Evidence map›Paper›PMID 30838436›Full record

ReviewRheumatology international2019

Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review.

Han Jie Soh, Courtney Samuel, Victoria Heaton, William Douglas Renton, Angela Cox, Jane Munro

Abstract readCase ReportsReview
PubMed Publisher
In one paragraph

Review in Rheumatology international, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed, 1 pooled it
2.5field-weighted citation impact, top 11% 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

13 citing papers in PubMed, 1 synthesis or guideline pooled it, 35 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Sclerosing diseases of the skin.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2025
    Review
  4. Review
  5. [Juvenile localized scleroderma].Dermatologie (Heidelberg, Germany) · 2024
    Review
  6. Recent Advances in Treatment of Systemic Sclerosis and Morphea.American journal of clinical dermatology · 2024
    Review
  7. VariantThe New England journal of medicine · 2023
    Article
  8. Morphea: The 2023 update.Frontiers in medicine · 2023
    Review
  9. Upcoming treatments for morphea.Immunity, inflammation and disease · 2021
    Review
  10. Article
  11. Article
  12. Review
  13. Review
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

6 authors at 1 institution in 1 country.

Han Jie SohPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia. hanjie.soh@gmail.com.ORCID 0000-0002-1079-4989
Courtney SamuelPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.ORCID 0000-0002-9052-2085
Victoria HeatonPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.ORCID 0000-0001-5153-3171
William Douglas RentonPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.ORCID 0000-0002-9631-4455
Angela CoxPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.ORCID 0000-0002-5221-8517
Jane MunroPaediatric Rheumatology Unit, The Royal Children's Hospital, Parkville, VIC, Australia.ORCID 0000-0003-3018-2194
Royal Children's Hospital · AU

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Disabling pansclerotic morphea of childhood (DPMC) is a rare subtype of juvenile localized scleroderma (JLS) characterized by pansclerosis mainly affecting children under the age of 14. This aggressive disease has a poor prognosis due to the rapid progression of deep musculoskeletal atrophy resulting in cutaneous ulceration and severe joint contractures. We describe the challenges in treating a previously well 5-year-old male who has refractory symptoms of DPMC. Over the 29 months, since his initial presentation, we trialed over ten therapies. There was subjective improvement with prednisolone and mycophenolate mofetil (MMF). However, other therapies including biologics and tyrosine kinase inhibitors (TKI) were ineffective. The patient has been referred for hematopoietic stem cell transplant given ongoing disease progression. We conducted a literature search focusing on English articles with keywords including DPMC. Publications with limited information or describing cases aged 20 and above were excluded. Thirty-seven case reports were identified and the reported treatments were evaluated. Methotrexate and corticosteroids have been the most commonly utilized. MMF has been anecdotally effective. Biologics, TKI, and Janus kinase inhibitors lack evidence in DPMC, but have had demonstrated efficacy in similar pathologies including systemic sclerosis, and, thus, have been used for DPMC. Phototherapy has been documented to be reducing skin thickness and stiffness of plaques. Eventually, most children require multi-modal and high-dose immunosuppressive therapies to reduce the inflammation inflicted by the disease. Long-term antibiotics and nutritional support are important in the ongoing care of these patients.

Indexed as

Antirheumatic AgentsBiological ProductsBiopsyChild, PreschoolContractureEdemaHematopoietic Stem Cell TransplantationHumansHydroxychloroquineImmunoglobulins, IntravenousImmunologic FactorsImmunosuppressive AgentsJanus Kinase InhibitorsMaleMethylprednisoloneMycophenolic AcidAntirheumatic AgentsBiological ProductsHydroxychloroquineImmunoglobulins, IntravenousImmunologic FactorsImmunosuppressive AgentsJanus Kinase InhibitorsMethylprednisoloneMycophenolic AcidPrednisoloneProtein Kinase InhibitorsChildrenDisabling pansclerotic morphea of childhoodPediatricTreatment

Identifiers

PMID30838436
OpenAlexW2920218937

What OpenQuestion holds

Textmetadata
Read underepoch 390

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.