ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 2195

Eculizumab Treatment of Malignant Atrophic Papulosis (Köhlmeier-Degos Disease): World Experience to Date

Aixa Toledo-Garcia1, Lee S. Shapiro2,3 and Jessica F. Farrell2,3,4, 1Rheumatology, The Center for Rheumatology, Albany, NY, 2Steffens Scleroderma Center, Saratoga Springs, NY, 3The Center for Rheumatology, Albany, NY, 4Pharmacy Practice, Albany College of Pharmacy & Health Sciences, Albany, NY

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords: Degos Disease and treatment

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

Title: Miscellaneous Rheumatic and Inflammatory Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose

Malignant atrophic papulosis (MAP) is an obliterative vasculopathy which presents with distinctive cutaneous lesions but can progress over months to years to systemic disease with a rapidly fatal course.  Pathologic findings of involved tissues reveal dense deposition of membrane attack complex (MAC).  Eculizumab is a monoclonal antibody which prevents activation of C5 to C5b and C5a.  Within the past five years, based on immunopathology of the disease, eculizumab has been used in MAP.  The relative importance of inhibition of formation of C5a and of inhibition of formation of MAC has not been determined.

Methods

A literature review and personal communication with physicians treating MAP patients throughout the World identified survivors and treatment failures (deaths).  We attempted to identify those characteristics which distinguished the survivors from those who died. 

 

Results  

 We identified eight patients who were treated with eculizumab at different stages of disease and in different circumstances.  Among those eight patients, only three are alive, two for nearly five years since initiation of eculizumab.  Two out of the three survivors presented with GI perforations and cardiovascular decompensation and were placed on eculizumab with an immediate and dramatic response.   They did not receive systemic steroids.    The third live patient presented with CNS involvement affecting the right eye requiring enucleation.  Treprostinil was started after that event, temporarily suppressing cutaneous lesions, but because of gastrointestinal disease progression, eculizumab was later added.  All other patients had received high doses of systemic steroids and may have had bacteremia at the time of treatment with eculizumab.  In addition, one patient had very aggressive dermatomyositis overlap.

Conclusion

Our results suggest that eculizumab is a vital treatment option for patients with rapidly progressive systemic MAP.  These individuals have a life expectancy of less than one year if left untreated.  Treatment benefits likely arise both from the inhibition of C5a formation and from inhibition of formation of membrane attack complex.   Treatment experience to date has been associated with high mortality, which we feel most likely is the consequence of increased risk of bowel perforation and septicemia in those who had already received systemic steroids.   The avoidance of systemic steroid therapy is essential for a good outcome.  Also, earlier identification of those at high risk for bowel perforation should improve outcome by reducing risk of bacteremia developing during treatment.   We report long term survival of several individuals, but in none was eculizumab effective long-term as monotherapy.


Disclosure:

A. Toledo-Garcia,
None;

L. S. Shapiro,
None;

J. F. Farrell,
None.

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to 2014 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/eculizumab-treatment-of-malignant-atrophic-papulosis-kohlmeier-degos-disease-world-experience-to-date/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology