A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria
Abstract Background Urticarial vasculitis (UV) is defined by long‐lasting urticarial lesions combined with the histopathologic findings of leukocytoclastic vasculitis. As one of the major unmet needs in UV, diagnostic criteria are rather vague and not standardized. Moreover, there seems to be consid...
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2021-04-01
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Online Access: | https://doi.org/10.1002/clt2.12031 |
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doaj-0df84610ce1047e79e9129d4832e71362021-09-02T18:57:41ZengWileyClinical and Translational Allergy2045-70222021-04-01112n/an/a10.1002/clt2.12031A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticariaViktoria Puhl0Hanna Bonnekoh1Jörg Scheffel2Tomasz Hawro3Karsten Weller4Peter von den Driesch5Hans‐Joachim Röwert‐Huber6José Cardoso7Margarida Gonçalo8Marcus Maurer9Karoline Krause10Dermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDepartment of Dermatology Klinikum Stuttgart Stuttgart GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDepartment of Dermatology and Venereology University Hospital and Faculty of Medicine University of Coimbra Coimbra PortugalDepartment of Dermatology and Venereology University Hospital and Faculty of Medicine University of Coimbra Coimbra PortugalDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyDermatological Allergology Allergie‐Centrum‐Charité Department of Dermatology and Allergy Charité – Universitätsmedizin Berlin Berlin GermanyAbstract Background Urticarial vasculitis (UV) is defined by long‐lasting urticarial lesions combined with the histopathologic findings of leukocytoclastic vasculitis. As one of the major unmet needs in UV, diagnostic criteria are rather vague and not standardized. Moreover, there seems to be considerable overlap with chronic spontaneous urticaria (CSU), particularly for the normocomplementemic variant of UV. Therefore, this study aimed to develop a diagnostic scoring system that improves the histopathologic discrimination between UV and CSU. Methods Lesional skin sections of patients with clinical and histopathologic diagnosis of UV (n = 46) and CSU (n = 51) were analyzed (blinded to the diagnosis) for the following pre‐defined criteria: presence of leukocytoclasia, erythrocyte extravasation, fibrin deposits, endothelial cell swelling, ectatic vessels, blurred vessel borders, dermal edema, intravascular neutrophil, and eosinophil numbers and numbers of dermal neutrophils, macrophages and mast cells. Results The greatest differences between UV and CSU samples were observed for leukocytoclasia (present in 76% of UV vs. 3.9% of CSU samples; p < 0.0001), erythrocyte extravasation (present in 41.3% of UV vs. 2.0% of CSU samples; p < 0.0001), and fibrin deposits (present in 27.9% of UV vessels vs. 9.7% of CSU vessels; p < 0.0001). Based on these findings, we developed a diagnostic score, the urticarial vasculitis score (UVS), which correctly assigned 37 of 46 cases of UV and 49 of 51 cases of CSU to the previously established diagnosis. Conclusion Our results suggest that the UVS, a combined quantitative assessment of the three criteria leukocytoclasia, fibrin deposits and extravasated erythrocytes, distinguishes UV from CSU in skin histopathology. The UVS, if validated in larger patient samples, may help to improve the diagnostic approach to UV.https://doi.org/10.1002/clt2.12031chronische sponstane urtikariaendothelzellschwellungerythrozyten‐extravasatefibrinhistopathologieleukozytoklasie |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Viktoria Puhl Hanna Bonnekoh Jörg Scheffel Tomasz Hawro Karsten Weller Peter von den Driesch Hans‐Joachim Röwert‐Huber José Cardoso Margarida Gonçalo Marcus Maurer Karoline Krause |
spellingShingle |
Viktoria Puhl Hanna Bonnekoh Jörg Scheffel Tomasz Hawro Karsten Weller Peter von den Driesch Hans‐Joachim Röwert‐Huber José Cardoso Margarida Gonçalo Marcus Maurer Karoline Krause A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria Clinical and Translational Allergy chronische sponstane urtikaria endothelzellschwellung erythrozyten‐extravasate fibrin histopathologie leukozytoklasie |
author_facet |
Viktoria Puhl Hanna Bonnekoh Jörg Scheffel Tomasz Hawro Karsten Weller Peter von den Driesch Hans‐Joachim Röwert‐Huber José Cardoso Margarida Gonçalo Marcus Maurer Karoline Krause |
author_sort |
Viktoria Puhl |
title |
A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
title_short |
A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
title_full |
A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
title_fullStr |
A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
title_full_unstemmed |
A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
title_sort |
novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria |
publisher |
Wiley |
series |
Clinical and Translational Allergy |
issn |
2045-7022 |
publishDate |
2021-04-01 |
description |
Abstract Background Urticarial vasculitis (UV) is defined by long‐lasting urticarial lesions combined with the histopathologic findings of leukocytoclastic vasculitis. As one of the major unmet needs in UV, diagnostic criteria are rather vague and not standardized. Moreover, there seems to be considerable overlap with chronic spontaneous urticaria (CSU), particularly for the normocomplementemic variant of UV. Therefore, this study aimed to develop a diagnostic scoring system that improves the histopathologic discrimination between UV and CSU. Methods Lesional skin sections of patients with clinical and histopathologic diagnosis of UV (n = 46) and CSU (n = 51) were analyzed (blinded to the diagnosis) for the following pre‐defined criteria: presence of leukocytoclasia, erythrocyte extravasation, fibrin deposits, endothelial cell swelling, ectatic vessels, blurred vessel borders, dermal edema, intravascular neutrophil, and eosinophil numbers and numbers of dermal neutrophils, macrophages and mast cells. Results The greatest differences between UV and CSU samples were observed for leukocytoclasia (present in 76% of UV vs. 3.9% of CSU samples; p < 0.0001), erythrocyte extravasation (present in 41.3% of UV vs. 2.0% of CSU samples; p < 0.0001), and fibrin deposits (present in 27.9% of UV vessels vs. 9.7% of CSU vessels; p < 0.0001). Based on these findings, we developed a diagnostic score, the urticarial vasculitis score (UVS), which correctly assigned 37 of 46 cases of UV and 49 of 51 cases of CSU to the previously established diagnosis. Conclusion Our results suggest that the UVS, a combined quantitative assessment of the three criteria leukocytoclasia, fibrin deposits and extravasated erythrocytes, distinguishes UV from CSU in skin histopathology. The UVS, if validated in larger patient samples, may help to improve the diagnostic approach to UV. |
topic |
chronische sponstane urtikaria endothelzellschwellung erythrozyten‐extravasate fibrin histopathologie leukozytoklasie |
url |
https://doi.org/10.1002/clt2.12031 |
work_keys_str_mv |
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