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MCID: LCL006
MIFTS: 65

Localized Scleroderma

Categories: Bone diseases, Genetic diseases, Immune diseases, Nephrological diseases, Neuronal diseases, Rare diseases, Respiratory diseases, Skin diseases
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Aliases & Classifications for Localized Scleroderma

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MalaCards integrated aliases for Localized Scleroderma:

Name: Localized Scleroderma 12 54 60 38 15 74
Morphea 12 54 56 74
Localized Fibrosing Scleroderma 54 60
Scleroderma, Circumscribed or Localized 12
Circumscribed Scleroderma 12
Scleroderma, Localized 54
Localised Morphoea 12
Localized Morphea 12

Characteristics:

Orphanet epidemiological data:

60
localized scleroderma
Inheritance: Not applicable; Prevalence: 1-9/100000 (Europe); Age of onset: All ages; Age of death: normal life expectancy;

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases
Anatomical: Skin diseases Immune diseases Neuronal diseases Respiratory diseases Bone diseases Nephrological diseases
See all MalaCards categories (disease lists)
ICD10: 34 35
Orphanet: 60  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:8472
KEGG 38 H01493
ICD9CM 36 701.0
MeSH 45 D012594
NCIt 51 C72069
SNOMED-CT 69 90424004
ICD10 34 L94.0
MESH via Orphanet 46 D012594
ICD10 via Orphanet 35 L94.0
UMLS via Orphanet 75 C0036420
Orphanet 60 ORPHA90289
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Summaries for Localized Scleroderma

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NIH Rare Diseases : 54 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.  

MalaCards based summary : Localized Scleroderma, also known as morphea, is related to keloids and fasciitis, 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 Degradation of the extracellular matrix and NF-KappaB Family Pathway. The drugs Lidocaine and Vaccines have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and bone, and related phenotypes are hyperpigmentation of the skin and hypopigmentation of the skin

Wikipedia : 77 Morphea, also called localized scleroderma or circumscribed scleroderma, is a form of scleroderma that... more...

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Related Diseases for Localized Scleroderma

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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 288)
# Related Disease Score Top Affiliating Genes
1 keloids 30.2 MMP1 SMAD3 TGFB1
2 fasciitis 30.1 BMP6 IL6 TNF
3 central nervous system vasculitis 29.7 IL6 TNF
4 polyarteritis nodosa 29.7 IL6 SELE SMAD3
5 vasculitis 29.4 SELE TNF VCAM1
6 dermatitis, atopic 29.4 DEFB4A SELE TNF
7 arthritis 29.3 IL6 MMP1 MMP13 TGFB1 TNF
8 systemic lupus erythematosus 29.0 IL6 SELE TGFB1 TNF VCAM1
9 osteoarthritis 29.0 IL6 MMP1 MMP13 TGFB1 TNF
10 rheumatoid arthritis 28.4 IL6 MMP1 MMP13 SELE TGFB1 TNF
11 systemic scleroderma 12.2
12 linear scleroderma 11.6
13 morpheaform basal cell carcinoma 11.6
14 hemifacial atrophy, progressive 11.5
15 facial hemiatrophy 11.5
16 atrophoderma of pierini and pasini 11.1
17 reynolds syndrome 10.4
18 fibromatosis, gingival, 1 10.4 MMP1 TGFB1
19 scleroderma, familial progressive 10.4
20 periodontitis, chronic 10.3 IL6 MMP1
21 cholecystolithiasis 10.3 BMP6 TGFB1
22 diabetic foot ulcers 10.3 IL6 TGFB1
23 tuberculoid leprosy 10.3 BMP6 TGFB1
24 kashin-beck disease 10.3 MMP1 TNF
25 scorpion envenomation 10.3 IL6 TNF
26 leukomalacia 10.2 IL6 TNF
27 angioimmunoblastic lymphadenopathy with dysproteinemia 10.2 IL6 TNF
28 autoimmune myocarditis 10.2 IL6 TNF
29 streptococcal toxic-shock syndrome 10.2 IL6 TNF
30 juvenile ankylosing spondylitis 10.2 IL6 TNF
31 anthracosis 10.2 BMP6 TNF
32 cardiogenic shock 10.2 IL6 TNF
33 gingival disease 10.2 IL6 MMP1 TGFB1
34 dupuytren contracture 10.2 BMP6 DCN TGFB1
35 clonorchiasis 10.2 TGFB1 TNF
36 null-cell leukemia 10.2 IL6 TNF
37 retinitis pigmentosa 55 10.2 IL6 TNF
38 crouzon syndrome 10.2 BMP6 DCN TGFB1
39 glossitis 10.2 IL6 TNF
40 hypertrophic scars 10.2 BMP6 SMAD3 TGFB1
41 bone chondrosarcoma 10.2 MMP1 MMP13
42 peyronie's disease 10.2 DCN SMAD3 TGFB1
43 acute vascular insufficiency of intestine 10.2 IL6 TNF
44 nephrogenic systemic fibrosis 10.2 DCN SMAD3 TGFB1
45 scleritis 10.2 SELE TNF
46 nontuberculous mycobacterial lung disease 10.2 BMP6 TNF
47 osteosclerotic myeloma 10.2 IL6 TNF
48 renal fibrosis 10.2 BMP6 SMAD3 TGFB1
49 lymphocytic vasculitis 10.2 SELE VCAM1
50 poems syndrome 10.2 IL6 TNF

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
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Symptoms & Phenotypes for Localized Scleroderma

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Human phenotypes related to Localized Scleroderma:

60 33 (show all 16)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperpigmentation of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0000953
2 hypopigmentation of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0001010
3 stiff skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0030053
4 cigarette-paper scars 60 33 hallmark (90%) Very frequent (99-80%) HP:0001073
5 skeletal muscle atrophy 60 33 frequent (33%) Frequent (79-30%) HP:0003202
6 arthralgia 60 33 occasional (7.5%) Occasional (29-5%) HP:0002829
7 lipoatrophy 60 33 occasional (7.5%) Occasional (29-5%) HP:0100578
8 myalgia 60 33 occasional (7.5%) Occasional (29-5%) HP:0003326
9 split hand 60 33 occasional (7.5%) Occasional (29-5%) HP:0001171
10 hemiatrophy of upper limb 60 33 occasional (7.5%) Occasional (29-5%) HP:0100558
11 flexion contracture of toe 60 33 occasional (7.5%) Occasional (29-5%) HP:0005830
12 scarring alopecia of scalp 60 33 occasional (7.5%) Occasional (29-5%) HP:0004552
13 hemiatrophy of lower limb 60 33 occasional (7.5%) Occasional (29-5%) HP:0100557
14 flexion contracture 60 Occasional (29-5%)
15 hemiatrophy 60 Occasional (29-5%)
16 dermal atrophy 60 Very frequent (99-80%)

UMLS symptoms related to Localized Scleroderma:


pruritus, exanthema

MGI Mouse Phenotypes related to Localized Scleroderma:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 growth/size/body region MP:0005378 10.09 BMP6 DCN IL6 MMP13 SELE SMAD3
2 cellular MP:0005384 10.06 DCN IL6 MMP13 SELE SMAD3 TGFB1
3 homeostasis/metabolism MP:0005376 10.06 DCN IL6 MMP1 MMP13 SELE SMAD3
4 cardiovascular system MP:0005385 10.02 IL6 MMP13 SELE SMAD3 TGFB1 TNF
5 immune system MP:0005387 9.97 DCN IL6 MMP1 SELE SMAD3 TGFB1
6 integument MP:0010771 9.87 DCN IL6 MMP13 SELE SMAD3 TGFB1
7 neoplasm MP:0002006 9.73 DCN IL6 MMP1 SMAD3 TGFB1 TNF
8 reproductive system MP:0005389 9.7 BMP6 DCN IL6 SMAD3 TGFB1 TNF
9 skeleton MP:0005390 9.5 BMP6 DCN IL6 MMP13 SMAD3 TGFB1
10 vision/eye MP:0005391 9.1 DCN IL6 SELE SMAD3 TGFB1 TNF
Sources

