MCID: LCL006
MIFTS: 62

Localized Scleroderma

Categories: Bone diseases, Genetic diseases, Immune diseases, Nephrological diseases, Rare diseases, Respiratory diseases, Skin diseases

Aliases & Classifications for Localized Scleroderma

MalaCards integrated aliases for Localized Scleroderma:

Name: Localized Scleroderma 12 52 58 36 15 71
Morphea 12 52 54 71
Localized Fibrosing Scleroderma 52 58
Localised Scleroderma 12 17
Scleroderma, Circumscribed or Localised 12
Scleroderma, Circumscribed or Localized 12
Circumscribed Scleroderma 12
Scleroderma, Localized 52
Localised Morphoea 12
Localised Morphea 12
Localized Morphea 12

Characteristics:

Orphanet epidemiological data:

58
localized scleroderma
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:8472
KEGG 36 H01493
ICD9CM 34 701.0
MeSH 43 D012594
NCIt 49 C72069
SNOMED-CT 67 201048007
ICD10 32 L94.0
MESH via Orphanet 44 D012594
ICD10 via Orphanet 33 L94.0
UMLS via Orphanet 72 C0036420
Orphanet 58 ORPHA90289
UMLS 71 C0036420 C1527383

Summaries for Localized Scleroderma

KEGG : 36 Localized scleroderma, also known as morphea, is a rare fibrosing disorder of the skin and underlying tissues. Sclerosis is mainly limited to the skin, but subcutaneous tissue, fascia, and underlying muscles and bone may also be involved. It is generally differentiated from systemic scleroderma by the lack of Raynaud's phenomenon and visceral involvements. Localized scleroderma is classified into plaque morphea, generalized morphea, and linear scleroderma subtypes according to the clinical presentation and depth of tissue involvement. Localized scleroderma generally appears at any age, and onset at 20-50 years old is common, with the exception of the linear type, which often develops in childhood. Extracutaneous manifestations in morphea are not rare. The most common extracutaneous manifestations are arthralgia and myalgia. Neurologic manifestations and ocular complications are the most common in those children with head and neck involvement.

MalaCards based summary : Localized Scleroderma, also known as morphea, is related to systemic scleroderma and scleroderma, familial progressive, and has symptoms including pruritus and exanthema. An important gene associated with Localized Scleroderma is MMP1 (Matrix Metallopeptidase 1), and among its related pathways/superpathways are ERK Signaling and PEDF Induced Signaling. The drugs Vaccines and Sodium citrate have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related phenotypes are hypopigmentation of the skin and cigarette-paper scars

NIH Rare Diseases : 52 Localized scleroderma is characterized by thickening of the skin from excessive collagen deposits. Collagen is a protein normally present in our skin that provides structural support. However, when too much collagen is made, the skin becomes stiff and hard. Localized types of scleroderma are those limited to the skin and related tissues and, in some cases, the muscle below. Internal organs are not affected by localized scleroderma, and localized scleroderma can never progress to the systemic form of the disease. Often, localized conditions improve or go away on their own over time, but the skin changes and damage that occur when the disease is active can be permanent. For some people, localized scleroderma is serious and disabling. There are two generally recognized types of localized scleroderma: morphea and linear.

Wikipedia : 74 Morphea, is a form of scleroderma that involves isolated patches of hardened skin on the face, hands,... more...

Related Diseases for Localized Scleroderma

Diseases in the Systemic Scleroderma family:

Scleroderma, Familial Progressive Localized Scleroderma

Diseases related to Localized Scleroderma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 642)
# Related Disease Score Top Affiliating Genes
1 systemic scleroderma 33.0 TNF TGFB1 SELE IL6 IL4 IL13
2 scleroderma, familial progressive 31.6 COL1A1 CCN2 BMP6
3 diffuse scleroderma 30.7 TGFB1 COL1A1 CCN2
4 keloid disorder 30.7 TGFB1 SMAD3 BMP6
5 scleredema adultorum 30.6 MMP1 DCN CD34
6 hypertrophic scars 30.5 TGFB1 SMAD3 BMP6
7 fasciitis 30.4 TNF MMP1 IL6 IL2 CD34
8 basal cell carcinoma 30.3 TNF MMP1 IL6 IL2 CD34
9 tuberculoid leprosy 30.3 TGFB1 IL2 BMP6
10 gastroesophageal reflux 30.3 TNF TGFB1 IL6 IL4
11 chronic ulcer of skin 30.2 TNF MMP1 IL6
12 esophagitis 30.2 TNF TGFB1 IL6 IL4 IL13
13 rubella 30.2 TNF IL6 IL4 IL2
14 aortic aneurysm, familial abdominal, 1 30.1 MMP1 IL6 DCN
15 purpura 30.1 TNFSF13B TNF IL6 IL4
16 septic arthritis 30.1 TNF MMP1 IL6
17 uveitis 30.1 TNF IL6 IL4 IL2 CXCL10
18 thyroiditis 30.1 TNF IL6 IL4 IL2
19 lichen planus 30.1 TNF IL6 IL4 IL2
20 exanthem 30.1 TNF IL6 IL2
21 toxoplasmosis 30.0 TNF TGFB1 IL6 IL4
22 chronic venous insufficiency 30.0 VCAM1 TGFB1 MMP1
23 carpal tunnel syndrome 30.0 TGFB1 IL6 IL2 COL1A1 CCN2
24 hashimoto thyroiditis 30.0 TNF IL6 IL4 IL2
25 keratoconjunctivitis sicca 30.0 TNFSF13B TNF IL4
26 bronchiolitis obliterans 29.9 TNF TGFB1 IL6 IL13
27 graft-versus-host disease 29.9 TNF IL6 IL2
28 neuritis 29.9 TNF IL6 IL4 CXCL10
29 diphtheria 29.9 TNF IL4 IL2 IL13
30 deficiency anemia 29.9 TNF IL6 CD34 BMP6
31 chlamydia 29.9 TNF IL6 IL4
32 leprosy 3 29.9 TNF IL6 IL4 IL2 CXCL10
33 cutaneous lupus erythematosus 29.9 TNFSF13B TNF SELE IL6
34 mixed connective tissue disease 29.9 TNFSF13B TNF IL6
35 chickenpox 29.9 TNF IL6 IL4 IL2
36 severe acute respiratory syndrome 29.9 TNF IL6 IL2 CXCL10
37 cellulitis 29.8 TNF IL6 IL4 IL2
38 osteomyelitis 29.8 TNF IL6 IL4 IL13 CXCL10
39 crohn's disease 29.8 TNF IL6 IL4 IL2
40 synovitis 29.8 VCAM1 TNF SELE MMP1 IL6
41 cryoglobulinemia 29.8 TNFSF13B TNF IL6 CXCL10
42 bullous pemphigoid 29.7 TNFSF13B TNF IL4 IL13
43 tetanus 29.7 TNF IL6 IL4 IL2 IL13
44 sarcoidosis 1 29.7 TNF IL2 IL13 CXCL10
45 celiac disease 1 29.7 TNF IL6 IL4 IL2 IL13
46 connective tissue disease 29.7 TNFSF13B TNF SMAD3 IL6 IL4 COL1A1
47 polyarteritis nodosa 29.7 VCAM1 SMAD3 SELE IL6
48 dermatitis 29.6 TNF IL6 IL4 IL2 IL13
49 bronchiolitis 29.6 TNF IL6 IL4 IL13 CXCL10
50 osteoarthritis 29.6 TNF TGFB1 SMAD3 MMP1 IL6 DCN

Comorbidity relations with Localized Scleroderma via Phenotypic Disease Network (PDN):


Systemic Scleroderma Vulva Cancer

Graphical network of the top 20 diseases related to Localized Scleroderma:



Diseases related to Localized Scleroderma

Symptoms & Phenotypes for Localized Scleroderma

Human phenotypes related to Localized Scleroderma:

58 31 (show all 16)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypopigmentation of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0001010
2 cigarette-paper scars 58 31 hallmark (90%) Very frequent (99-80%) HP:0001073
3 hyperpigmentation of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0000953
4 stiff skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0030053
5 skeletal muscle atrophy 58 31 frequent (33%) Frequent (79-30%) HP:0003202
6 lipoatrophy 58 31 occasional (7.5%) Occasional (29-5%) HP:0100578
7 arthralgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002829
8 myalgia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003326
9 split hand 58 31 occasional (7.5%) Occasional (29-5%) HP:0001171
10 flexion contracture of toe 58 31 occasional (7.5%) Occasional (29-5%) HP:0005830
11 scarring alopecia of scalp 58 31 occasional (7.5%) Occasional (29-5%) HP:0004552
12 hemiatrophy of lower limb 58 31 occasional (7.5%) Occasional (29-5%) HP:0100557
13 hemiatrophy of upper limb 58 31 occasional (7.5%) Occasional (29-5%) HP:0100558
14 flexion contracture 58 Occasional (29-5%)
15 hemiatrophy 58 Occasional (29-5%)
16 dermal atrophy 58 Very frequent (99-80%)

UMLS symptoms related to Localized Scleroderma:


pruritus, exanthema

MGI Mouse Phenotypes related to Localized Scleroderma:

45 (show all 18)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.47 CCN2 CD34 COL1A1 CXCL10 DCN IL13
2 homeostasis/metabolism MP:0005376 10.42 CCN2 CD34 COL1A1 CXCL10 DCN IL13
3 hematopoietic system MP:0005397 10.41 CD34 COL1A1 CXCL10 DCN IL13 IL2
4 immune system MP:0005387 10.41 CD34 COL1A1 CXCL10 DCN IL13 IL2
5 growth/size/body region MP:0005378 10.4 BMP6 CCN2 COL1A1 DCN IL13 IL2
6 cardiovascular system MP:0005385 10.35 CCN2 COL1A1 CXCL10 IL2 IL6 SELE
7 digestive/alimentary MP:0005381 10.33 CCN2 COL1A1 DCN IL13 IL2 IL4
8 endocrine/exocrine gland MP:0005379 10.31 CCN2 COL1A1 DCN IL13 IL2 IL4
9 mortality/aging MP:0010768 10.28 CCN2 COL1A1 CXCL10 DCN IL13 IL2
10 integument MP:0010771 10.27 CCN2 CD34 COL1A1 DCN IL13 IL4
11 craniofacial MP:0005382 10.21 CCN2 COL1A1 DCN IL4 SMAD3 SMAD4
12 liver/biliary system MP:0005370 10.13 CCN2 COL1A1 IL2 IL4 IL6 SELE
13 reproductive system MP:0005389 10.07 BMP6 COL1A1 DCN IL13 IL2 IL4
14 neoplasm MP:0002006 10.02 CD34 COL1A1 DCN IL2 IL6 MMP1
15 muscle MP:0005369 9.97 COL1A1 DCN IL13 IL6 SMAD4 SMAD7
16 respiratory system MP:0005388 9.85 CCN2 COL1A1 DCN IL13 IL2 IL4
17 skeleton MP:0005390 9.7 BMP6 CCN2 COL1A1 DCN IL13 IL4
18 vision/eye MP:0005391 9.32 CCN2 COL1A1 DCN IL2 IL4 IL6

