Raras
Buscar doenças, sintomas, genes...
Esclerodermia localizada
ORPHA:90289CID-10 · L94.0CID-11 · EB61.0PCDT · SUSDOENÇA RARA

A esclerodermia localizada é a forma localizada de esclerodermia caracterizada por fibrose da pele causando placas ou faixas cutâneas.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A esclerodermia localizada é a forma localizada de esclerodermia caracterizada por fibrose da pele causando placas ou faixas cutâneas.

Pesquisas ativas
6 ensaios
33 total registrados no ClinicalTrials.gov
Publicações científicas
1.243 artigos
Último publicado: 2026 Apr 8

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
All ages
🏥
SUS: Cobertura mínimaScore: 30%
PCDT disponívelCID-10: L94.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧬
Pele e cabelo
10 sintomas
😀
Face
6 sintomas
💪
Músculos
4 sintomas
🦴
Ossos e articulações
3 sintomas
❤️
Coração
2 sintomas
🫃
Digestivo
2 sintomas

+ 17 sintomas em outras categorias

Características mais comuns

90%prev.
Pele espessada
Muito frequente (99-80%)
55%prev.
Artralgia
Frequente (79-30%)
55%prev.
Placa esclerótica cutânea
Frequente (79-30%)
55%prev.
Lesão cutânea localizada
Frequente (79-30%)
55%prev.
Pele rígida
Frequente (79-30%)
17%prev.
Assimetria facial
Ocasional (29-5%)
55sintomas
Muito frequente (1)
Frequente (4)
Ocasional (28)
Muito raro (22)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 55 características clínicas mais associadas, ordenadas por frequência.

Pele espessadaThickened skin
Muito frequente (99-80%)90%
ArtralgiaArthralgia
Frequente (79-30%)55%
Placa esclerótica cutâneaCutaneous sclerotic plaque
Frequente (79-30%)55%
Lesão cutânea localizadaLocalized skin lesion
Frequente (79-30%)55%
Pele rígidaStiff skin
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.243PubMed
Últimos 10 anos200publicações
Pico202578 papers
Linha do tempo
2026Hoje · 2026🧪 1992Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 31
2Fase 24
1Fase 13
·Pré-clínico12
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Esclerodermia localizada

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

5 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

33 ensaios clínicos encontrados, 6 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
567 papers (10 anos)
#1

Methotrexate and Mycophenolate Mofetil and Clinical Response in Juvenile Localized Scleroderma.

JAMA dermatology2026 Mar 01

Currently, there are no treatments approved by the US Food and Drug Administration for juvenile localized scleroderma (JLS), a rare disease. While methotrexate (MTX) is regularly used as a first-line therapy, emerging data from case series and applications in systemic sclerosis suggest that mycophenolate mofetil (MMF) may be clinically comparable, with potential benefits in tolerability and adherence. To compare clinical outcomes of patients with JLS treated with MTX and MMF using standardized clinical outcome measures. This retrospective cohort study of patients with clinician-diagnosed JLS who were enrolled in the National Registry of Childhood Onset Scleroderma was conducted at UPMC Children's Hospital of Pittsburgh. Data were from January 2010 to January 2023 and first analyzed in April 2023. All patients were evaluated and followed up by the same physician. Patients were included if they had disease onset before age 18 years and a localized scleroderma diagnosis and enrollment in the National Registry of Childhood Onset Scleroderma before age 21 years. Patients were required to be receiving MTX monotherapy, MMF monotherapy, or combination therapy (CT) of the 2 for their localized scleroderma. Patients were treated with MTX, MMF, or CT as prescribed by the examining physician. This study measured comparative medication treatment response through associations with disease activity, as measured using the Localized Scleroderma Cutaneous Assessment Tool. Of 114 patients, 77 (67.5%) were female, and the median (IQR) age at onset was 8.3 (5.4-11.2) years. The MTX, MMF, and CT groups included 68 (59.6%), 28 (24.6%), and 18 patients (15.8%), respectively. There were no significant differences in baseline demographic characteristics, disease subtype, or disease severity between groups, but patients in the MMF group had longer disease duration. Mixed-effects modeling showed statistically significant decreases in activity across all groups (β = -0.14; 95% CI, -0.62 to 0.33). A Kaplan-Meier analysis showed no significant difference in disease flare rate over the follow-up interval (hazard ratio, 0.85; 95% CI, 0.51-1.33). However, patients treated with MTX compared with those treated with MMF had significantly higher rates of fatigue (47% vs. 11%, P = .001) and nausea (60% vs. 7%; P = .001). The study results suggest that MMF demonstrated a similar response to treatment as MTX in reducing disease activity in JLS, with comparable flare rates and improved tolerability. These initial findings support MMF as a potential candidate for first-line treatment of JLS. Prospective, randomized, noninferiority trials are warranted to confirm these results and guide future treatment recommendations.

#2

Association of MMP9 and NOS3 Polymorphisms with Distinct Clinical Forms of Juvenile Scleroderma and Characteristics of Humoral Immunity.

International journal of molecular sciences2026 Jan 22

Juvenile scleroderma (JS), comprising localized (JLSd) and systemic (JSSc) forms, is a rare autoimmune disorder. This study investigated associations of polymorphisms in extracellular matrix (MMP1, MMP9) and vascular homeostasis (NOS3) genes with JS risk and immunological phenotypes. A case-control study involved 215JS patients (194 JLSd, 21 JSSc) and 72 controls. SNPs (MMP1 rs1799750, MMP9 rs3918242, NOS3 rs1799983) were genotyped using real-time PCR followed by minisequencing and mass spectrometric analysis of reaction products. Associations with disease risk, subtypes, and immunological markers were analyzed statistically. The MMP9 (rs3918242) CT genotype was significantly associated with JLSd (OR = 2.23, 95% CI: 1.14-4.37, p = 0.022), showing a trend in linear facial forms. The NOS3 (rs1799983) GG genotype demonstrated a trend toward association with JSSc (OR = 2.61, 95% CI: 0.92-7.37, p = 0.065). No significant associations were found for rs1799750 MMP1 and risk of disease development. The MMP9 risk genotype did not correlate with scleroderma-specific autoantibodies, while the NOS3 GG genotype was associated with lower serum levels of anti-collagen IV antibodies (p = 0.039). Genetic associations differ for JS subtypes: MMP9 with JLSd and NOS3 with JSSc. Children with CT polymorphism MMP9 (rs3918242) and with NOS3 (rs1799983) GG genotype were found to be genetically predisposed for the development of JS.

#3

Successful Treatment of Refractory Severe Localized Scleroderma With Anifrolumab.

JAMA dermatology2026 Feb 01

This case report describes a woman in her 20s with a history of linear morphea diagnosed in childhood who presented with disease progression despite long-term treatment.

#4

Remote Skin and Blood Sampling for Translational Connective Tissue Disorder Research: A Proof-of-Concept Pilot Study.

JID innovations : skin science from molecules to population health2026 Jan

Remote, noninvasive biospecimen collection methods can address geographic and mobility-related barriers to participation in translational research for connective tissue disorders. We evaluated the feasibility of combining noninvasive skin tape stripping and Tasso capillary blood collection to molecularly profile lupus and morphea in remote settings using mailed kits. RNA extraction was optimized from 20 serial tape strips per site, with overnight shipping and scraping samples in buffer without sonication, yielding the highest concentration and quality. We enrolled 8 patients with lupus, 3 patients with morphea, and 8 healthy controls. Microarray analysis identified upregulation of CXCL9, CCR6, and PDGFRA in lupus and morphea skin. Proteomic analysis revealed elevated IFN-γ, TNFRSF4, and ANGPT2 in lupus serum, with lower IL-13 than in controls. Morphea samples showed increased expression of markers, including PDGFRA, P2RX1, and KALRN. Distinct gene expression signatures of CTGF, TNFRSF4, and MAFB in lupus skin and ALOX15, KALRN, and P2RX1 in morphea skin enabled differentiation between morphea and lupus. CXCL9 and IFN-γ biomarkers were confirmed in other publicly available datasets at both the protein and RNA levels. These findings provide proof of concept that remote RNA and protein profiling is feasible and reproducibly identifies canonical and, to our knowledge, previously unreported disease markers; however, the small number of patients and the lack of more complete characterization of the participants studied require that further studies are needed to establish the role of these findings in the pathogenesis of lupus and morphea. This approach enables broader inclusion of underserved patients and offers a scalable model for remote biomarker studies in dermatologic and autoimmune research.

#5

A practical clinical approach to the diagnosis and management of morphea (localized scleroderma).

Best practice &amp; research. Clinical rheumatology2026 Mar 20

Morphea, also referred to as localized cutaneous scleroderma, encompasses a heterogeneous group of inflammatory and fibrosing skin disorders characterized by variable depth of tissue involvement and different clinical course. The diagnostic delay remains common due to insidious onset, broad differential diagnosis, and lack of validated biomarkers of disease activity. Clinical assessment relies therefore on expert evaluation, supported by clinical scoring systems and selected imaging techniques. Therapeutic strategies depend on disease subtype, activity, depth, and risk of irreversible damage, ranging from topical agents for limited superficial forms to systemic immunosuppression for deep, linear, generalized, and pansclerotic variants. Methotrexate combined with systemic corticosteroids represents the current first-line systemic therapy, while mycophenolate mofetil and biologics or targeted synthetic agents are increasingly used in refractory disease. This review provides a comprehensive and updated overview of the epidemiology, clinical spectrum, diagnostic approach, and management of morphea across age groups, highlighting recent therapeutic advances, and unmet clinical needs.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC637 artigos no totalmostrando 199

2026

A practical clinical approach to the diagnosis and management of morphea (localized scleroderma).

Best practice &amp; research. Clinical rheumatology
2026

Circumscribed Morphea Successfully Treated With Excimer Laser: Analysis of Histopathological Changes.

The Journal of dermatology
2026

Human MAGI1 expression in endothelial cells protects from the development of localized and systemic scleroderma in mice.

Arthritis research &amp; therapy
2026

Case of generalized morphea complicated with systemic sclerosis.

Dermatology reports
2026

Injectable platelet-rich fibrin as a valid therapeutic option for inactive linear morphea (en coup de saber): report of cases.

The Journal of dermatological treatment
2026

Generalized Morphea Successfully Treated With Tripterygium Glycosides and Hydroxychloroquine: A Case Report and a Systematic Review.

Cureus
2026

Successful tocilizumab-based combination therapy for a case of rapidly progressive adult deep morphea with multiple antiphospholipid antibodies: a case report and literature review.

Frontiers in immunology
2026

Replay: Comparative Study of Adipose-Derived Stem Cells from Localized Scleroderma Patients and Healthy Donors in Treating Skin Fibrosis.

Plastic and reconstructive surgery
2026

Hyaluronic acid fillers in systemic sclerosis and localized scleroderma: A systematic review.

Autoimmunity reviews
2026

Ustekinumab-associated morphoea: systematic review of the literature and a real-world case.

Drugs in context
2026

Atypical Presentation of Morphoea in an Elderly Male: Diagnostic Challenges in the Absence of Autoantibodies and Malignancy.

Cureus
2026

Treatment of juvenile localized scleroderma with upadacitinib: A case series.

Journal of the European Academy of Dermatology and Venereology : JEADV
2026

Methotrexate and Mycophenolate Mofetil and Clinical Response in Juvenile Localized Scleroderma.

JAMA dermatology
2026

Association of MMP9 and NOS3 Polymorphisms with Distinct Clinical Forms of Juvenile Scleroderma and Characteristics of Humoral Immunity.

International journal of molecular sciences
2026

JAK inhibitors for the treatment of juvenile localized scleroderma: a case report and literature review.

Pediatric rheumatology online journal
2026

Botulinum neurotoxin type A1 alleviates bleomycin-induced skin fibrosis by inhibiting phosphatidylinositol 3-kinase-protein kinase B (PI3K-Akt) pathway and associated cell cycle and proliferation.

The Journal of investigative dermatology
2026

Tofacitinib treatment in a girl with localized scleroderma of the head and face: a case-based review.

Rheumatology international
2026

Skin Cancer Arising in Morphea: A Systematic Review.

Journal of cutaneous medicine and surgery
2026

Disease Course and Risk of Relapse in Juvenile Localized Scleroderma: A Single-Center Retrospective Study.

International journal of rheumatic diseases
2025

Combination of Oral Hydroxychloroquine and Topical Pimecrolimus 1% for Linear Morphea in Breastfeeding: A Rare Case with Favorable Outcome.

International journal of women's health
2025

Parry-Romberg Syndrome Associated With En Coup De Sabre: A Clinical Case Report.

Cureus
2025

Single-Cell Profiling Reveals Divergent Mechanisms of Fibrosis in Localized Scleroderma and Systemic Sclerosis.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2025

Phosphodiesterase-4 inhibition in localized scleroderma: A case series of topical roflumilast.

SAGE open medical case reports
2026

Successful Treatment of Refractory Severe Localized Scleroderma With Anifrolumab.

JAMA dermatology
2026

Evaluation of localized scleroderma by line-field confocal optical coherence tomography.

Italian journal of dermatology and venereology
2025

Parry-Romberg Syndrome With Localized Scleroderma: A Report of Two Pediatric Cases From Oman.

Cureus
2025

[En coup de sabre linear scleroderma with central nervous system involvement in a pediatric patient].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2025

Janus Kinase Inhibitors for the Treatment of Pediatric Morphea: A Systematic Review.

Pediatric dermatology
2025

Temporal dynamics of ADSC therapy in skin fibrosis: unraveling the roles of ROS/NF-κB/TSG-6 signaling axis.

Stem cell research &amp; therapy
2026

Remote Skin and Blood Sampling for Translational Connective Tissue Disorder Research: A Proof-of-Concept Pilot Study.

JID innovations : skin science from molecules to population health
2025

Barriers to Surgical Care in Craniofacial Scleroderma (Parry-Romberg Syndrome).

The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association
2026

Localized scleroderma and related comorbidities: a single-centre cohort study.

The British journal of dermatology
2025

Pansclerotic Morphea, Refractory to Multiple Systemic Therapies, Successfully Treated with UVA-1 Phototherapy.

Case reports in dermatology
2025

Are systemic sclerosis and localized scleroderma (morphea) part of a common disease spectrum? A systematic review on their coexistence.

Clinics in dermatology
2025

Pediatric Localized Scleroderma With Favorable Response to 308-nm Excimer Light Therapy.

Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]
2025

Sclerosing diseases of the skin.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2025

Revealing Microvascular Involvement in Pediatric Localized Scleroderma Through Nailfold Capillaroscopy.

