Cargando…

Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance

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 l...

Descripción completa

Detalles Bibliográficos
Autores principales: Shalhout, Sophia Z., Nahas, Myrna R., Drews, Reed E., Miller, David M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7037480/
https://www.ncbi.nlm.nih.gov/pubmed/32099688
http://dx.doi.org/10.1155/2020/7480607
_version_ 1783500437789343744
author Shalhout, Sophia Z.
Nahas, Myrna R.
Drews, Reed E.
Miller, David M.
author_facet Shalhout, Sophia Z.
Nahas, Myrna R.
Drews, Reed E.
Miller, David M.
author_sort Shalhout, Sophia Z.
collection PubMed
description 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.
format Online
Article
Text
id pubmed-7037480
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher Hindawi
record_format MEDLINE/PubMed
spelling pubmed-70374802020-02-25 Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance Shalhout, Sophia Z. Nahas, Myrna R. Drews, Reed E. Miller, David M. Case Rep Dermatol Med Case Report 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. Hindawi 2020-02-12 /pmc/articles/PMC7037480/ /pubmed/32099688 http://dx.doi.org/10.1155/2020/7480607 Text en Copyright © 2020 Sophia Z. Shalhout et al. http://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Shalhout, Sophia Z.
Nahas, Myrna R.
Drews, Reed E.
Miller, David M.
Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title_full Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title_fullStr Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title_full_unstemmed Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title_short Generalized Acquired Cutis Laxa Associated with Monoclonal Gammopathy of Dermatological Significance
title_sort generalized acquired cutis laxa associated with monoclonal gammopathy of dermatological significance
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7037480/
https://www.ncbi.nlm.nih.gov/pubmed/32099688
http://dx.doi.org/10.1155/2020/7480607
work_keys_str_mv AT shalhoutsophiaz generalizedacquiredcutislaxaassociatedwithmonoclonalgammopathyofdermatologicalsignificance
AT nahasmyrnar generalizedacquiredcutislaxaassociatedwithmonoclonalgammopathyofdermatologicalsignificance
AT drewsreede generalizedacquiredcutislaxaassociatedwithmonoclonalgammopathyofdermatologicalsignificance
AT millerdavidm generalizedacquiredcutislaxaassociatedwithmonoclonalgammopathyofdermatologicalsignificance