Evidence map›Paper›PMID 36847829›Full record

ArticleArchives of dermatological research2023

Correlation of systemic involvement and presence of pathological skin calcification assessed by ex vivo nonlinear microscopy in Pseudoxanthoma elasticum.

Luca Fésűs, Norbert Kiss, Klára Farkas, Dóra Plázár, Sára Pálla, Nastassia Navasiolava, Lili Róbert, Norbert M Wikonkál, Ludovic Martin, Márta Medvecz

Open access · hybridAbstract read
In one paragraph

Article in Archives of dermatological research, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

0numbers the graph read from it
0cells of the map it votes in
0citing papers in PubMed
0.6field-weighted citation impact, top 25% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

0 citing papers in PubMed, 2 citations in OpenAlex.

No citing paper in PubMed yet.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 2 institutions in 2 countries.

Luca FésűsDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0003-2196-3896
Norbert KissDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0002-9947-1755
Klára FarkasDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0002-8185-1346
Dóra PlázárDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0001-9301-6703
Sára PállaDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0002-9614-4724
Nastassia NavasiolavaPXE National Reference Centre, Angers University Hospital, 4 Rue Larrey, 49100, Angers, France.ORCID http://orcid.org/0000-0002-6405-6498
Lili RóbertDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0002-2495-1164
Norbert M WikonkálDepartment of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary.ORCID http://orcid.org/0000-0003-4949-8711
Ludovic Martin *PXE National Reference Centre, Angers University Hospital, 4 Rue Larrey, 49100, Angers, France.ORCID http://orcid.org/0000-0002-4325-6765
Márta Medvecz *Department of Dermatology, Venereology and Dermatooncology, Semmelweis University, Mária Street 41, Budapest, 1085, Hungary. medvecz.marta@med.semmelweis-univ.hu.ORCID http://orcid.org/0000-0002-3126-096X
Semmelweis University · HUUniversité d'Angers · FR

Funding

Development of scientific workshops of medical, health sciences and pharmaceutical educations of the European Structural and Investment Funds EFOP-3.6.3-VEKOP-16-2017-00009EuroSoftCalcNet COST action CA16115Ministry For Innovation and Technology ÚNKP-20-3-I-SE-24Ministry For Innovation and Technology ÚNKP-20-4-II-SE-7Ministry For Innovation and Technology ÚNKP-21-4-II-SE-10Nemzeti Kutatási, Fejlesztési és Innovaciós Alap EFOP-3.6.3-VEKOP-16-2017-00009Nemzeti Kutatási, Fejlesztési és Innovaciós Alap FK_131916
6 · The paper itself

Abstract

Pseudoxanthoma elasticum (PXE (OMIM 264800)) is an autosomal recessive connective tissue disorder mainly caused by mutations in the ABCC6 gene. PXE results in ectopic calcification primarily in the skin, eye and blood vessels that can lead to blindness, peripheral arterial disease and stroke. Previous studies found correlation between macroscopic skin involvement and severe ophthalmological and cardiovascular complications. This study aimed to investigate correlation between skin calcification and systemic involvement in PXE. Ex vivo nonlinear microscopy (NLM) imaging was performed on formalin fixed, deparaffinized, unstained skin sections to assess the extent of skin calcification. The area affected by calcification (CA) in the dermis and density of calcification (CD) was calculated. From CA and CD, calcification score (CS) was determined. The number of affected typical and nontypical skin sites were counted. Phenodex + scores were determined. The relationship between the ophthalmological, cerebro- and cardiovascular and other systemic complications and CA, CD and CS, respectively, and skin involvement were analyzed. Regression models were built for adjustment to age and sex. We found significant correlation of CA with the number of affected typical skin sites (r = 0.48), the Phenodex + score (r = 0.435), extent of vessel involvement (V-score) (r = 0.434) and disease duration (r = 0.48). CD correlated significantly with V-score (r = 0.539). CA was significantly higher in patients with more severe eye (p = 0.04) and vascular (p = 0.005) complications. We found significantly higher CD in patients with higher V-score (p = 0.018), and with internal carotid artery hypoplasia (p = 0.045). Significant correlation was found between higher CA and the presence of macula atrophy (β = - 0.44, p = 0.032) and acneiform skin changes (β = 0.40, p = 0.047). Based on our results, the assessment of skin calcification pattern with nonlinear microscopy in PXE may be useful for clinicians to identify PXE patients who develop severe systemic complications.

Indexed as

Connective TissuePseudoxanthoma ElasticumSkinAdultAgedATP-Binding Cassette, Sub-Family C ProteinsCalcification, PhysiologicElastinFemaleHumansMaleMiddle AgedMutationRetrospective StudiesABCC6 protein, humanATP-Binding Cassette, Sub-Family C ProteinsElastinCalcium depositEctopic calcificationElastinMultiphoton microscopyNonlinear optical microscopyPseudoxanthoma elasticum

Identifiers

PMID36847829
PMCPMC10366029
OpenAlexW4322496262

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.