Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis
Behçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases...
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Frontiers Media S.A.
2022-12-01
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| Series: | Frontiers in Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fmed.2022.987393/full |
| _version_ | 1811316330461134848 |
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| author | Doyoung Kim Koichiro Nakamura Fumio Kaneko Erkan Alpsoy Dongsik Bang |
| author_facet | Doyoung Kim Koichiro Nakamura Fumio Kaneko Erkan Alpsoy Dongsik Bang |
| author_sort | Doyoung Kim |
| collection | DOAJ |
| description | Behçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases, such as aortic aneurysm and deep vein thrombosis. The full etiology of BD remains unclear, but it is considered a polygenetic disease with multiple genetic risk factors that promote immune dysregulation and thrombophilia. Inflammation can be triggered by environmental factors, such as bacteria or viruses, and the dysregulation of innate and adaptive immune cell subsets. Neutrophils and lymphocytes are the primary players involved in BD pathogenesis, with specific innate (i.e., neutrophil-derived reactive oxygen species and neutrophil extracellular traps) and adaptive (i.e., anti-endothelial cell antibodies) processes inducing endothelial cell activation and chemotaxis of inflammatory cells, leading to coagulation and vasculitis. These inflammation-induced vasculitic or vasculopathic features are observed in most mucocutaneous BD lesions, although vasculitis per se is often pathologically evident only during a brief period of the disease process. Due to the multifactorial nature of BD-associated inflammation, broad-spectrum anti-inflammatory medications, including glucocorticoids and immunosuppressive drugs, have been the mainstay for managing BD. In addition, inhibitors of interleukin (IL)-1, tumor necrosis factor (TNF)-α, and IL-17, which target innate and adaptive immune functions dysregulated in BD, have emerged as promising new therapeutics. In this review, we discuss the muco-cutaneous manifestations of BD by focusing on the underlying vasculitic components in their pathologies, as well as the current array of treatment options. |
| first_indexed | 2024-04-13T11:48:14Z |
| format | Article |
| id | doaj.art-4b18c861be2b4d71807798d1a380f4a5 |
| institution | Directory Open Access Journal |
| issn | 2296-858X |
| language | English |
| last_indexed | 2024-04-13T11:48:14Z |
| publishDate | 2022-12-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Medicine |
| spelling | doaj.art-4b18c861be2b4d71807798d1a380f4a52022-12-22T02:48:08ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2022-12-01910.3389/fmed.2022.987393987393Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitisDoyoung Kim0Koichiro Nakamura1Fumio Kaneko2Erkan Alpsoy3Dongsik Bang4Department of Dermatology, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, South KoreaDepartment of Dermatology, Saitama Medical University, Saitama, JapanInstitute of Dermato-Immunology and Allergy, Southern Tohoku General Hospital, Fukushima, JapanDepartment of Dermatology and Venereology, School of Medicine, Akdeniz University, Antalya, TurkeyDepartment of Dermatology, Catholic Kwandong University, International St. Mary’s Hospital, Incheon, South KoreaBehçet’s disease (BD) is a systemic inflammatory disorder characterized by vasculitis affecting blood vessels of any caliber or type. It can present with a wide spectrum of vasculitic lesions, including erythema nodosum-like lesions and retinal vasculitis, and may also lead to larger vessel diseases, such as aortic aneurysm and deep vein thrombosis. The full etiology of BD remains unclear, but it is considered a polygenetic disease with multiple genetic risk factors that promote immune dysregulation and thrombophilia. Inflammation can be triggered by environmental factors, such as bacteria or viruses, and the dysregulation of innate and adaptive immune cell subsets. Neutrophils and lymphocytes are the primary players involved in BD pathogenesis, with specific innate (i.e., neutrophil-derived reactive oxygen species and neutrophil extracellular traps) and adaptive (i.e., anti-endothelial cell antibodies) processes inducing endothelial cell activation and chemotaxis of inflammatory cells, leading to coagulation and vasculitis. These inflammation-induced vasculitic or vasculopathic features are observed in most mucocutaneous BD lesions, although vasculitis per se is often pathologically evident only during a brief period of the disease process. Due to the multifactorial nature of BD-associated inflammation, broad-spectrum anti-inflammatory medications, including glucocorticoids and immunosuppressive drugs, have been the mainstay for managing BD. In addition, inhibitors of interleukin (IL)-1, tumor necrosis factor (TNF)-α, and IL-17, which target innate and adaptive immune functions dysregulated in BD, have emerged as promising new therapeutics. In this review, we discuss the muco-cutaneous manifestations of BD by focusing on the underlying vasculitic components in their pathologies, as well as the current array of treatment options.https://www.frontiersin.org/articles/10.3389/fmed.2022.987393/fullBehçet’s diseasevasculitismucocutaneouspathogenesisthrombosis |
| spellingShingle | Doyoung Kim Koichiro Nakamura Fumio Kaneko Erkan Alpsoy Dongsik Bang Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis Frontiers in Medicine Behçet’s disease vasculitis mucocutaneous pathogenesis thrombosis |
| title | Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis |
| title_full | Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis |
| title_fullStr | Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis |
| title_full_unstemmed | Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis |
| title_short | Mucocutaneous manifestations of Behçet’s disease: Pathogenesis and management from perspectives of vasculitis |
| title_sort | mucocutaneous manifestations of behcet s disease pathogenesis and management from perspectives of vasculitis |
| topic | Behçet’s disease vasculitis mucocutaneous pathogenesis thrombosis |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2022.987393/full |
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