Drugs & Therapeutics for Localized Scleroderma

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

(show top 50) (show all 186)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Lidocaine Approved, Vet_approved Phase 4,Phase 2,Phase 3,Not Applicable 137-58-6 3676
2 Vaccines Phase 4
3 Immunologic Factors Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
4 Sodium Channel Blockers Phase 4,Phase 2,Phase 3
5 Anti-Arrhythmia Agents Phase 4,Phase 2,Phase 3
6 Central Nervous System Depressants Phase 4,Phase 2,Phase 3,Not Applicable
7 Anesthetics Phase 4,Phase 2,Phase 3,Not Applicable
8 Anesthetics, Local Phase 4,Phase 2,Phase 3
9 Diuretics, Potassium Sparing Phase 4,Phase 2,Phase 3
10 Peripheral Nervous System Agents Phase 4,Phase 2,Phase 3,Phase 1,Not Applicable
11
Imiquimod Approved, Investigational Phase 3 99011-02-6 57469
12
Sodium Citrate Approved, Investigational Phase 3 68-04-2
13
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
14
Cyclophosphamide Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 6055-19-2, 50-18-0 2907
15
Lenograstim Approved, Investigational Phase 3 135968-09-1
16
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
17
Acetylcholine Approved, Investigational Phase 3 51-84-3 187
18
Bosentan Approved, Investigational Phase 2, Phase 3 147536-97-8 104865
19
Omeprazole Approved, Investigational, Vet_approved Phase 3 73590-58-6 4594
20
Domperidone Approved, Investigational, Vet_approved Phase 3,Not Applicable 57808-66-9 3151
21
Dopamine Approved Phase 3 62-31-7, 51-61-6 681
22
Macitentan Approved Phase 2, Phase 3 441798-33-0
23
Citric Acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
24
Calcium Approved, Nutraceutical Phase 3,Phase 2,Phase 1,Early Phase 1 7440-70-2 271
25 Antirheumatic Agents Phase 3,Phase 2,Phase 1
26 Interferon Inducers Phase 3
27 interferons Phase 3,Phase 1
28 Adjuvants, Immunologic Phase 3
29 Phosphodiesterase Inhibitors Phase 3,Phase 2
30 Phosphodiesterase 5 Inhibitors Phase 3
31 Citrate Phase 3
32 Vasodilator Agents Phase 3,Phase 2
33 Sildenafil Citrate Phase 3,Not Applicable 171599-83-0
34 Pharmaceutical Solutions Phase 2, Phase 3,Phase 3,Not Applicable
35 Antineoplastic Agents, Alkylating Phase 3,Phase 2,Phase 1
36 Alkylating Agents Phase 3,Phase 2,Phase 1
37 Immunosuppressive Agents Phase 3,Phase 1,Phase 2
38 Anticoagulants Phase 3,Phase 1
39 Calcium, Dietary Phase 3,Phase 2,Phase 1,Early Phase 1
40 Chelating Agents Phase 3
41 Insulin, Globin Zinc Phase 3
42 Neuromuscular Agents Phase 3
43 Neurotransmitter Agents Phase 3
44 abobotulinumtoxinA Phase 3
45 Cholinergic Agents Phase 3
46 Acetylcholine Release Inhibitors Phase 3
47 insulin Phase 3
48 Botulinum Toxins Phase 3
49 Botulinum Toxins, Type A Phase 3
50 Antihypertensive Agents Phase 2, Phase 3,Phase 1

Interventional clinical trials:

(show top 50) (show all 90)
# Name Status NCT ID Phase Drugs
1 Influenza Vaccination in Patients With Scleroderma Unknown status NCT01002508 Phase 4
2 Efficacy and Safety of PLACENTEX ® i.m. in Patients With Scleroderma Diseases Recruiting NCT03388255 Phase 4 Polydeoxyribonucleotides
3 Pain Outcomes Following Intralesional Corticosteroid Injections Recruiting NCT03630198 Phase 4 Corticosteroid with lidocaine;Corticosteroid with normal saline
4 Imiquimod in Children With Plaque Morphea Completed NCT00147771 Phase 3 Imiquimod 5% cream
5 Sildenafil Effect on Digital Ulcer Healing in sClerodErma SEDUCE STUDY Completed NCT01295736 Phase 3 Sildenafil;placebo
6 Effectiveness and Safety of Lidocaine for Scleroderma Completed NCT00740285 Phase 2, Phase 3 Lidocaine 2% without vessel constrictor
7 High Dose Cyclophosphamide for Treatment of Scleroderma Completed NCT00501995 Phase 3 IV Cyclophosphamide
8 A Randomized Control Trial to Assess the Efficacy of Tadalafil in Raynaud's Phenomenon in Scleroderma Completed NCT01117298 Phase 3 Tadalafil;Placebo
9 Effect of Sildenafil on the Microcirculatory Blood Flow and Endothelial Progenitor Cells in Systemic Sclerosis Completed NCT01347008 Phase 3 Sildenafil citrate;Placebo (Sugar pill)
10 Efficacy of Botulinum Toxin In Scleroderma-Associated Raynaud's Syndrome Completed NCT02165111 Phase 3 Onabotulinumtoxin A;sterile saline solution
11 Efficacy and Safety of Oral Bosentan in Pulmonary Fibrosis Associated With Scleroderma Completed NCT00070590 Phase 2, Phase 3 Bosentan
12 Gastroesophageal Reflux Treatment in Scleroderma Completed NCT01878526 Phase 3 Alginic acid;placebo (for domperidone);Domperidone;placebo (of alginic acid)
13 Scleroderma Lung Disease Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
14 CompRehensive Phenotypic Characterization of Patients With Scleroderma-Associated ILD and PH Recruiting NCT03726398 Phase 2, Phase 3 Opsumit 10 Mg Tablet
15 Imiquimod 5% Cream in Plaque-type Morphea: A Pilot, Prospective Open-label Study Withdrawn NCT00230373 Phase 3 imiquimod 5% cream (Aldara)
16 A Protocol Based Treatment for Debilitating Fibrosing Skin Disorders With (Anti-CD 20), Rituximab, Evaluating Safety and Efficacy Unknown status NCT00936546 Phase 2 Rituximab
17 Efficacy and Safety of Imatinib in Scleroderma Completed NCT00479934 Phase 2 imatinib mesylate
18 Ultraviolet B (UVB) Light Therapy in the Treatment of Skin Conditions With Altered Dermal Matrix Completed NCT00129428 Phase 1, Phase 2
19 Effect of Bosentan in Scleroderma Renal Crisis Completed NCT01241383 Phase 2 Bosentan
20 Digital Ischemic Lesions in Scleroderma Treated With Oral Treprostinil Diethanolamine Completed NCT00775463 Phase 2 treprostinil diethanolamine;placebo
21 Safety Evaluation of Dasatinib in Subjects With Scleroderma Pulmonary Fibrosis Completed NCT00764309 Phase 1, Phase 2 dasatinib
22 A Study to Evaluate the Safety and Efficacy of Abatacept in Patients With Diffuse Systemic Sclerosis (Scleroderma) Completed NCT00442611 Phase 1, Phase 2 Abatacept;Placebo
23 Cyclophosphamide and rATG With Hematopoietic Stem Cell Support in Systemic Scleroderma Completed NCT00278525 Phase 2 standard of care
24 IL1-TRAP, Rilonacept, in Systemic Sclerosis Completed NCT01538719 Phase 1, Phase 2 Rilonacept
25 Oral Type I Collagen for Relieving Scleroderma Completed NCT00005675 Phase 2 Oral bovine type I collagen;Placebo