Children (Basel, Switzerland)
2025

Injectable Immune-Regulating Mesenchymal Stem Cells Hydrogel for Localized Scleroderma Treatment.

ACS nano
2025

Sclerotic GVHD and scleroderma share dysregulated gene expression that is ameliorated by EREG therapeutic antibody.

Blood
2025

Adipose-derived mesenchymal stem cells and their derivatives in inflammatory skin diseases: a systematic review.

Frontiers in immunology
2025

Reviewing the Developing Significance of the Serine Protease PRSS23.

Frontiers in bioscience (Landmark edition)
2025

Recombinant Human Annexin A5 Ameliorates Localized Scleroderma by Inhibiting the Activation of Fibroblasts and Macrophages.

Pharmaceutics
2025

The Therapeutic Effects of Semaglutide in Congenital Linear Scleroderma.

Cureus
2025

Pirfenidone in Skin Fibrosis and Scarring: From Bench Insights to Clinical Data.

Medical sciences (Basel, Switzerland)
2025

Plastic Surgery Interventions for Localized Scleroderma Deformities: an Evidence Synthesis.

Aesthetic plastic surgery
2025

Comparative Efficacy and Safety of Treatments for Morphea: A Systematic Review.

Journal of cutaneous medicine and surgery
2025

Autoimmune inner ear disease and localized scleroderma in childhood: A case report.

Reumatologia clinica
2025

High-content Image-based Drug Testing of Patients' Primary Fibroblasts Reveals Potential New Treatment Options for Localized Scleroderma.

Acta dermato-venereologica
2025

Nodular morphea on the breast of a pregnant woman-case report and literature review.

Wiener medizinische Wochenschrift (1946)
2025

Asymptomatic Anterior Uveitis without Systemic Activity in en Coup de Sabre Scleroderma: A Case Report.

Ocular immunology and inflammation
2025

Janus kinase inhibitors in localized scleroderma: a systematic literature review.

Turkish journal of medical sciences
2025

Single-cell sequencing combined with spatial transcriptomics reveals the characteristics of follicle-targeted inflammation patterns in primary cicatricial alopecia.

Cell &amp; bioscience
2025

Validation of CXCL9 as a biomarker of morphoea disease state and severity through longitudinal analysis.

The British journal of dermatology
2025

Scleroderma Overlapping With Sjögren's Syndrome: A Case Report of a Renal Emergency.

Cureus
2025

Localized Morphea in a Pediatric Patient Successfully Treated With Topical Ruxolitinib.

Pediatric dermatology
2025

The role of selected cytokines from the interleukin 1 family in the pathophysiology of morphea.

Archives of dermatological research
2025

Pseudo oculomotor palsy associated with Parry-Romberg syndrome.

Archivos argentinos de pediatria
2025

Is adiponectin involved in morphea pathogenesis? - first observational study.

Frontiers in immunology
2025

[A case of autoinflammatory disease with STAT4 variant and localized scleroderma].

Zhonghua er ke za zhi = Chinese journal of pediatrics
2025

Juvenile localized scleroderma: a large retrospective cohort study from a tertiary care center.

Pediatric rheumatology online journal
2025

Localized scleroderma: multiparametric MRI with diffusion-weighted imaging and gadolinium-based perfusion imaging for assessing disease activity.

Skeletal radiology
2025

Devastating neurologic consequences of localized scleroderma en coup de sabre of the scalp-2 case studies.

Journal of scleroderma and related disorders
2025

New Challenging Systemic Therapies for Juvenile Scleroderma: A Comprehensive Review.

Pharmaceuticals (Basel, Switzerland)
2025

Effect of UV-A1 Phototherapy Treatment on Scleroderma: A Systematic Review.

Cureus
2025

[Disseminated atrophoderma of Pasini and Pierini with blaschkoid appearance].

Dermatologie (Heidelberg, Germany)
2025

Successful treatment of localized scleroderma using brodalumab: A case report.

SAGE open medical case reports
2025

Use of the oral Janus kinase inhibitor tofacitinib in the treatment of morphea: A retrospective study.

Journal of the American Academy of Dermatology
2025

Histopathological and molecular analysis in dermis and epidermis of patients with systemic and localized scleroderma.

Northern clinics of Istanbul
2025

miR-143-3p boosts extracellular vesicles to improve the dermal fibrosis of localized scleroderma.

Journal of autoimmunity
2025

A clinical guide to oral manifestations and diagnosis of limited systemic sclerosis: a case report.

General dentistry
2025

Rasmussen encephalitis and localized scleroderma of the lower limb: Another piece of the puzzle. A case-report and literature review with individual participant data.

Journal of neuroimmunology
2025

Incidence, prevalence, and mortality of localized scleroderma in Quebec, Canada: a population-based study.

Lancet regional health. Americas
2025

Unique and shared transcriptomic signatures underlying localized scleroderma pathogenesis identified using interpretable machine learning.

JCI insight
2025

The burden of extracutaneous manifestations in juvenile localized scleroderma: A literature review.