Drugs & Therapeutics for Localized Scleroderma

Drugs for Localized Scleroderma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 144)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Vaccines Phase 4
2
Sodium citrate Approved, Investigational Phase 3 68-04-2
3
Lidocaine Approved, Vet_approved Phase 2, Phase 3 137-58-6 3676
4
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
5
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
6
Acetylcholine Approved, Investigational Phase 3 51-84-3 187
7
Lenograstim Approved, Investigational Phase 3 135968-09-1
8
Sargramostim Approved, Investigational Phase 3 83869-56-1, 123774-72-1
9
Macitentan Approved Phase 2, Phase 3 441798-33-0
10
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
11 Pharmaceutical Solutions Phase 2, Phase 3
12 Immunosuppressive Agents Phase 3
13 Antirheumatic Agents Phase 3
14 Phosphodiesterase 5 Inhibitors Phase 3
15 Phosphodiesterase Inhibitors Phase 3
16 Vasodilator Agents Phase 3
17 Citrate Phase 3
18 Sodium Channel Blockers Phase 2, Phase 3
19 Anti-Arrhythmia Agents Phase 2, Phase 3
20 Diuretics, Potassium Sparing Phase 2, Phase 3
21 Anesthetics, Local Phase 2, Phase 3
22 Chelating Agents Phase 3
23 Calcium, Dietary Phase 3
24 Anticoagulants Phase 3
25 Insulin, Globin Zinc Phase 3
26 abobotulinumtoxinA Phase 3
27 Cholinergic Agents Phase 3
28 insulin Phase 3
29 Botulinum Toxins Phase 3
30 Botulinum Toxins, Type A Phase 3
31 Neurotransmitter Agents Phase 3
32 Alkylating Agents Phase 3
33 Endothelin Receptor Antagonists Phase 2, Phase 3
34 Endothelin A Receptor Antagonists Phase 2, Phase 3
35
Calcium Nutraceutical Phase 3 7440-70-2 271
36
Adapalene Approved Phase 1, Phase 2 106685-40-9 60164
37
Benzoyl peroxide Approved Phase 1, Phase 2 94-36-0 7187
38
Petrolatum Approved, Investigational Phase 2 8009-03-8
39
Treprostinil Approved, Investigational Phase 2 81846-19-7 6918140 54786
40
Epoprostenol Approved Phase 2 35121-78-9, 61849-14-7 5282411 5280427
41
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
42
Abatacept Approved Phase 1, Phase 2 332348-12-6 10237
43
Riociguat Approved Phase 2 625115-55-1
44
tannic acid Approved Phase 2 1401-55-4
45
Benzocaine Approved, Investigational Phase 2 94-09-7, 1994-09-7 2337
46
Diltiazem Approved, Investigational Phase 2 42399-41-7 39186
47
Nitroglycerin Approved, Investigational Phase 2 55-63-0 4510
48
Rilonacept Approved, Investigational Phase 1, Phase 2 501081-76-1 104924
49
Pirfenidone Approved, Investigational Phase 2 53179-13-8 40632
50
Bosentan Approved, Investigational Phase 2 147536-97-8 104865

Interventional clinical trials:

(show top 50) (show all 96)
# Name Status NCT ID Phase Drugs
1 Safety and Efficacy of Vaccination Against Influenza in Patients With Scleroderma Unknown status NCT01002508 Phase 4
2 A Phase IV, Single-arm, Open-label Clinical Trial to Evaluate the Efficacy and Safety of PLACENTEX ® Polydeoxyribonucleotide i.m. in Patients With Fibrotic and Atrophic Cutaneous Lesions in Scleroderma Diseases Active, not recruiting NCT03388255 Phase 4 Polydeoxyribonucleotides
3 Evaluation of the Efficacy and Safety of the Imiquimod 5% Topical Cream in Plaque Morphea: A Prospective, Multiple Baseline, Open Label Pilot Study Completed NCT00147771 Phase 3 Imiquimod 5% cream
4 Evaluation of the Efficacy of Sildenafil on Time to Healing in Patients With Scleroderma and Ischaemic Digital Ulcers: a Prospective, Longitudinal, Randomized, Comparative, Double-blind, 2-parallel-arm, Placebo-controlled Study Completed NCT01295736 Phase 3 Sildenafil;placebo
5 Effectiveness and Safety of Lidocaine for Scleroderma. Randomized Double-Blind Clinical Trial Completed NCT00740285 Phase 2, Phase 3 Lidocaine 2% without vessel constrictor
6 Effect of Sildenafil on the Microcirculatory Blood Flow and on the Endothelial Progenitor Cells in Patients With Systemic Sclerosis: a Randomized, Double-blind, Placebo-controlled Clinical Trial Completed NCT01347008 Phase 3 Sildenafil citrate;Placebo (Sugar pill)
7 A Randomized Control Trial to Assess the Efficacy of Tadalafil in Raynaud's Phenomenon in Scleroderma: A Double Blind, Parallel Group, Multicentric Study Completed NCT01117298 Phase 3 Tadalafil;Placebo
8 A Randomized, Double-Blinded, Placebo-Controlled Clinical Trial Assessing the Therapeutic Efficacy of Botulinum Toxin In Treating Scleroderma-Associated Raynaud's Syndrome Completed NCT02165111 Phase 3 Onabotulinumtoxin A;sterile saline solution
9 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
10 High Dose Cyclophosphamide for Treatment of Systemic Sclerosis (Scleroderma) Completed NCT00501995 Phase 3 IV Cyclophosphamide
11 CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated Interstitial Lung DiseasE and Pulmonary Hypertension (PH): The CRuSADE PH Study Recruiting NCT03726398 Phase 2, Phase 3 Opsumit 10 Mg Tablet
12 Evaluation of the Efficacy and Safety of Imiquimod 5% Cream in Plaque-type Morphea: A Pilot, Prospective Open-label Study Withdrawn NCT00230373 Phase 3 imiquimod 5% cream (Aldara)
13 A Protocol Based Treatment for Debilitating Fibrosing Skin Disorders With (Anti-CD 20), Rituximab, Evaluating Safety and Efficacy Unknown status NCT00936546 Phase 2 Rituximab
14 Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells Unknown status NCT02213705 Phase 1, Phase 2
15 Molecular and Clinical Study of the Effect of Zaxcell Versus Effezel in the Inflammatory and Scarring Process of Moderate and Severe Acne Unknown status NCT03076320 Phase 1, Phase 2
16 DISTOL-1: Digital Ischemic Lesions in Scleroderma Treated With Oral Treprostinil Diethanolamine: A Randomized, Double-blind, Placebo-controlled, Multicenter Study Completed NCT00775463 Phase 2 treprostinil diethanolamine;placebo
17 Phase II Randomized Double Blind Clinical Trial of'Imatinib Mesylate STI571 (Glivec®) Versus Placebo in Patients With Severe Cutaneous Scleroderma or Systemic Sclerosis With Severe Cutaneous Involvement. Completed NCT00479934 Phase 2 imatinib mesylate
18 Effect of Bosentan in the Course of Scleroderma Renal Crisis Completed NCT01241383 Phase 2 Bosentan
19 An Open Label Study to Evaluate the Safety of Dasatinib in the Treatment of Scleroderma Pulmonary Interstitial Fibrosis Completed NCT00764309 Phase 1, Phase 2 dasatinib
20 Multicenter Phase II Trial of Oral Type I Bovine Collagen in Scleroderma Completed NCT00005675 Phase 2 Oral bovine type I collagen;Placebo
21 A Pilot Study to Evaluate the Safety and Efficacy of Abatacept in Patients With Systemic Sclerosis Completed NCT00442611 Phase 1, Phase 2 Abatacept;Placebo
22 A Phase II, Single Centre, Randomised, Placebo-controlled, 3-part Trial to Assess the Safety, Tolerability and Efficacy of Zibotentan in Patients With Renal Disease Secondary to Scleroderma Completed NCT02047708 Phase 2 Zibotentan
23 The Effectiveness of UVB Irradiation in the Treatment of Skin Conditions With Altered Dermal Matrix: An Open Pilot Study Completed NCT00129428 Phase 1, Phase 2
24 A Multi-center Randomized, Double Blind, Placebo-controlled, Pilot Study to Assess the Efficacy and Safety of Riociguat in Scleroderma - Associated Digital Ulcers Completed NCT02915835 Phase 2 Riociguat;Placebo
25 Psychosocial Interventions for Scleroderma Completed NCT00007267 Phase 2