26 Riociguat in Scleroderma Associated Digital Ulcers Completed NCT02915835 Phase 2 Riociguat;Placebo
27 Diltiazem Gel Versus Nitroglycerin Ointment in Healing Process of Scleroderma Digital Ulcers Completed NCT02801305 Phase 2 Diltiazem Gel 2%;Nitroglycerin Ointment 2%;Vaseline
28 Zibotentan Better Renal Scleroderma Outcome Study Completed NCT02047708 Phase 2 Zibotentan
29 Comparison of Therapeutic Regimens for Scleroderma Interstitial Lung Disease (The Scleroderma Lung Study II) Completed NCT00883129 Phase 2 Mycophenolate mofetil;Cyclophosphamide;Placebo
30 Psychological Treatments for Scleroderma Completed NCT00007267 Phase 2
31 Study of Pomalidomide (CC-4047) to Evaluate Safety, Tolerability, Pharmacokinetics, Pharmacodynamics and Effectiveness for Subjects With Systemic Sclerosis With Interstitial Lung Disease Completed NCT01559129 Phase 2 Pomalidomide (CC-4047);Placebo
32 A Study of FCX-013 Plus Veledimex for the Treatment of Moderate to Severe Localized Scleroderma Recruiting NCT03740724 Phase 1, Phase 2 veledimex
33 Pilot Study Evaluating the Efficacy of a Topical PDE4 Inhibitor for Morphea Recruiting NCT03351114 Phase 2 Crisaborole
34 Pirfenidone Plus M-DDO Gel in Moderate and Severe Acne Recruiting NCT03076320 Phase 1, Phase 2
35 Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells Recruiting NCT02213705 Phase 1, Phase 2
36 Scleroderma Lung Study III - Combining Pirfenidone With Mycophenolate Recruiting NCT03221257 Phase 2 Pirfenidone (PFD);Placebo (Plac);Mycophenolate Mofetil (MMF)
37 Oral Ifetroban to Treat Diffuse Cutaneous Systemic Sclerosis (SSc) or SSc-associated Pulmonary Arterial Hypertension Recruiting NCT02682511 Phase 2 Oral Ifetroban;Oral Placebo
38 Atorvastatin for Microvascular Endothelial Function and Raynaud in Early Diffuse Scleroderma Active, not recruiting NCT02370784 Phase 2 atorvastatin;Placebo
39 Study to Assess Sarilumab in Halting Progression of Morphea Not yet recruiting NCT03679845 Phase 1, Phase 2 Sarilumab
40 Proof of Concept Trial of Gleevec (Imatinib) in Active Diffuse Scleroderma Terminated NCT01545427 Phase 2 Imatinib mesylate
41 Placebo Controlled Trial of Bosentan in Scleroderma Patients Terminated NCT00377455 Phase 2 Bosentan;Placebo
42 A Study of the Safety and Tolerability of MEDI-551 in Scleroderma Completed NCT00946699 Phase 1
43 A Study to Evaluate Safety and Tolerability of Multiple Doses of MEDI-546 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
44 Safety & Suitability of Dabigatran to Inhibit Thrombin in Scleroderma Completed NCT02426229 Phase 1 dabigatran etexilate
45 Safety and Value of Self Bone Marrow Transplants Following Chemotherapy in Scleroderma Patients Terminated NCT00040651 Phase 1 Fludarabine;Cyclophosphamide;Thymoglobulin
46 Comparative Effectiveness Trial in the Treatment of Pediatric Plaque Morphea Withdrawn NCT02680717 Phase 1 Clobetasol;Calcipotriene;Tacrolimus
47 Fractional Carbon Dioxide Laser Versus UVA 1 in Treatment of Localized Scleroderma Unknown status NCT02002897 Not Applicable
48 Genetic Variants in Linear Localized Scleroderma Unknown status NCT02222038 Not Applicable
49 Treatment Study Comparing UVA-1 Phototherapy Versus Placebo Treatment for Morphea Unknown status NCT01799174 Not Applicable
50 Empirical Comparative Study of Variation Blood Level Antibody Vitamin D at Scleroderma (SSc) Patients Compared Healthy Peoples Unknown status NCT01553890 Not Applicable