Autoimmunity reviews
2025

Solitary Morphea Profunda: A Case Report.

Mymensingh medical journal : MMJ
2025

EFFICACY AND TOLERABILITY OF JANUS KINASE INHIBITOR TOFACITINIB IN JUVENILE LINEAR SCLERODERMA. CASE SERIES OF 5 PATIENTS.

Georgian medical news
2025

Localized scleroderma: anatomic MRI findings of morphea with inter-reader analysis.

Skeletal radiology
2025

Socioeconomic factors associated with the development of localized scleroderma: a cross-sectional study.

Archives of dermatological research
2025

Development of Coup de Sabre in an HIV-Positive Patient: A Case Study from Lilongwe, Malawi.

The American journal of tropical medicine and hygiene
2025

Nationwide epidemiological and clinical survey of juvenile-onset morphea in Japan.

The Journal of dermatology
2025

[Oral localized scleroderma: case study].

Zhonghua kou qiang yi xue za zhi = Zhonghua kouqiang yixue zazhi = Chinese journal of stomatology
2025

Efficacy and safety of platelet-rich plasma therapy in systemic sclerosis and localized scleroderma; a systematic review.

Archives of dermatological research
2025

Coexistence of Lichen Sclerosus and Morphea in a Single Vulvar Lesion.

Cureus
2025

Markers of Type 2 Inflammation and Immunosenescence Are Upregulated in Localized Scleroderma.

International journal of molecular sciences
2025

Use of biologic drug in the treatment of localized scleroderma and systemic sclerosis in children: A scoping review.

Seminars in arthritis and rheumatism
2025

A Challenging Case of Eosinophilic Fasciitis Without Classic Histopathological Findings.

The Permanente journal
2025

A case of localized scleroderma with variable response to excimer light therapy.

The Journal of dermatology
2025

Comparative study between fractional CO2 laser (10,600 nm) and microneedling in treatment of morphea: dermoscopic and histopathological evaluation.

Archives of dermatological research
2025

A case series of dermatopathological features in different types of morphea and their clinical correlates.

Indian journal of pathology &amp; microbiology
2025

High-Density Fat Combined With Stromal Vascular Fraction Gel for the Treatment of Facial Localized Scleroderma.

The Journal of craniofacial surgery
2024

Advanced Neuroimaging and Clinical Findings in a Case of Linear Scleroderma En Coup De Sabre Presenting With Seizures.

Cureus
2024

Imiquimod for the treatment of morphea: a systematic review.

Archives of dermatological research
2024

What Is New in Morphea-Narrative Review on Molecular Aspects and New Targeted Therapies.

Journal of clinical medicine
2025

Benefits of the Topical JAK Inhibitor Delgocitinib in a Patient With Pediatric Localized Scleroderma.

Pediatric dermatology
2025

Linear Scleroderma En Coup de Sabre: A Case Series With Eyelid Involvement and Management.

Ophthalmic plastic and reconstructive surgery
2024

Profiling of Toll-like Receptors and Related Signaling Mediators in the Pathogenesis of Morphea.

Dermatology practical &amp; conceptual
2024

Evaluation of Thiol Disulfide, Ischemia Modified Albumin, and Prolidase Parameters in Patients with Localized Scleroderma.

Dermatology practical &amp; conceptual
2024

Association of Black Race with Demographic, Comorbidities, and Outcome Variables in Localized Scleroderma Patients: A Retrospective Analysis of the 2017 US National Inpatient Sample.

Dermatology practical &amp; conceptual
2024

JOINT LESIONS - COMMON EXTRACUTANEOUS MANIFESTATION IN JUVENILE LOCALIZED SCLERODERMA.

Georgian medical news
2024

STROKE AS A LIFE-THREATENING COMPLICATION IN CHILDREN WITH LINEAR SCLERODERMA OF FACE.

Georgian medical news
2024

Evaluation of autologous fat grafting in the treatment of juvenile localized scleroderma with facial involvement.

Journal of scleroderma and related disorders
2025

National Registry for Childhood Onset Scleroderma I: Insights from the first 341 juvenile localized scleroderma patients.

Journal of scleroderma and related disorders
2025

Disabling pansclerotic morphoea: a century of discovery.

The British journal of dermatology
2025

Heart transplantation in juvenile-onset systemic sclerosis with primary cardiac involvement: report of two cases and comprehensive literature review.

Current problems in cardiology
2025

Retrospective Review of Morphea in Paediatric Patients with Skin of Colour.

Journal of cutaneous medicine and surgery
2024

Outcomes of Fat Grafting in the Active Versus Quiescent Phase of Localized Scleroderma.

Plastic surgery (Oakville, Ont.)
2024

Calcium Hydroxyapatite as a Co-adjuvant Treatment Option in a Patient With Morphea: A Report of a Case With a One-Year Follow-Up.

Cureus
2024

Characterization of Endothelial Cell Subclusters in Localized Scleroderma Skin with Single-Cell RNA Sequencing Identifies NOTCH Signaling Pathway.