26 Trial of High Dose Cyclophosphamide and Rabbit Antithymocyte Globulin (rATG) With Hematopoietic Stem Cell Support in Patients With Systemic Scleroderma: A Randomized Trial Completed NCT00278525 Phase 2 standard of care
27 Mycophenolate vs. Oral Cyclophosphamide in Scleroderma Interstitial Lung Disease (Scleroderma Lung Study II) Completed NCT00883129 Phase 2 Mycophenolate mofetil;Cyclophosphamide;Placebo
28 Assessing and Comparing the Effect of Diltiazem Gel Versus Nitroglycerin Ointment in Healing Process of Scleroderma Digital Ulcers. Completed NCT02801305 Phase 2 Diltiazem Gel 2%;Nitroglycerin Ointment 2%;Vaseline
29 Randomized, Double-Blind, Placebo-Controlled Trial if IL1-TRAP, Rilonacept, in Systemic Sclerosis -A Phase I/II Biomarker Trial Completed NCT01538719 Phase 1, Phase 2 Rilonacept
30 Pilot Study Evaluating the Efficacy of a Topical PDE4 Inhibitor for Morphea Recruiting NCT03351114 Phase 2 Crisaborole
31 A Phase 1/2 Study of a Combination of FCX-013 (Genetically-Modified Autologous Human Dermal Fibroblasts) Plus Veledimex for the Treatment of Moderate to Severe Localized Scleroderma (Morphea) Recruiting NCT03740724 Phase 1, Phase 2 veledimex
32 A Pilot Study to Assess the Safety and Efficacy of Sarilumab in Halting Progression of Morphea Recruiting NCT03679845 Phase 1, Phase 2 Sarilumab
33 A Phase 2 Multicenter, Randomized, Double-blind, Placebo-controlled Study to Assess the Safety and Efficacy of Ifetroban in Patients With Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension (SSc-PAH) Recruiting NCT02682511 Phase 2 Oral Ifetroban;Oral Placebo
34 Scleroderma Lung Study III (SLS III): Combining the Anti-fibrotic Effects of Pirfenidone (PFD) With Mycophenolate (MMF) for Treating Scleroderma-related Interstitial Lung Disease Recruiting NCT03221257 Phase 2 Pirfenidone (PFD);Placebo (Plac);Mycophenolate Mofetil (MMF)
35 A Randomized, Placebo-controlled Phase IIa Clinical Trial to Evaluate the Efficacy and Safety of Subcutaneous Dupilumab in Localized Scleroderma Not yet recruiting NCT04200755 Phase 2 Dupilumab 300Mg Solution for Injection
36 Placebo Controlled Trial of Bosentan vs Placebo in NYHA Class I/II Scleroderma Patients With Exercise Induced Pulmonary Hypertension Terminated NCT00377455 Phase 2 Bosentan;Placebo
37 A Phase 2, Proof-Of-Concept, Multicenter, Randomized, Double-Blind, Placebo- Controlled, Study to Evaluate the Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Efficacy of Pomalidomide (CC-4047) in Subjects With Systemic Sclerosis With Interstitial Lung Disease Terminated NCT01559129 Phase 2 Pomalidomide (CC-4047);Placebo
38 A Proof of Concept Trial of Gleevec (Imatinib) in Active Diffuse Scleroderma Terminated NCT01545427 Phase 2 Imatinib mesylate
39 A Phase 1, Randomized, Double-blind, Placebo-controlled Study of the Safety and Tolerability of MEDI-551 in Scleroderma Completed NCT00946699 Phase 1
40 Safety & Suitability of Dabigatran to Inhibit Thrombin in Scleroderma Completed NCT02426229 Phase 1 dabigatran etexilate
41 A Phase 1 Multicenter, Open-label Study to Evaluate the Safety and Tolerability of Single and Multiple Intravenous Doses of MEDI-546, a Fully Human Monoclonal Antibody Directed Against Subunit 1 of the Type I Interferon Receptor, in Adult Subjects With Scleroderma Completed NCT00930683 Phase 1 MEDI-546;MEDI-546;MEDI-546;MEDI-546;MEDI-546;MEDI-546;MEDI-546;MEDI-546;MEDI-546
42 Comparative Effectiveness Trial of Topical Calcipotriene, Clobetasol, and Tacrolimus in the Treatment of Pediatric Plaque Morphea Withdrawn NCT02680717 Phase 1 Clobetasol;Calcipotriene;Tacrolimus
43 Investigation of the Genetic Architecture of Linear Localized Scleroderma (LLS) (Linear Morphea) by Whole Exome Sequencing. A Tailored Approach to Test the Hypothesis That LLS is a Genetic Mosaic Condition Unknown status NCT02222038
44 Fractional Carbon Dioxide Laser Versus UVA1 Phototherapy for Treatment of Localized Scleroderma: A Clinical & Immunohistochemical Comparative Study Unknown status NCT02002897
45 Correlation Between Mohs Surgery and Microscopic Fluorescence Photometry in Determination of Histological Borders in Basal Cell Carcinoma . Unknown status NCT00449358
46 A Prospective, Open Label Trial of High Dose UVA-1, 3x/Week or Medium Dose UVA-1, 3x/Week vs. Fluocinonide 0.05% Cream Treatment of Morphea Completed NCT00812188
47 Sleep Disturbances and Pulmonary Artery/Aorta Diameter in Scleroderma Patient Completed NCT00566137
48 Scleroderma Lung: Role of Gastroesophageal Reflux, Microaspiration and Cough Completed NCT01667042
49 A Randomized Clinical Study Comparing Topical 0.05% Clobetasol Propianate in Vaseline With UVA-1 Phototherapy in the Treatment of Vulvar Lichen Sclerosus Completed NCT01400022
50 Premature Coronary Atherosclerosis in Scleroderma: A Case Control Study Completed NCT00372398

