clinical and pathological diagnosis and edited the manuscript. STC provided patient care, performed the biopsy, edited and supervised the manuscript.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

    • Competing interests None declared.

    • Provenance and peer review Not commissioned; externally peer reviewed.