International journal of molecular sciences
2024

Rare Case of Limited Systemic Sclerosis with Left Ventricle Aneurysm.

The Journal of the Association of Physicians of India
2025

Comparative Study of Adipose-Derived Stem Cells from Localized Scleroderma Patients and Healthy Donors in Treating Skin Fibrosis.

Plastic and reconstructive surgery
2024

Interferon-Gamma-Inducible Protein-10 (IP-10) and Tumor Necrosis Factor-α (TNF-α) as Serological Predictors of Active Disease Status in Localized Scleroderma.

International journal of molecular sciences
2024

Challenges and complications in juvenile localized scleroderma: A practical approach.

Best practice &amp; research. Clinical rheumatology
2024

Histopathologic spectrum of morphea: a single-center retrospective study.

Dermatology reports
2024

The causal effects of inflammatory and autoimmune skin diseases on thyroid diseases: evidence from Mendelian randomization study.

Frontiers in endocrinology
2024

Spatial Transcriptomics Identifies Cellular and Molecular Characteristics of Scleroderma Skin Lesions: Pilot Study in Juvenile Scleroderma.

International journal of molecular sciences
2024

Exploring the relationship between morphea and malignancy: a decade-long single-center study of 204 patients.

Archives of dermatological research
2024

Case Report: Exacerbation after fat grafting in patients with active localized scleroderma.

Frontiers in surgery
2024

Single-cell RNA sequencing identifies inherent abnormalities of adipose-derived stem cells from nonlesional sites of patients with localized scleroderma.

Cellular &amp; molecular biology letters
2024

Advances in the Management of Localized Scleroderma: A Systematic Review of Laser Therapy and Injectable Filler Approaches.

Journal of personalized medicine
2024

Magnetic Resonance Imaging in the Assessment and Management of Post-injection-Site Morphea.

Cureus
2025

[Bullous morphea: A rare form of localized scleroderma].

Annales de pathologie
2024

Endpoints and outcomes for localized scleroderma/morphea: a scoping literature review.

Pediatric rheumatology online journal
2024

A Rare Case of Bullous Morphea Associated with Autoimmune Hepatitis.

Journal of clinical medicine
2024

The role of excimer light in dermatology: a review.

Anais brasileiros de dermatologia
2024

Scleroderma-like Lesions in a Patient Undergoing Combined Pembrolizumab and Routine Chemotherapy: A Case Report and Literature Review.

Medicina (Kaunas, Lithuania)
2024

Capillaroscopy in the daily clinic of the pediatric rheumatologist.

Best practice &amp; research. Clinical rheumatology
2024

Atypical chemokine receptor 1-positive endothelial cells mediate leucocyte infiltration and synergize with secreted frizzled-related protein 2/asporin-positive fibroblasts to promote skin fibrosis in systemic sclerosis.

The British journal of dermatology
2024

A consensus-based electronic medical record template for pediatric morphea patient visits.

Pediatric dermatology
2024

Extragenital lichen sclerosus et atrophicus-morphea overlap as an initial presentation of genital lichen sclerosus.

Dermatology online journal
2024

High-frequency ultrasound evaluation of morphea: Retrospective analytical study.

Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)
2024

The Serological Evidence of Positive Rheumatoid Factor in Morphea Patients and Its Relation to Disease Severity.

Cureus
2024

Clinical and ultrasonographic evaluation of efficacy and safety of intralesional injection of autologous platelet-rich plasma in morphea: A comparative case series.

Journal of scleroderma and related disorders
2024

Type-2 immunity associated with type-1 related skin inflammatory diseases: friend or foe?

Frontiers in immunology
2024

Localized Scleroderma Burden: A Scoping Review Focusing on the Health-Related Quality of Life, Mental Health, and Productivity.

Journal of cutaneous medicine and surgery
2024

Responsiveness to Change of the Morphea Activity Measure in Pediatric Patients.

JAMA dermatology
2024

Ultrasonographic Evaluation of Juvenile Localized Scleroderma: Enhancing Objectivity in Diagnosis and Management.

Current medical imaging
2024

Evidence for the causal relationship between rheumatoid arthritis and localized scleroderma: a two-sample mendelian randomization study.

Archives of dermatological research
2024

Overlap of Scleroderma and Class V Lupus Nephritis: A Rare Autoimmune Confluence.

Cureus
2025

Tracking Fat Grafts by Magnetic Resonance Imaging: A Comparative Study of Adolescent and Adult Patients with Stable Localized Scleroderma.

Plastic and reconstructive surgery
2024

Intraoral Involvement in Linear Scleroderma En Coup De Sabre: A Case Report.

Cureus
2024

Interferons dominate damage and activity in juvenile scleroderma.

Modern rheumatology
2024

Automated anatomical landmark detection on 3D facial images using U-NET-based deep learning algorithm.

Quantitative imaging in medicine and surgery
2024

Transition to Adult Rheumatology Care: A Disease-Specific Guide.

