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CASE REPORT article

Front. Hematol.

Sec. Blood Cancer

Volume 4 - 2025 | doi: 10.3389/frhem.2025.1502322

Contrasting outcomes and use of desmosine to monitor two patients treated with plasma cell directed chemotherapy for gammopathy driven pulmonary elastolysis in acquired cutis laxa

Provisionally accepted
Lydia LeeLydia Lee1*Emma FosburyEmma Fosbury2Rakesh PopatRakesh Popat1Kwee YongKwee Yong1Ashu WechalekarAshu Wechalekar1Jeremy S BrownJeremy S Brown3Jeffrey HuangJeffrey Huang4Sandy Robyn McBrideSandy Robyn McBride2John R HurstJohn R Hurst2
  • 1Division of Cancer, University College London Hospitals NHS Foundation Trust, London, London, United Kingdom
  • 2Royal Free London NHS Foundation Trust, London, United Kingdom
  • 3University College London Hospitals NHS Foundation Trust, London, United Kingdom
  • 4University of Dundee, Dundee, Scotland, United Kingdom

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Acquired cutis laxa (ACL) is a rare connective tissue disorder characterised by loose pendulous skin and can be associated with systemic elastolysis and plasma cell dyscrasias (PCD). While myeloma therapy have been used in these patients, experience is limited, particularly where organ dysfunction limits tolerance of chemotherapy. We present 2 cases of ACL presenting with progressive pulmonary emphysema that received treatment targeting a coexisting PCD with differing outcomes. For the first time, we also monitor desmosine as a direct measurement of elastolysis through treatment and suggest this may be useful to guide therapy in these rare and complex patients.

Keywords: monoclonal gammopathies, Acquired cutis laxa, Desmosine, chemotherapy, Elastolysis

Received: 26 Sep 2024; Accepted: 22 May 2025.

Copyright: © 2025 Lee, Fosbury, Popat, Yong, Wechalekar, Brown, Huang, McBride and Hurst. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Lydia Lee, Division of Cancer, University College London Hospitals NHS Foundation Trust, London, NW1 2BU, London, United Kingdom

Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

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