Evidence map›Paper›PMID 42696396›Full record

ArticlePediatric dermatology2026

Epidermolysis Bullosa Simplex-Severe Caused by KRT5 p.Glu477Lys: Challenges Encountered in This High-Risk Subtype.

Nataliia Zhovta, Joana Lanz, Bettina Hafner, Karin Willi, Sven-Eric Baller, Stephanie Adzikah, Martin Theiler, Lisa Weibel, Isabelle Luchsinger, Kathrin Neuhaus and 6 more

Abstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Pediatric dermatology, 2026. 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
–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

0 citing papers in PubMed.

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

16 authors.

Nataliia ZhovtaDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Joana LanzDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Bettina HafnerDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Karin WilliDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Sven-Eric BallerNeonatology Department, University Children's Hospital Zurich, Zurich, Switzerland.
Stephanie AdzikahNeonatology Department, University Children's Hospital Zurich, Zurich, Switzerland.
Martin TheilerDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Lisa WeibelDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Isabelle LuchsingerDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Kathrin NeuhausChildren's Research Center (CRC), University Children's Hospital Zurich, University of Zurich, Zurich, Switzerland.ORCID https://orcid.org/0000-0003-2438-1779
Simon KüpperDivision of Plastic and Reconstructive Surgery, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Christa RellyChildren's Research Center (CRC), University Children's Hospital Zurich, University of Zurich, Zurich, Switzerland.
Jörg ThomasChildren's Research Center (CRC), University Children's Hospital Zurich, University of Zurich, Zurich, Switzerland.
Eva BergsträsserChildren's Research Center (CRC), University Children's Hospital Zurich, University of Zurich, Zurich, Switzerland.
Nicole KnöpfelDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.ORCID https://orcid.org/0000-0002-6438-6550
Agnes Schwieger-BrielDermatology Department, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.ORCID https://orcid.org/0000-0001-7679-3981

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Epidermolysis bullosa simplex-severe (EBS-severe) caused by KRT5 p.Glu477Lys is a rare and particularly severe subtype associated with high neonatal morbidity and mortality. We report an infant who during the neonatal period required prolonged multidisciplinary intensive care for the management of several complications, including extensive wounds with blood loss leading to secondary anemia, failure to thrive with gastroesophageal reflux, respiratory distress with stridor and a necrotizing soft tissue infection, ultimately requiring surgical debridement and grafting. Pain control proved inadequate despite continuous morphine infusion, leading to initiation of methadone, which provided effective and stable analgesia. This case underscores the severe multisystem morbidity of KRT5 p.Glu477Lys-associated GS-EBS and underscores the role of methadone in effective pain management in critically affected EB neonates.

Indexed as

Epidermolysis Bullosa SimplexKeratin-5DebridementHumansInfant, NewbornKeratin-5

Identifiers

PMID42696396
PMCPMC13544513

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.