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Disabling pansclerotic morphoea: a century of discovery
Vivian K Hua1, Johanna Chang2,3, Ronald M Laxer4,5
1Department of Pediatrics, University of California-San Diego, La Jolla, CA, USA.
Insights
Disabling pansclerotic morphoea (DPM) is a rare, severe scleroderma affecting children, characterized by rapid, widespread skin and tissue hardening. Current treatments show limited efficacy, and outcomes are not influenced by the number of interventions used.
Area of Science:
- Dermatology
- Rheumatology
- Pediatrics
Background:
- Disabling pansclerotic morphoea (DPM) is a rare, severe systemic inflammatory disorder within the localized scleroderma spectrum.
- Primarily affecting children under 14, DPM involves rapid, circumferential sclerosis extending to deep tissues, causing immobility and high mortality.
- While internal organ fibrosis is typically absent, the aggressive nature necessitates multidisciplinary care, yet current treatments offer limited efficacy.
Purpose of the Study:
- To comprehensively review all reported English-language cases of DPM.
- To summarize common clinical symptoms, diagnostic findings, and therapeutic interventions for DPM.
- To analyze treatment efficacy and patient outcomes in DPM.
Main Methods:
- A systematic literature search was performed using PubMed and Google Scholar.
- All English-language publications, including original articles, case reports, and letters, were reviewed.
- Data extraction focused on diagnosis, clinical presentation, laboratory/histological findings, treatments, and outcomes.
Main Results:
- Eighty-six patients from 52 reports published up to December 2023 were identified.
- The number of treatments administered did not appear to influence disease outcomes.
- Female patients were observed to be younger at the time of death.
Conclusions:
- Early and accurate diagnosis of DPM hinges on clinician familiarity with its common symptoms.
- Expanding knowledge of effective treatments is crucial for improving disease management and mitigating progression.
- Further research into novel therapeutic strategies for DPM is warranted.
Background:
Disabling pansclerotic morphoea (DPM) is a rare systemic inflammatory disorder at the severe end of the localized scleroderma spectrum. It primarily affects children < 14 years old. DPM is characterized by rapid sclerosis with circumferential involvement that frequently extends to the fascia, muscle and bone. Disease progression often involves the development of sclerotic plaques, chronic skin ulcers and painful joint contractures, leading to patient immobility and a high mortality rate. Internal organ fibrosis is typically absent. The aggressive and systemic nature of DPM leads patients to seek multidisciplinary care. Current treatments are targeted toward immunomodulation and measures to preserve mobility while limiting infection, but they often have limited efficacy.
Objectives:
To summarize all patients with DPM reported in the English-language literature, common clinical symptoms, laboratory investigations and treatments reported to date.
Methods:
A literature search was conducted on PubMed and Google Scholar. All English-language original articles, case reports, abstracts and letters to the editor were included. Each publication was reviewed for diagnosis, clinical presentation, available laboratory/histological studies, treatment and outcome.
Results:
We identified 52 reports comprising 86 patients published up to December 2023. Assessment of published cases suggested that the number of treatments does not influence disease outcome and that female patients are younger at the time of reported death.
Conclusions:
Clinician familiarity and awareness of common DPM symptoms are important for an accurate and early diagnosis. Knowledge of treatments that have been reported to be effective in mitigating disease progression may be helpful in expanding the available treatment options.
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