Case Report: 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

Lydia Lee; Emma Fosbury; Rakesh Popat; Kwee Yong; Ashutosh Wechalekar; Jeremy S. Brown; Jeffrey Huang; Sandy Mcbride; John R. Hurst

doi:10.3389/frhem.2025.1502322

Case Report: 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

Lydia Lee (Lead / Corresponding author)
, Emma Fosbury
, Rakesh Popat
, Kwee Yong
, Ashutosh Wechalekar
, Jeremy S. Brown
, Jeffrey Huang
, Sandy Mcbride
, John R. Hurst

Research output: Contribution to journal › Article › peer-review

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Abstract

Acquired cutis laxa (ACL) is a rare connective tissue disorder that is characterised by loose, pendulous skin and can be associated with systemic elastolysis and plasma cell dyscrasias (PCD). While myeloma therapy has been used for these patients, experience is limited, particularly when organ dysfunction limits a patient’s tolerance of chemotherapy. We present two cases of ACL with progressive pulmonary emphysema that received treatment for coexisting PCD, resulting in differing outcomes. For the first time, we also monitored desmosine as a direct measurement of elastolysis throughout treatment and suggest that this may be useful for guiding therapy in these rare and complex patients.

Original language	English
Article number	1502322
Number of pages	6
Journal	Frontiers in Hematology
Volume	4
DOIs	https://doi.org/10.3389/frhem.2025.1502322
Publication status	Published - 23 Jun 2025

Keywords

acquired cutis laxa
chemotherapy
desmosine
elastolysis
monoclonal gammopathies

ASJC Scopus subject areas

Hematology
Oncology

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10.3389/frhem.2025.1502322Licence: CC BY

Final published versionFinal published version, 997 KBLicence: CC BY

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Lee, L., Fosbury, E., Popat, R., Yong, K., Wechalekar, A., Brown, J. S., Huang, J., Mcbride, S., & Hurst, J. R. (2025). Case Report: 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. Frontiers in Hematology, 4, Article 1502322. https://doi.org/10.3389/frhem.2025.1502322

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title = "Case Report: 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",

abstract = "Acquired cutis laxa (ACL) is a rare connective tissue disorder that is characterised by loose, pendulous skin and can be associated with systemic elastolysis and plasma cell dyscrasias (PCD). While myeloma therapy has been used for these patients, experience is limited, particularly when organ dysfunction limits a patient{\textquoteright}s tolerance of chemotherapy. We present two cases of ACL with progressive pulmonary emphysema that received treatment for coexisting PCD, resulting in differing outcomes. For the first time, we also monitored desmosine as a direct measurement of elastolysis throughout treatment and suggest that this may be useful for guiding therapy in these rare and complex patients.",

keywords = "acquired cutis laxa, chemotherapy, desmosine, elastolysis, monoclonal gammopathies",

author = "Lydia Lee and Emma Fosbury and Rakesh Popat and Kwee Yong and Ashutosh Wechalekar and Brown, \{Jeremy S.\} and Jeffrey Huang and Sandy Mcbride and Hurst, \{John R.\}",

note = "Publisher Copyright: Copyright {\textcopyright} 2025 Lee, Fosbury, Popat, Yong, Wechalekar, Brown, Huang, Mcbride and Hurst.",

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doi = "10.3389/frhem.2025.1502322",

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Lee, L, Fosbury, E, Popat, R, Yong, K, Wechalekar, A, Brown, JS, Huang, J, Mcbride, S & Hurst, JR 2025, 'Case Report: 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', Frontiers in Hematology, vol. 4, 1502322. https://doi.org/10.3389/frhem.2025.1502322

Case Report: 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. / Lee, Lydia (Lead / Corresponding author); Fosbury, Emma; Popat, Rakesh et al.
In: Frontiers in Hematology, Vol. 4, 1502322, 23.06.2025.

Research output: Contribution to journal › Article › peer-review

TY - JOUR

T1 - Case Report

T2 - 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

AU - Lee, Lydia

AU - Fosbury, Emma

AU - Popat, Rakesh

AU - Yong, Kwee

AU - Wechalekar, Ashutosh

AU - Brown, Jeremy S.

AU - Huang, Jeffrey

AU - Mcbride, Sandy

AU - Hurst, John R.

PY - 2025/6/23

Y1 - 2025/6/23

N2 - Acquired cutis laxa (ACL) is a rare connective tissue disorder that is characterised by loose, pendulous skin and can be associated with systemic elastolysis and plasma cell dyscrasias (PCD). While myeloma therapy has been used for these patients, experience is limited, particularly when organ dysfunction limits a patient’s tolerance of chemotherapy. We present two cases of ACL with progressive pulmonary emphysema that received treatment for coexisting PCD, resulting in differing outcomes. For the first time, we also monitored desmosine as a direct measurement of elastolysis throughout treatment and suggest that this may be useful for guiding therapy in these rare and complex patients.

AB - Acquired cutis laxa (ACL) is a rare connective tissue disorder that is characterised by loose, pendulous skin and can be associated with systemic elastolysis and plasma cell dyscrasias (PCD). While myeloma therapy has been used for these patients, experience is limited, particularly when organ dysfunction limits a patient’s tolerance of chemotherapy. We present two cases of ACL with progressive pulmonary emphysema that received treatment for coexisting PCD, resulting in differing outcomes. For the first time, we also monitored desmosine as a direct measurement of elastolysis throughout treatment and suggest that this may be useful for guiding therapy in these rare and complex patients.

KW - acquired cutis laxa

KW - chemotherapy

KW - desmosine

KW - elastolysis

KW - monoclonal gammopathies

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DO - 10.3389/frhem.2025.1502322

M3 - Article

AN - SCOPUS:105010963658

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JO - Frontiers in Hematology

JF - Frontiers in Hematology

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ER -

Case Report: 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

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