Degos disease: a case report and review of the literature

BACKGROUND: Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. CASE PRESENTATION: A 35-year-old Hind...

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Autores principales: Tummidi, Santosh, Nagendran, Prabhakaran, Gedela, Swaroopa, Ramani, Jami Rupa, Shankaralingappa, Arundhathi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7594340/
https://www.ncbi.nlm.nih.gov/pubmed/33115514
http://dx.doi.org/10.1186/s13256-020-02514-6
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author Tummidi, Santosh
Nagendran, Prabhakaran
Gedela, Swaroopa
Ramani, Jami Rupa
Shankaralingappa, Arundhathi
author_facet Tummidi, Santosh
Nagendran, Prabhakaran
Gedela, Swaroopa
Ramani, Jami Rupa
Shankaralingappa, Arundhathi
author_sort Tummidi, Santosh
collection PubMed
description BACKGROUND: Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. CASE PRESENTATION: A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of depigmented painful lesions. A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease. CONCLUSION: The diagnosis of Degos disease is usually based on the presence of the pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of necrosis with thrombotic occlusion of the small arterioles. No specific treatment is currently available for this disease.
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spelling pubmed-75943402020-10-30 Degos disease: a case report and review of the literature Tummidi, Santosh Nagendran, Prabhakaran Gedela, Swaroopa Ramani, Jami Rupa Shankaralingappa, Arundhathi J Med Case Rep Case Report BACKGROUND: Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. CASE PRESENTATION: A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of depigmented painful lesions. A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease. CONCLUSION: The diagnosis of Degos disease is usually based on the presence of the pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of necrosis with thrombotic occlusion of the small arterioles. No specific treatment is currently available for this disease. BioMed Central 2020-10-29 /pmc/articles/PMC7594340/ /pubmed/33115514 http://dx.doi.org/10.1186/s13256-020-02514-6 Text en © The Author(s) 2020 Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Tummidi, Santosh
Nagendran, Prabhakaran
Gedela, Swaroopa
Ramani, Jami Rupa
Shankaralingappa, Arundhathi
Degos disease: a case report and review of the literature
title Degos disease: a case report and review of the literature
title_full Degos disease: a case report and review of the literature
title_fullStr Degos disease: a case report and review of the literature
title_full_unstemmed Degos disease: a case report and review of the literature
title_short Degos disease: a case report and review of the literature
title_sort degos disease: a case report and review of the literature
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7594340/
https://www.ncbi.nlm.nih.gov/pubmed/33115514
http://dx.doi.org/10.1186/s13256-020-02514-6
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