Evidence map›Paper›PMID 33767715›Full record

ArticleFrontiers in immunology2021

Case Report: Pansclerotic Morphea-Clinical Features, Differential Diagnoses and Modern Treatment Concepts.

Sarah Ventéjou, Agnes Schwieger-Briel, Rebecca Nicolai, Stephanie Christen-Zaech, Caroline Schnider, Michael Hofer, Sofia Bogiatzi, Daniel Hohl, Fabrizio De Benedetti, Marie-Anne Morren

Open access · goldAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Frontiers in immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
1.4field-weighted citation impact, top 21% 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

4 citing papers in PubMed, 13 citations in OpenAlex.

  1. Article
  2. Review
  3. Review
  4. 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

10 authors at 3 institutions in 2 countries.

Sarah VentéjouPediatric Dermatology Unit, Department of Pediatrics and Dermatology and Venereology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Agnes Schwieger-BrielDepartment of Dermatology, Pediatric Skin Center, University Children's Hospital Zurich, Zurich, Switzerland.
Rebecca NicolaiDivision of Rheumatology, Istituto di Ricovero e Cura a Carattera Scientifico, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Stephanie Christen-ZaechPediatric Dermatology Unit, Department of Pediatrics and Dermatology and Venereology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Caroline SchniderDepartment of Pediatric Rheumatology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Michael HoferDepartment of Pediatric Rheumatology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Sofia BogiatziLaboratory of Dermato-Histopathology, Department of Dermato-Venereology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Daniel HohlLaboratory of Dermato-Histopathology, Department of Dermato-Venereology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
Fabrizio De BenedettiDivision of Rheumatology, Istituto di Ricovero e Cura a Carattera Scientifico, Ospedale Pediatrico Bambino Gesù, Rome, Italy.
Marie-Anne MorrenPediatric Dermatology Unit, Department of Pediatrics and Dermatology and Venereology, University Hospital Lausanne and University of Lausanne, Lausanne, Switzerland.
University of Lausanne · CHBambino Gesù Children's Hospital · ITUniversity Children's Hospital Zurich · CH

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Pansclerotic morphea (PSM) is a rare skin disease characterized by progressive stiffening of the skin with or without the typical superficial skin changes usually seen in morphea (localized scleroderma). Standard therapy, consisting of a combination of systemic glucocorticoids and methotrexate or mycophenolate mofetil, does rarely stop disease progression, which may lead to severe cutaneous sclerosis and secondary contractures. Little is known about the efficacy of newer biologicals such as abatacept, a fusion protein antibody against CTLA-4, or tocilizumab, a fully humanized IL-6R antibody, in the treatment of this pathology. We present the case of an 8 years old girl with an unusual, progressive stiffening of the skin, which was eventually diagnosed as pansclerotic morphea. A treatment with systemic glucocorticoids and methotrexate combined with tocilizumab led to a good clinical response within 2 months after initiation. In this paper, we discuss differential diagnoses to be considered and this new promising treatment option based on a case review of the literature.

Indexed as

BiomarkersBiopsyChildDiagnosis, DifferentialDisease ManagementFemaleHumansImmunohistochemistryImmunosuppressive AgentsMagnetic Resonance ImagingScleroderma, LocalizedSkinSkin DiseasesSymptom AssessmentTreatment OutcomeBiomarkersImmunosuppressive Agentscase reportIL-6pansclerotic morpheasclerodermastiff skintocilizumab

Identifiers

PMID33767715
PMCPMC7985437
OpenAlexW3133740838

What OpenQuestion holds

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LicenceCC BY
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Registered trials

None linked

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.