Search NIH Clinical Center for Localized Scleroderma

Genetic Tests for Localized Scleroderma

Anatomical Context for Localized Scleroderma

MalaCards organs/tissues related to Localized Scleroderma:

40
Skin, Breast, Lung, Bone, Testes, Endothelial, Thyroid

Publications for Localized Scleroderma

Articles related to Localized Scleroderma:

(show top 50) (show all 1931)
# Title Authors PMID Year
1
Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. 54 61
18565735 2008
2
Differential expression of decorin in localized scleroderma following ultraviolet-A1 irradiation. 54 61
17184878 2007
3
Collagen degradation products and proinflammatory cytokines in systemic and localized scleroderma. 61 54
17448296 2007
4
Suggested mechanisms of action of UVA phototherapy in morphea: a molecular study. 61 54
15030594 2004
5
Induction of interstitial collagenase (MMP-1) by UVA-1 phototherapy in morphea fibroblasts. 61 54
9357412 1997
6
Clinical significance of serum levels of soluble interleukin-2 receptor in patients with localized scleroderma. 61 54
8736323 1996
7
Increased expression of lysyl oxidase in skin with scleroderma. 54 61
8555021 1995
8
Decreased expression of the human progenitor cell antigen (CD34) in morphea. 61 54
8599452 1995
9
Stimulated expression of decorin and the decorin gene in fibroblasts cultured from patients with localized scleroderma. 54 61
7625849 1995
10
Serum levels of soluble interleukin-2 receptor. A marker of disease activity in localized scleroderma. 61 54
8003062 1994
11
Immunocytochemical localization and serologic detection of transforming growth factor beta 1. Association with type I procollagen and inflammatory cell markers in diffuse and limited systemic sclerosis, morphea, and Raynaud's phenomenon. 61 54
7510487 1994
12
Expression of osteonectin, decorin, and transforming growth factor-beta 1 genes in fibroblasts cultured from patients with systemic sclerosis and morphea. 61 54
2023219 1991
13
The influence of interferon on healthy and diseased skin. 61
30527631 2020
14
Pigmented linear discoid lupus erythematosus following the lines of Blaschko: A retrospective study of a Chinese series. 61
32270767 2020
15
Sarcopenia in juvenile localized scleroderma: new insights on deep involvement. 61
32144460 2020
16
Jak Inhibition Prevents Bleomycin-Induced Fibrosis in Mice and Is Effective in Patients with Morphea. 61
31954727 2020
17
Localized scleroderma histologically characterized by liquefaction degeneration and upper dermis fibrosis: a possible association with chemotherapy. 61
32170760 2020
18
MicroRNA in localized scleroderma: a review of literature. 61
31637470 2020
19
Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre. 61
32527172 2020
20
Cerebellar Atrophy in Craniofacial Morphea: A Report of 2 Cases with 1-year Neuroimaging Followup. 61
32541079 2020
21
Risk of connective tissue disease, morphea and systemic vasculitis in patients with hidradenitis suppurativa. 61
32531094 2020
22
Morphea-like skin lesions reported in the Phase III Long-term Odanacatib Fracture Trial (LOFT) in postmenopausal women with osteoporosis. 61
32553680 2020
23
Risk of scleroderma according to the type of immune checkpoint inhibitors. 61
32540450 2020
24
Challenges faced by patients with morphea in the era of SARS-CoV-2. 61
32502370 2020
25
A Pediatrician's Alert: Misdiagnosis of Mixed Localized Scleroderma in a Child. 61
32578362 2020
26
Multicentric B-cell lymphoma with presumed paraneoplastic generalized cutaneous sclerosis in a dog. 61
31930629 2020
27
Long-standing morphea and the risk of squamous cell carcinoma of the skin. 61
32364667 2020
28
Association between major depressive disorder and subsequent autoimmune skin diseases: A nationwide population-based cohort study. 61
32469824 2020
29
Dermatologic Sequelae Associated with Radiation Therapy. 61
32410134 2020
30
Histopathology of fluoroscopy-induced radiation ulcer: a case series study in comparison with morphea. 61
32413216 2020
31
Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. 61
32441406 2020
32
Paediatric morphoea: a holistic review. Part 1: epidemiology, aetiopathogenesis and clinical classification. 61
32472964 2020
33
Paediatric morphoea: a holistic review. Part 2: diagnosis, measures of disease activity, management and natural history. 61
32449205 2020
34
Ultraviolet A1 phototherapy for the treatment of localized scleroderma. 61
32383187 2020
35
Scleredema of Buschke associated with lichen sclerosus: Three cases. 61
30289118 2020
36
A Case of Extensive Debilitating Generalized Morphea. 61
32542170 2020
37
DLQI-R scoring improves the discriminatory power of the Dermatology Life Quality Index in patients with psoriasis, pemphigus and morphea. 61
31419310 2020
38
Health-related quality of life and its influencing factors in adult patients with localized scleroderma - a cross-sectional study. 61
32398135 2020
39
Development of keloidal morphea after treatment with cyclosporine in a case of recalcitrant generalized morphea. 61
32477528 2020
40
Abatacept in the treatment of localized scleroderma: A pediatric case series and systematic literature review. 61
32504991 2020
41
Morphea (Localized Scleroderma) Presenting With Recurrent Skin and Soft Tissue Infections: A Diagnostic Dilemma. 61
32542124 2020
42
Methotrexate in Linear Scleroderma: long-term efficacy in 50 children from a single Pediatric Rheumatology Centre. 61
32433808 2020
43
Full histological and clinical regression of morphea with tofacitinib. 61
32424655 2020
44
Association of juvenile idiopathic arthritis and morphea: a case series. 61
31838640 2020
45
Progressive hemifacial atrophy or Parry-Romberg syndrome: A pediatric case report. 61
32428924 2020
46
Cosmetic Treatment in Patients with Autoimmune Connective Tissue Diseases. Part I. Best Practices for Patients with Morphea/Systemic Sclerosis. 61
32360721 2020
47
An Unusual Case of Morphea in the Setting of Aplastic Anemia. 61
32382464 2020
48
Patch-type granuloma annulare: An institution-based study of 23 cases. 61
32279342 2020
49
Evaluation of the Effectiveness and Tolerability of Mycophenolate Mofetil and Mycophenolic Acid for the Treatment of Morphea. 61
32236497 2020
50
At the Leading Edge in Morphea-New Insights Into Disease Course and Management Options. 61
32236492 2020

Variations for Localized Scleroderma

Expression for Localized Scleroderma

Search GEO for disease gene expression data for Localized Scleroderma.