Search NIH Clinical Center for Localized Scleroderma

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Genetic Tests for Localized Scleroderma

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Anatomical Context for Localized Scleroderma

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MalaCards organs/tissues related to Localized Scleroderma:

42
Skin, Breast, Bone, Lung, Endothelial, Bone Marrow, Skeletal Muscle
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Publications for Localized Scleroderma

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Articles related to Localized Scleroderma:

(show top 50) (show all 922)
# Title Authors Year
1
Comparison of 2-D shear wave elastography with clinical score in localized scleroderma: A new method to increase the diagnostic accuracy. ( 30506714 )
2019
2
Identifying the Signature Immune Phenotypes Present in Pediatric Localized Scleroderma. ( 30616925 )
2019
3
An extended high-frequency ultrasound protocol for assessing and quantifying of inflammation and fibrosis in localized scleroderma. ( 30638285 )
2019
4
Inflammatory morphea presenting as a hemifacial acquired port-wine stain. ( 29449213 )
2019
5
A Case of Pansclerotic Morphea Treated With Tocilizumab. ( 30649148 )
2019
6
Juvenile case of multiple morphea profunda resulting in joint contracture that was successfully treated with cyclosporin A: A case report and review of the published works. ( 30701571 )
2019
7
Treatment of Morphea With Hydroxychloroquine: A Retrospective Review of 84 Patients at Mayo Clinic, 1996-2013. ( 30703458 )
2019
8
Cutaneous lymphoid hyperplasia arising in pre-existing morphea plaques treated with methotrexate. ( 30705931 )
2019
9
An evaluation of long-term outcomes and recurrence rates in patients with morphea. ( 30710337 )
2019
10
A case of linear morphea involving the oral cavity. ( 30733981 )
2019
11
Radiation-induced morphea: autoimmunity as a risk factor. ( 30774102 )
2019
12
Dermoscopy of Morphea. ( 30775314 )
2019
13
Isomorphic Morphea in a Girl Motorcyclist. ( 30795836 )
2019
14
Radiation-induced morphea: association with autoimmune comorbidities, severity, and response to therapy. ( 30797843 )
2019
15
Challenges in the diagnosis and treatment of disabling pansclerotic morphea of childhood: case-based review. ( 30838436 )
2019
16
Collagen-enriched serpiginous skin lesion in a cat resembling the linear form of localized scleroderma in humans. ( 29794370 )
2018
17
Impact of a new simplified disability scoring system for adult patients with localized scleroderma. ( 29369402 )
2018
18
Development of minimum standards of care for juvenile localized scleroderma. ( 29728839 )
2018
19
Diagnostic criteria, severity classification and guidelines of localized scleroderma. ( 29687475 )
2018
20
Supportive Use of Adipose-Derived Stem Cells in Cell-Assisted Lipotransfer for Localized Scleroderma. ( 29794702 )
2018
21
Tocilizumab as a potential therapeutic option for children with severe, refractory juvenile localized scleroderma. ( 29077971 )
2018
22
Detection of autoimmune antibodies in localized scleroderma by synthetic oligonucleotide antigens. ( 29641558 )
2018
23
Abatacept Improves Skin-score and Reduces Lesions in Patients with Localized Scleroderma: A Case Series. ( 29313055 )
2018
24
Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review. ( 29729451 )
2018
25
Localized scleroderma occurring after treatment of chronic hepatitis C with sofosbuvir and ribavirin. ( 29863777 )
2018
26