Journal of clinical rheumatology : practical reports on rheumatic &amp; musculoskeletal diseases
2024

Pre-scleroderma and new opportunities for preventive medicine.

The Lancet. Rheumatology
2024

Antibody status against measles and rubella in juvenile localized scleroderma patients on synthetic DMARDs: a prospective case-control study.

Rheumatology (Oxford, England)
2024

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

Raynaud's phenomenon associated with psoriatic arthritis.

BMJ case reports
2024

S2k guideline: Diagnosis and therapy of localized scleroderma.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

Damage evaluation of craniofacial localized scleroderma using magnetic resonance imaging.

Quantitative imaging in medicine and surgery
2024

Juvenile eosinophilic fasciitis: a single center case series.

Pediatric rheumatology online journal
2024

Nintedanib in systemic sclerosis treatment: a case report.

Journal of medical case reports
2024

A Comparison of Clinical, Demographic and Treatment Characteristics of Pediatric-Onset and Adult-Onset Patients Diagnosed With Localized Scleroderma.

Dermatology practical &amp; conceptual
2024

Mass spectrometry -based proteomic analysis of the skin of patients with localized scleroderma.

Journal of dermatological science
2024

Clinical, Histologic, and Transcriptomic Evaluation of Sequential Fat Grafting for Morphea: A Nonrandomized Controlled Trial.

JAMA dermatology
2024

Novel multispectral imaging to predict disease progression in pediatric morphea.

Pediatric dermatology
2023

Perspective to precision medicine in scleroderma.

Frontiers in immunology
2023

The role of skin ultrasound in systemic sclerosis: looking below the surface to understand disease evolution.

The Lancet. Rheumatology
2023

MRI Digital Artery Volume Index (DAVIX) as a surrogate outcome measure of digital ulcer disease in patients with systemic sclerosis: a prospective cohort study.

The Lancet. Rheumatology
2024

Development of Morphea Following Treatment with an ADA Biosimilar: A Case Report.

Current rheumatology reviews
2024

[Juvenile localized scleroderma].

Dermatologie (Heidelberg, Germany)
2024

Measurement equivalence of the English and French versions of the self-efficacy to manage chronic disease scale: a Scleroderma Patient-Centered Intervention Network (SPIN) study.

Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation
2025

Invited Response on: "Fat Grafting Reduces Skin Hyperpigmentation of Localized Scleroderma Patients: A Prospective Self-controlled Study".

Aesthetic plastic surgery
2023

Morphea Involving the Lips and Gingiva: A Rare Case Report.

Cureus
2024

Best clinical practice in the treatment of juvenile systemic sclerosis: expert panel guidance - the result of the International Hamburg Consensus Meeting December 2022.

Expert review of clinical immunology
2024

Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.

Arthritis care &amp; research
2023

Synthetic Nucleic Acid Antigens in Localized Scleroderma.

International journal of molecular sciences
2024

Clinical characteristics of juvenile systemic sclerosis in Korea: 31-year single-center study.

Journal of rheumatic diseases
2025

Letter on Fat Grafting Reduces Skin Hyperpigmentation of Localized Scleroderma Patients: A Prospective Self-controlled Study.

Aesthetic plastic surgery
2023

Brown adipose tissue transplantation improves skin fibrosis in localized scleroderma.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology
2024

Patients with autoimmune skin diseases are at increased risk of adverse pregnancy outcomes.

American journal of obstetrics &amp; gynecology MFM
2023

Connective tissue nevus misdiagnosed as juvenile localized scleroderma.

Pediatric rheumatology online journal
2023

BULGARIAN PATIENT WITH ATROPHODERMA OF PASINI AND PIERINI- DESCRIPTION OF A CASE AND SHORT UPDATE.

Georgian medical news
2023

Nodular (keloidal) scleroderma.

Reumatologia clinica
2023

Orthodontic management of a patient with linear morphea.

Journal of clinical orthodontics : JCO
2023

Morphea, Eosinophilic Fasciitis and Cancer: A Scoping Review.

Cancers
2024

Systematic Review of Health-Related Quality of Life Impact in Juvenile Localized Scleroderma.

Arthritis care &amp; research
2023

Exosomes from human adipose-derived mesenchymal stem cells attenuate localized scleroderma fibrosis by the let-7a-5p/TGF-βR1/Smad axis.

Journal of dermatological science
2023

Clinical and Histological Characteristics of Localized Morphea, Generalized Morphea and Systemic Sclerosis: A Comparative Study Aided by Machine Learning.

Acta dermato-venereologica
2023

A case of transient paradoxical early morphea-like eruption after the first injection of dupilumab in a patient with chronic rhinosinusitis with nasal polyps.

Italian journal of dermatology and venereology
2023

Arsenic trioxide demonstrates efficacy in a mouse model of preclinical systemic sclerosis.

Arthritis research &amp; therapy
2023

Bony Hyperplasia Beneath Atrophic Soft Tissue: A Rare Case of En Coup de Sabre and Literature Review.

