Evidence map›Paper›PMID 40988071›Full record

SynthesisOrphanet journal of rare diseases2025

Tissue and systemic inflammation in dystrophic epidermolysis bullosa: a systematic review and meta-analysis.

Meropi Karakioulaki, Nana-Adjoa Kwarteng, Adriani Nikolakopoulou, Hanning Yang, Moritz Hess, Harald Binder, Kilian Eyerich, Cristina Has

Abstract readSystematic ReviewNetwork Meta-Analysis
In one paragraph

Synthesis in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
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

3 citing papers in PubMed.

  1. Trial
  2. Review
  3. Article
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

8 authors.

Meropi KarakioulakiDepartment of Dermatology and Venereology, Faculty of Medicine and Medical Center, University Hospital Freiburg, Hauptstraße 7, 79104, Freiburg, Germany. meropi.karakioulaki@uniklinik-freiburg.de.ORCID 0000-0002-1067-8582
Nana-Adjoa KwartengInstitute of Medical Biometry and Statistics, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
Adriani NikolakopoulouInstitute of Medical Biometry and Statistics, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
Hanning YangInstitute of Medical Biometry and Statistics, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
Moritz HessInstitute of Medical Biometry and Statistics, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
Harald BinderInstitute of Medical Biometry and Statistics, Faculty of Medicine and Medical Center, University of Freiburg, Freiburg, Germany.
Kilian EyerichDepartment of Dermatology and Venereology, Faculty of Medicine and Medical Center, University Hospital Freiburg, Hauptstraße 7, 79104, Freiburg, Germany.
Cristina HasDepartment of Dermatology and Venereology, Faculty of Medicine and Medical Center, University Hospital Freiburg, Hauptstraße 7, 79104, Freiburg, Germany. cristina.has@uniklinik-freiburg.de.ORCID 0000-0001-6066-507X

Funding

Deutsche Forschungsgemeinschaft (DFG- German Research Foundation)
6 · The paper itself

Abstract

backgroundDystrophic epidermolysis bullosa (DEB) is a rare inherited skin disorder caused by mutations in the type VII collagen gene, leading to mucocutaneous blistering. Subsequent inflammation contributes to chronic wounds, scarring, and systemic complications. There is controversy over whether and how inflammation should be therapeutically targeted.

objectiveThis systematic review and meta-analysis aim to question tissue and systemic inflammation in DEB and identify inflammatory patterns and research gaps to improve patient management.

methodsA comprehensive search of MEDLINE via PubMed was conducted to identify studies examining "DEB and tissue or systemic inflammation". Out of 663 studies identified, 37 met the inclusion criteria. Data for synthesis were extracted from studies assessing systemic inflammatory parameter levels in DEB patients. For outcomes with multiple available studies, we performed an exploratory network meta-analysis to compare the standardized mean difference in systemic inflammatory parameters across three patient groups: DEB patients, healthy controls, and patients with other types of epidermolysis bullosa (EB).

resultsThe point estimate results for IL-4, IL-6, tumor necrosis factor-alpha, C-reactive protein, immunoglobulin (Ig) A, IgG, and IgM, as well as anti-collagen VII, anti-BP230, anti-BP180 autoantibodies suggested elevated values in DEB patients compared to healthy patients or other EB patients. The estimated standardized mean differences showed lower values of interleukin (IL)-10, hemoglobin and serum albumin in DEB patients compared to controls or other EB patients.

conclusionCurrent evidence is limited by small and heterogeneous patient cohorts, variability in study designs and reporting methods, and a predominant reliance on observational and retrospective descriptive studies. Well-designed clinical trials and prospective studies are necessary to further investigate inflammatory pathways and assess the efficacy of (targeted) anti-inflammatory therapies but are difficult to perform and cost-intensive. AI tools for small-data may support research in this field. PROSPERO Registration Number CRD42024535352.

Indexed as

Epidermolysis Bullosa DystrophicaInflammationCollagen Type VIIHumansCollagen Type VIIAutoantibodiesCytokinesDystrophic epidermolysis bullosaEpidermolysis bullosaNetwork meta-analysisSystemic inflammationTissue inflammation

Identifiers

PMID40988071
PMCPMC12455789

What OpenQuestion holds

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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.