Comparing ultraviolet light A photo(chemo)therapy with Methotrexate protocol in childhood localized scleroderma: Evidence from systematic review and meta-analysis approach. ( 29655520 )
2018
27
From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution. ( 29666638 )
2018
28
Reliability of LoSCAT score for activity and tissue damage assessment in a large cohort of patients with Juvenile Localized Scleroderma. ( 29914516 )
2018
29
Localized Scleroderma of the Head and Face Area: A Retrospective Cross-sectional Study of 96 Patients from 5 German Tertiary Referral Centres. ( 29507998 )
2018
30
Juvenile Localized Scleroderma with Hyaline Deposits in the Renal Arteriole. ( 29805370 )
2018
31
Giant morphea-form basal cell carcinoma of the umbilicus: Successful debulking with vismodegib. ( 29760872 )
2018
32
Comorbidity of localized scleroderma and primary biliary cholangitis. ( 30395408 )
2018
33
Fluoroscopy-induced chronic radiation dermatitis masquerading as morphea: A diagnostic pitfall. ( 30004062 )
2018
34
Localized scleroderma causing enophthalmos: A rare entity. ( 30355873 )
2018
35
Treatment of generalized deep morphea and eosinophilic fasciitis with the Janus kinase inhibitor tofacitinib. ( 29984277 )
2018
36
Granulomatous fasciitis followed by morphea profunda: Is granulomatous fasciitis part of a spectrum of deep morphea? A case report and review of the literature. ( 30147873 )
2018
37
Morphea «En Coup De Sabre» at the Site of Healed Herpes Zoster Ophthalmicus. ( 30201544 )
2018
38
Assessing the response of morphea and limited scleroderma to tranilast: a small prospective study comparing topical corticosteroids to a combination of topical corticosteroids and tranilast. ( 30013378 )
2018
39
Erratum to "From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution". ( 29973952 )
2018
40
Detecting and quantifying activity/inflammation in localized scleroderma with thermal imaging. ( 30030915 )
2018
41
Scleroderma in Children and Adolescents: Localized Scleroderma and Systemic Sclerosis. ( 30031497 )
2018
42
Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) adapted for use in adult patients: report from an initial validation study. ( 30217204 )
2018
43
New Features for Measuring Disease Activity in Pediatric Localized Scleroderma. ( 30219769 )
2018
44
Progranulin overproduction due to constitutively activated c-Abl/PKC-δ/Fli1 pathway contributes to the resistance of dermal fibroblasts to the anti-fibrotic effect of tumor necrosis factor-α in localized scleroderma. ( 30268392 )
2018
45
Prediction of disease relapse in a cohort of paediatric patients with localized scleroderma. ( 30315656 )
2018
46
A case of juvenile localized scleroderma with anti-topoisomerase I antibody. ( 30442630 )
2018
47
The correlation between serum E-selectin levels and soluble interleukin-2 receptors with relation to disease activity in localized scleroderma. ( 30618531 )
2018
48
Morphea associated with primary biliary cirrhosis and Waldenstrom macroglobulinemia: Response to rituximab. ( 30246126 )
2018
49
Morphea and systemic sclerosis are associated with an increased risk of melanoma and non-melanoma skin cancer. ( 30352282 )
2018
50
Early morphea simulating patch-stage mycosis fungoides in two cases. ( 29760873 )
2018
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Variations for Localized Scleroderma

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Expression for Localized Scleroderma

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Search GEO for disease gene expression data for Localized Scleroderma.
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Pathways for Localized Scleroderma

