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A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria

Filename 430. Puhl et al., Scoring system urtic. vasculitis CSU, CTA 2021.pdf
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Date added Juni 1, 2021
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Category Original Work
Tags chronischesponstaneurtikaria, endothelzellschwellung, erythrozyten‐extravasate, fibrin, histopathologie, leukozytoklasie, urtikariavaskulitis
Authors Puhl, V., Bonnekoh, H., Scheffel, J., Hawro, T., Weller, K., von den Driesch, P., Röwert-Huber, H.-J., Cardoso, J., Gonçalo, M., Maurer, M., and Krause, K.
Citation Puhl, V., Bonnekoh, H., Scheffel, J., Hawro, T., Weller, K., von den Driesch, P., Röwert-Huber, H.-J., Cardoso, J., Gonçalo, M., Maurer, M., and Krause, K.: A novel histopathological scoring system to distinguish urticarial vasculitis from chronic spontaneous urticaria. Clin. Transl. Allergy 2021: 11; e12031.
Corresponding authors Krause, K.
DocNum o.430
DocType PDF
IF TBD (IF 2019: 5.13)
Publisher Clin. Transl. Allergy
ReleaseDate 2021

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 stan- dardized. Moreover, there seems to be considerable overlap with chronic sponta- neous 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 diag- nosis 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.

(Last update: 05.2021)

Number of publications (original work and reviews) in peer-reviewed journals: 636
Number of original publications in peer-reviewed journals: 462
Number of reviews in peer-reviewed journals: 174
Cumulative IF of publications (original work & reviews) in peer-reviewed journals: 3834,12
Cumulative IF for original publications in peer-reviewed journals: 3043,14
Cumulative IF for reviews in peer-reviewed journals: 790,98
Citations, Hirsch index: (view on Web of Science) 26429