Pathways for Localized Scleroderma

Pathways related to Localized Scleroderma according to GeneCards Suite gene sharing:

(show top 50) (show all 86)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.88 TNFSF13B TNF TGFB1 SMAD4 SMAD3 IL6
2
Show member pathways
13.77 TNFSF13B TNF TGFB1 SMAD4 SMAD3 IL6
3
Show member pathways
13.6 TNFSF13B TNF TGFB1 SMAD7 SMAD4 SMAD3
4
Show member pathways
13.52 TNFSF13B TNF TGFB1 IL6 IL4 IL2
5
Show member pathways
13.46 VCAM1 TNFSF13B TNF TGFB1 MMP1 IL6
6
Show member pathways
13.28 TNFSF13B TNF TGFB1 SMAD4 SMAD3 IL6
7
Show member pathways
13.15 TNF TGFB1 SMAD4 SMAD3 IL6 IL2
8
Show member pathways
13.14 VCAM1 TGFB1 IL6 IL4 IL2 CCN2
9
Show member pathways
13.13 TGFB1 IL6 IL2 COL1A1 CCN2 BMP6
10
Show member pathways
12.97 TNFSF13B TNF TGFB1 IL6 IL4 IL2
11 12.95 TGFB1 SMAD4 SMAD3 MMP1 IL6 IL4
12
Show member pathways
12.83 TNFSF13B TNF TGFB1 IL6 IL2 CCN2
13
Show member pathways
12.81 VCAM1 TGFB1 MMP1 DCN COL1A1
14 12.69 VCAM1 TNFSF13B TNF IL4 IL2 CXCL10
15
Show member pathways
12.68 TNF TGFB1 IL6 IL4 IL2
16
Show member pathways
12.66 TNF TGFB1 SMAD4 SMAD3 IL6 IL4
17 12.48 TNF TGFB1 SMAD4 SMAD3 IL6 IL2
18
Show member pathways
12.47 TNF MMP1 IL6 IL4 IL13 CXCL10
19
Show member pathways
12.45 TGFB1 IL6 IL2 CCN2 BMP6
20
Show member pathways
12.39 TNF IL6 IL2 CXCL10
21
Show member pathways
12.39 TNF IL6 IL4 IL2
22 12.36 TNF TGFB1 DCN COL1A1
23
Show member pathways
12.35 TGFB1 SMAD7 SMAD4 SMAD3
24 12.35 TGFB1 SMAD4 SMAD3 CCN2 BMP6
25
Show member pathways
12.32 TNF TGFB1 IL6 IL4 IL2 IL13
26 12.31 TNF TGFB1 SMAD4 SMAD3 MMP1 IL6
27 12.28 TGFB1 SMAD7 SMAD4 SMAD3 CCN2 BMP6
28
Show member pathways
12.26 TNF IL6 IL4 CXCL10
29 12.21 TGFB1 SMAD7 SMAD4 SMAD3 MMP1
30
Show member pathways
12.17 TGFB1 SMAD4 SMAD3 IL6
31 12.15 TNF TGFB1 SMAD3 IL6
32 12.14 TNF IL6 IL4 IL2 CD34
33 12.12 VCAM1 TNF SELE IL6 CXCL10
34 12.09 TGFB1 SMAD4 SMAD3 IL6 COL1A1
35
Show member pathways
12.05 TNF TGFB1 IL6 IL2
36 12.03 TNF TGFB1 IL6 COL1A1
37 12.03 TNFSF13B TNF TGFB1 IL6 IL4 IL2
38 12.01 TNF IL6 IL4 CD34
39 12.01 TNFSF13B TNF TGFB1 MMP1 IL6
40 11.99 TGFB1 SMAD7 SMAD4 SMAD3
41
Show member pathways
11.99 TGFB1 SMAD4 SMAD3 IL6 IL4
42
Show member pathways
11.97 TGFB1 SMAD4 SMAD3 IL6
43 11.97 VCAM1 TNF TGFB1 MMP1 IL6 IL4
44 11.95 VCAM1 TGFB1 IL6 DCN CD34 BMP6
45 11.93 TNF IL4 IL2 IL13 CD34
46 11.88 TNF TGFB1 IL6 IL2 CD34
47 11.87 SELE MMP1 IL6 IL4 IL2 IL13
48 11.86 TGFB1 SMAD7 SMAD4 SMAD3
49
Show member pathways
11.84 TGFB1 SMAD7 SMAD4 SMAD3
50 11.84 TNF TGFB1 SMAD7 SMAD4 SMAD3 DCN

GO Terms for Localized Scleroderma

Cellular components related to Localized Scleroderma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.8 TNFSF13B TNF TGFB1 MMP1 IL6 IL4
2 cell GO:0005623 9.76 TGFB1 SMAD4 SMAD3 IL6 IL2 IL13
3 external side of plasma membrane GO:0009897 9.72 VCAM1 TNF IL13 CXCL10 CD34
4 transcription factor complex GO:0005667 9.71 SMAD7 SMAD4 SMAD3 SKOR1
5 extracellular matrix GO:0031012 9.65 TGFB1 MMP1 DCN COL1A1 CCN2
6 extracellular space GO:0005615 9.47 VCAM1 TNFSF13B TNF TGFB1 SELE IL6
7 SMAD protein complex GO:0071141 9.37 SMAD4 SMAD3

Biological processes related to Localized Scleroderma according to GeneCards Suite gene sharing:

