The diagnostic value of skin biopsies in Sneddon syndrome.

<h4>Background</h4>Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin...

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Autores principales:	N L P Starmans, S Zoetemeyer, M R van Dijk, L J Kappelle, C J M Frijns
Formato:	article
Lenguaje:	EN
Publicado:	Public Library of Science (PLoS) 2021
Materias:	Medicine R Science Q
Acceso en línea:	https://doaj.org/article/26f1ccaff37c45feb5e20a602c1feb4d
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spelling	oai:doaj.org-article:26f1ccaff37c45feb5e20a602c1feb4d2021-12-02T20:09:55ZThe diagnostic value of skin biopsies in Sneddon syndrome.1932-620310.1371/journal.pone.0253365https://doaj.org/article/26f1ccaff37c45feb5e20a602c1feb4d2021-01-01T00:00:00Zhttps://doi.org/10.1371/journal.pone.0253365https://doaj.org/toc/1932-6203<h4>Background</h4>Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin biopsies in SS patients.<h4>Materials and methods</h4>We studied skin biopsies from patients with a clinical diagnosis of SS or isolated LR. We also studied controls with vitiligo or normal skin. Biopsies were considered standardized if 3 biopsies were taken from the white centre of the livedo and reached until the dermis-subcutis border. Biopsies were scored for features of an occlusive microangiopathy without knowledge of the clinical features. Sensitivity and specificity of the biopsy findings were calculated with the clinical criteria as the reference standard.<h4>Results</h4>We included 34 SS patients, 14 isolated LR patients and 41 control patients. Biopsies of 17 patients with SS (50%), 4 with isolated LR (31%) and 10 control patients (24%) showed at least one artery in the deep dermis with a thickened vessel wall combined with recanalization or neovascularization (sensitivity 50% and specificity 69% with LR as reference). Standardized biopsies increased the sensitivity to 70%. In a post hoc analysis the combination of an occlusive microangiopathy and the presence of a livedo pattern in the superficial dermis increased the specificity to 92%.<h4>Conclusions</h4>Standardized skin biopsies can support the clinical diagnosis of SS. An occlusive microangiopathy as the only positive criterion for the diagnosis of SS had insufficient specificity for a definite diagnosis.N L P StarmansS ZoetemeyerM R van DijkL J KappelleC J M FrijnsPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 16, Iss 6, p e0253365 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Medicine R Science Q
spellingShingle	Medicine R Science Q N L P Starmans S Zoetemeyer M R van Dijk L J Kappelle C J M Frijns The diagnostic value of skin biopsies in Sneddon syndrome.
description	<h4>Background</h4>Sneddon syndrome (SS) is defined by widespread livedo reticularis (LR) and stroke. There is no single diagnostic test for SS and diagnosis can be solely based on clinical features. This cross-sectional case-control study aimed to determine the diagnostic value of skin biopsies in SS patients.<h4>Materials and methods</h4>We studied skin biopsies from patients with a clinical diagnosis of SS or isolated LR. We also studied controls with vitiligo or normal skin. Biopsies were considered standardized if 3 biopsies were taken from the white centre of the livedo and reached until the dermis-subcutis border. Biopsies were scored for features of an occlusive microangiopathy without knowledge of the clinical features. Sensitivity and specificity of the biopsy findings were calculated with the clinical criteria as the reference standard.<h4>Results</h4>We included 34 SS patients, 14 isolated LR patients and 41 control patients. Biopsies of 17 patients with SS (50%), 4 with isolated LR (31%) and 10 control patients (24%) showed at least one artery in the deep dermis with a thickened vessel wall combined with recanalization or neovascularization (sensitivity 50% and specificity 69% with LR as reference). Standardized biopsies increased the sensitivity to 70%. In a post hoc analysis the combination of an occlusive microangiopathy and the presence of a livedo pattern in the superficial dermis increased the specificity to 92%.<h4>Conclusions</h4>Standardized skin biopsies can support the clinical diagnosis of SS. An occlusive microangiopathy as the only positive criterion for the diagnosis of SS had insufficient specificity for a definite diagnosis.
format	article
author	N L P Starmans S Zoetemeyer M R van Dijk L J Kappelle C J M Frijns
author_facet	N L P Starmans S Zoetemeyer M R van Dijk L J Kappelle C J M Frijns
author_sort	N L P Starmans
title	The diagnostic value of skin biopsies in Sneddon syndrome.
title_short	The diagnostic value of skin biopsies in Sneddon syndrome.
title_full	The diagnostic value of skin biopsies in Sneddon syndrome.
title_fullStr	The diagnostic value of skin biopsies in Sneddon syndrome.
title_full_unstemmed	The diagnostic value of skin biopsies in Sneddon syndrome.
title_sort	diagnostic value of skin biopsies in sneddon syndrome.
publisher	Public Library of Science (PLoS)
publishDate	2021
url	https://doaj.org/article/26f1ccaff37c45feb5e20a602c1feb4d
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The diagnostic value of skin biopsies in Sneddon syndrome.

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