Clinical, cosmetic and investigational dermatology
2023

IL-6 trans-signaling in a humanized mouse model of scleroderma.

Proceedings of the National Academy of Sciences of the United States of America
2024

Does the Impact of COVID-19 on Patients With Systemic Sclerosis Change Over Time?

Arthritis care &amp; research
2023

Diagnosing and Managing Linear Scleroderma in a Low-Resource Setting.

Case reports in pediatrics
2023

Images in Vascular Medicine: Subcutaneous panniculitis-like T-cell lymphoma mimicking lipodermatosclerosis.

Vascular medicine (London, England)
2023

Fat Grafting Reduces Skin Hyperpigmentation of Localized Scleroderma Patients: A Prospective Self-controlled Study.

Aesthetic plastic surgery
2023

The Reactivation of Scleroderma After Autologous Fat Grafting.

The Journal of craniofacial surgery
2023

Free Superficial Circumflex Iliac Artery/Superficial Inferior Epigastric Artery Adipofascial Flaps for Facial Reconstruction of Linear Scleroderma.

Annals of plastic surgery
2023

En Coup de Sabre in a Pediatric Patient Treated With Calcipotriene.

Cureus
2023

Color Doppler Ultrasound Assessment of Subclinical Activity With Scoring of Morphea.

Journal of cutaneous medicine and surgery
2024

Non-invasive imaging and clinical skin scores in juvenile localized scleroderma.

Rheumatology (Oxford, England)
2023

Role of imaging in morphea assessment: A review of the literature.

Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)
2023

The Collaborative National Quality and Efficacy Registry for Scleroderma: association of medication use on gastrointestinal tract symptoms in early disease and the importance of tobacco cessation.

Clinical and experimental rheumatology
2023

Pansclerotic morphea is characterized by IFN-γ responses priming dendritic cell fibroblast crosstalk to promote fibrosis.

JCI insight
2023

Systemic therapy in juvenile localized scleroderma.

Expert review of clinical immunology
2023

Case Report: Real NPSLE? A patient with systemic sclerosis overlapping systemic lupus erythematosus presenting as epilepsy.

Frontiers in immunology
2023

Spontaneous crateriform indentation in a child.

Pediatric dermatology
2023

Transplantation of in vitro prefabricated adipose organoids attenuates skin fibrosis by restoring subcutaneous fat and inducing dermal adipogenesis.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology
2023

Learnings from clinical trials in patients with connective tissue disease-associated interstitial lung disease.

Arthritis research &amp; therapy
Ver todos os 637 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Methotrexate and Mycophenolate Mofetil and Clinical Response in Juvenile Localized Scleroderma.
    JAMA dermatology· 2026· PMID 41604178mais citado
  2. Association of MMP9 and NOS3 Polymorphisms with Distinct Clinical Forms of Juvenile Scleroderma and Characteristics of Humoral Immunity.
    International journal of molecular sciences· 2026· PMID 41596747mais citado
  3. Successful Treatment of Refractory Severe Localized Scleroderma With Anifrolumab.
    JAMA dermatology· 2026· PMID 41405904mais citado
  4. Remote Skin and Blood Sampling for Translational Connective Tissue Disorder Research: A Proof-of-Concept Pilot Study.
    JID innovations : skin science from molecules to population health· 2026· PMID 41209845mais citado
  5. A practical clinical approach to the diagnosis and management of morphea (localized scleroderma).
    Best practice &amp; research. Clinical rheumatology· 2026· PMID 41864763mais citado
  6. Conventional ultrasound combined with shear wave elastography for assessing disease activity in pediatric localized scleroderma: a prospective pilot study.
    Pediatr Radiol· 2026· PMID 41949609recente
  7. Circumscribed Morphea Successfully Treated With Excimer Laser: Analysis of Histopathological Changes.
    J Dermatol· 2026· PMID 41847891recente
  8. Human MAGI1 expression in endothelial cells protects from the development of localized and systemic scleroderma in mice.
    Arthritis Res Ther· 2026· PMID 41814414recente
  9. Case of generalized morphea complicated with systemic sclerosis.
    Dermatol Reports· 2026· PMID 41789881recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:90289(Orphanet)
  2. MONDO:0019562(MONDO)
  3. Esclerose Sistemica(PCDT · Ministério da Saúde)
  4. GARD:7058(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q3324389(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Esclerodermia localizada
Compêndio · Raras BR

Esclerodermia localizada

ORPHA:90289 · MONDO:0019562
🇧🇷 Brasil SUS
Geral
Prevalência
1-9 / 100 000
Herança
Not applicable
CID-10
L94.0 · Esclerodermia localizada [morféia]
CID-11
Ensaios
6 ativos
Início
All ages
Prevalência
0.0 (Europe)
MedGen
UMLS
C0036420
Repurposing
2 candidatos
halofuginonecollagenase inhibitor
potassium-p-aminobenzoate
EuropePMC
Wikidata
Papers 10a
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