(show top 50) (show all 66)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 10.2 TNF TGFB1 SMAD7 SMAD4 SMAD3 SKOR1
2 positive regulation of transcription, DNA-templated GO:0045893 10.15 TNF TGFB1 SMAD4 SMAD3 IL6 IL4
3 positive regulation of cell proliferation GO:0008284 10.12 TGFB1 IL6 IL4 IL2 CXCL10 CCN2
4 extracellular matrix organization GO:0030198 10.04 VCAM1 TNF MMP1 DCN COL1A1
5 positive regulation of cell migration GO:0030335 10.04 TGFB1 SMAD3 IL4 CXCL10 COL1A1
6 inflammatory response GO:0006954 10.04 TNF TGFB1 SELE IL6 IL13 CXCL10
7 regulation of cell proliferation GO:0042127 10.02 TNF TGFB1 SMAD4 CXCL10
8 positive regulation of protein phosphorylation GO:0001934 10.01 TNF TGFB1 IL2 CCN2
9 response to lipopolysaccharide GO:0032496 10.01 SELE IL13 DCN CXCL10
10 regulation of immune response GO:0050776 10 VCAM1 TNFSF13B SMAD3 IL4 COL1A1 CD34
11 positive regulation of transcription by RNA polymerase II GO:0045944 10 TNF TGFB1 SMAD7 SMAD4 SMAD3 IL6
12 negative regulation of inflammatory response GO:0050728 9.97 SMAD3 IL4 IL2 IL13
13 transforming growth factor beta receptor signaling pathway GO:0007179 9.95 TGFB1 SMAD7 SMAD4 SMAD3
14 leukocyte migration GO:0050900 9.95 TNF TGFB1 SELE MMP1 COL1A1 CD34
15 wound healing GO:0042060 9.93 TGFB1 SMAD3 DCN COL1A1
16 BMP signaling pathway GO:0030509 9.92 TGFB1 SMAD7 SMAD4 BMP6
17 cellular response to mechanical stimulus GO:0071260 9.92 TGFB1 IL13 COL1A1 BMP6
18 negative regulation of transforming growth factor beta receptor signaling pathway GO:0030512 9.91 TGFB1 SMAD7 SMAD3
19 response to glucocorticoid GO:0051384 9.9 TNF IL6 BMP6
20 response to mechanical stimulus GO:0009612 9.89 IL13 DCN COL1A1
21 positive regulation of smooth muscle cell proliferation GO:0048661 9.89 TNF IL6 IL13
22 cellular response to transforming growth factor beta stimulus GO:0071560 9.89 TGFB1 SMAD7 SMAD3 COL1A1
23 positive regulation of protein complex assembly GO:0031334 9.88 TNF TGFB1 MMP1
24 T cell activation GO:0042110 9.88 TGFB1 SMAD3 IL4
25 SMAD protein signal transduction GO:0060395 9.88 TGFB1 SMAD4 SMAD3 BMP6
26 positive regulation of pathway-restricted SMAD protein phosphorylation GO:0010862 9.87 TGFB1 SMAD4 BMP6
27 positive regulation of protein secretion GO:0050714 9.87 TGFB1 IL13 BMP6
28 extrinsic apoptotic signaling pathway GO:0097191 9.87 TNF TGFB1 SMAD3
29 negative regulation of fat cell differentiation GO:0045599 9.87 TNF TGFB1 SMAD3 IL6
30 ureteric bud development GO:0001657 9.86 TGFB1 SMAD7 SMAD3
31 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.85 TNF TGFB1 SMAD3
32 positive regulation of bone mineralization GO:0030501 9.85 TGFB1 SMAD3 BMP6
33 positive regulation of B cell proliferation GO:0030890 9.85 TNFSF13B IL4 IL2 IL13
34 negative regulation of mitotic cell cycle GO:0045930 9.84 TNF TGFB1 SMAD3
35 endoderm development GO:0007492 9.83 TGFB1 SMAD4 SMAD3
36 microglial cell activation GO:0001774 9.83 TNF IL4 IL13
37 negative regulation of myoblast differentiation GO:0045662 9.81 TNF TGFB1 CXCL10
38 immune response GO:0006955 9.81 TNFSF13B TNF SMAD3 IL6 IL4 IL2
39 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.8 TNF IL6 IL4 IL2 IL13
40 leukocyte tethering or rolling GO:0050901 9.79 VCAM1 TNF SELE
41 positive regulation of SMAD protein signal transduction GO:0060391 9.77 TGFB1 SMAD4 BMP6
42 positive regulation of T cell proliferation GO:0042102 9.77 VCAM1 TNFSF13B IL6 IL4 IL2
43 positive regulation of interleukin-10 secretion GO:2001181 9.74 IL4 IL13
44 positive regulation of production of miRNAs involved in gene silencing by miRNA GO:1903800 9.73 TGFB1 IL6
45 positive regulation of mast cell degranulation GO:0043306 9.73 IL4 IL13
46 negative regulation of production of miRNAs involved in gene silencing by miRNA GO:1903799 9.73 TNF TGFB1
47 positive regulation of isotype switching to IgG isotypes GO:0048304 9.73 IL4 IL2
48 endothelial cell activation GO:0042118 9.73 SMAD4 CXCL10
49 negative regulation of lipid storage GO:0010888 9.73 TNF IL6
50 negative regulation of T-helper 17 cell differentiation GO:2000320 9.73 SMAD7 IL4 IL2

Molecular functions related to Localized Scleroderma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription regulatory region DNA binding GO:0044212 9.67 TNF SMAD7 SMAD4 SMAD3
2 collagen binding GO:0005518 9.46 SMAD7 SMAD4 SMAD3 DCN
3 growth factor activity GO:0008083 9.43 TGFB1 IL6 IL4 IL2 CCN2 BMP6
4 I-SMAD binding GO:0070411 9.4 SMAD7 SMAD4
5 type I transforming growth factor beta receptor binding GO:0034713 9.32 TGFB1 SMAD7
6 cytokine activity GO:0005125 9.28 TNFSF13B TNF TGFB1 IL6 IL4 IL2
7 sulfate binding GO:0043199 9.26 SMAD4 CD34

Sources for Localized Scleroderma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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