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Commentary Open Access
Volume 3 | Issue 1 | DOI: https://doi.org/10.46439/hematol.3.018

Acquired cutis laxa: Hematological insights and therapeutic implications in a paraneoplastic context

  • 1University of Balamand, Faculty of Medicine and Medical Sciences, Department of Dermatology at Saint Georges Hospital University Medical Center, Lebanon
  • 2University of Balamand, Faculty of Medicine and Medical Sciences, Department of Family medicine at Saint Georges Hospital University Medical Center, Lebanon
+ Affiliations - Affiliations

Corresponding Author

 Joe Khodeir, joe.khodeir@std.balamand.edu.lb

Received Date: July 14, 2024

Accepted Date: July 25, 2024

Abstract

Background: Acquired cutis laxa (ACL) is an uncommon dermatological condition characterized by loose, inelastic skin due to the degradation of elastic fibers. Traditionally studied within dermatology, recent insights have highlighted significant associations with hematological disorders, particularly monoclonal gammopathies.
Objective: This commentary extends the discussion presented in the review article "Acquired cutis laxa: a clinical review" by Khodeir et al., emphasizing recent advancements in the understanding of ACL’s hematological connections. It focuses on paraneoplastic associations and their treatment, alongside a newly documented case linking ACL with IgA gammopathy.

Methods: A comprehensive review of recent literature and new cumulative data on ACL associated with neoplastic disorders was conducted. A detailed case report illustrates the clinical and pathological findings of ACL associated with IgA lambda paraproteinemia.

Results: Among 110 patients reviewed, 30 cases (27%) were linked to paraneoplastic disorders, primarily multiple myeloma and monoclonal gammopathy of undetermined significance. ACL typically preceded the diagnosis of these conditions by an average of 2.4 years. The newly reported case of ACL with IgA gammopathy further reinforces the link between ACL and monoclonal gammopathies. The pathogenesis involves complex mechanisms including enzymatic degradation of elastic fibers, immunoglobulin-mediated elastolysis, and chronic inflammation.

Conclusion: ACL presents a dermatological challenge increasingly linked to hematological disorders, particularly monoclonal gammopathies. Recognizing ACL as a potential paraneoplastic marker underscores the need for thorough hematological evaluation and long-term follow-up in patients. Continued research into the pathogenesis and therapeutic strategies is crucial for improving clinical outcomes and refining management guidelines for ACL.

Keywords

Acquired cutis laxa, Acquired elastolysis, paraneoplastic skin changes, Monoclonal gammopathy of cutaneous significance

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