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Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance.
Case Rep Dermatol Med. 2020; 2020:7480607.CR

Abstract

Background

Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis, systemic lupus erythematosus, and plasma-cell dyscrasias. Case Presentation. We report an unusual case of a 35-year-old male with progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory eruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His cutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by aortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone and high-dose prednisone, respectively, with no improvement to his cutis laxa.

Conclusions

The presence of monoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new term for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance, or MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including scleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that exemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of dermatological significance.

Authors+Show Affiliations

Division of Hematology/Oncology and Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.Division of Hematology/Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.Division of Hematology/Oncology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA.Division of Hematology/Oncology and Department of Dermatology, Massachusetts General Hospital, Harvard Medical School, Boston, MA 02114, USA.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32099688

Citation

Shalhout, Sophia Z., et al. "Generalized Acquired Cutis Laxa Associated With Monoclonal Gammopathy of Dermatological Significance." Case Reports in Dermatological Medicine, vol. 2020, 2020, p. 7480607.
Shalhout SZ, Nahas MR, Drews RE, et al. Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance. Case reports in dermatological medicine. 2020;2020:7480607.
Shalhout, S. Z., Nahas, M. R., Drews, R. E., & Miller, D. M. (2020). Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance. Case Reports in Dermatological Medicine, 2020, 7480607. https://doi.org/10.1155/2020/7480607
Shalhout SZ, et al. Generalized Acquired Cutis Laxa Associated With Monoclonal Gammopathy of Dermatological Significance. Case reports in dermatological medicine. 2020;2020:7480607. PubMed PMID: 32099688.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance. AU - Shalhout,Sophia Z, AU - Nahas,Myrna R, AU - Drews,Reed E, AU - Miller,David M, Y1 - 2020/02/12/ PY - 2019/10/18/received PY - 2020/01/22/revised PY - 2020/01/30/accepted PY - 2020/2/27/entrez PY - 2020/2/27/pubmed PY - 2020/2/27/medline SP - 7480607 EP - 7480607 JF - Case reports in dermatological medicine VL - 2020 N2 - Background: Cutis laxa is a rare dermatosis that is inherited or acquired and clinically features loose, wrinkled, and redundant skin with decreased elasticity. This heterogeneous connective tissue disorder may be localized or generalized, with or without internal manifestations. Generalized cutis laxa often has a cephalocaudal progression and is attributed to inflammatory cutaneous eruptions, medications, and infections. Cutis laxa is also associated with several other conditions including rheumatoid arthritis, systemic lupus erythematosus, and plasma-cell dyscrasias. Case Presentation. We report an unusual case of a 35-year-old male with progression of generalized acquired cutis laxa and vasculitis that occurred over a period of one year. No cutaneous inflammatory eruption preceded or accompanied his decreased skin elasticity, and a biopsy of the skin showed elastolysis. His cutaneous manifestation led to systemic evaluation and an eventual diagnosis of smoldering multiple myeloma accompanied by aortitis and anemia. His myeloma and vasculitis were successfully treated with cyclophosphamide, bortezomib, and dexamethasone and high-dose prednisone, respectively, with no improvement to his cutis laxa. Conclusions: The presence of monoclonal gammopathy is strongly associated with several dermatological entities such as acquired cutis laxa. We propose a new term for the dermatological manifestations caused by paraproteinemia: monoclonal gammopathy of dermatological significance, or MGODS, and stress the evaluation of an underlying gammopathy in the setting of certain dermatologic conditions, including scleromyxedema and amyloidosis. We present a case of a newly acquired cutis laxa secondary to plasma-cell dyscrasias that exemplifies MGODS, alongside a brief literature review, and underscore the clinical relevance of monoclonal gammopathies of dermatological significance. SN - 2090-6463 UR - https://www.unboundmedicine.com/medline/citation/32099688/Generalized_Acquired_Cutis_Laxa_Associated_with_Monoclonal_Gammopathy_of_Dermatological_Significance_ L2 - https://doi.org/10.1155/2020/7480607 DB - PRIME DP - Unbound Medicine ER -
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