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

(show top 50) (show all 53)
# Super pathways Score Top Affiliating Genes
1
Degradation of the extracellular matrix 67
Show member pathways
 12.63 DCN MMP1 MMP13 TGFB1 VCAM1
2
NF-KappaB Family Pathway 65
Show member pathways
 12.6 BMP6 IL6 TGFB1 TNF
3
Th17 cell differentiation 38
Show member pathways
 12.48 IL6 SMAD3 TGFB1 TNF
4
Human T-cell leukemia virus 1 infection 38
12.22 IL6 SMAD3 TGFB1 TNF VCAM1
5
IL-17 Family Signaling Pathways 66
Show member pathways
 12.19 DEFB4A IL6 MMP1 MMP13 TNF
6
Immune response IFN alpha/beta signaling pathway 23
Show member pathways
 12.15 BMP6 IL6 TGFB1 TNF
7
Cell surface interactions at the vascular wall 67
Show member pathways
 12.14 MMP1 SELE TGFB1
8
Tuberculosis 38
12.14 IL6 TGFB1 TNF
9
Dilated cardiomyopathy (DCM) 38
Show member pathways
 12.13 IL6 TGFB1 TNF
10
Bacterial infections in CF airways 23
Show member pathways
 12.1 DEFB4A IL6 TNF
11
Cellular senescence (KEGG) 38
12.08 IL6 SMAD3 TGFB1
12
Hippo signaling pathway 38
12.06 BMP6 SMAD3 TGFB1
13
Colorectal Cancer Metastasis 65
12.05 IL6 MMP1 SMAD3 TGFB1 TNF
14
Transcription Androgen Receptor nuclear signaling 23
Show member pathways
 12.03 IL6 SMAD3 TGFB1
15
Fluid shear stress and atherosclerosis 38
12 SELE TNF VCAM1
16
FoxO signaling pathway 38
Show member pathways
 12 IL6 SMAD3 TGFB1
17
SMAD Signaling Network 65
11.97 BMP6 SMAD3 TGFB1
18
Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers 66
11.97 IL6 TGFB1 VCAM1
19
TGF-beta Signaling Pathway (WikiPathways) 1
11.97 MMP1 SMAD3 TGFB1
20
Adipogenesis 1
11.97 IL6 SMAD3 TGFB1 TNF
21
Immune response IFN gamma signaling pathway 23
Show member pathways
 11.96 BMP6 IL6 SMAD3 TGFB1
22
Th1 Differentiation Pathway 66
Show member pathways
 11.94 IL6 SMAD3 TGFB1 TNF
23
Spinal Cord Injury 1
11.91 IL6 TGFB1 TNF
24
Senescence and Autophagy in Cancer 1
11.85 IL6 SMAD3 TGFB1
25
TNF signaling pathway 38
11.85 IL6 SELE TNF VCAM1
26
Matrix Metalloproteinases 1
Show member pathways
 11.8 MMP1 MMP13 TNF
27
Amoebiasis 38
11.79 IL6 TGFB1 TNF
28
Interleukin-4 and 13 signaling 67
11.78 IL6 MMP1 TGFB1 TNF VCAM1
29
Cytokine production by Th17 cells in CF (Mouse model) 23
Show member pathways
 11.77 IL6 SMAD3 TGFB1
30
Rheumatoid arthritis 38
11.75 IL6 MMP1 TGFB1 TNF
31
Glucocorticoid receptor regulatory network 1
11.71 IL6 MMP1 SELE
32
Dendritic Cells Developmental Lineage Pathway 66
11.66 IL6 TGFB1 TNF
33
Microglia Activation During Neuroinflammation: Microglia Polarization 66
11.63 IL6 TGFB1 TNF
34
Transcription_P53 signaling pathway 23
11.61 BMP6 SMAD3 TGFB1
35
TGF-beta signaling pathway (KEGG) 38
11.61 BMP6 DCN SMAD3 TGFB1 TNF
36
Endochondral Ossification 1
11.59 BMP6 MMP13 TGFB1
37
Lung fibrosis 1
11.57 IL6 TGFB1 TNF
38
TGF-beta Receptor Signaling (WikiPathways) 1
11.52 SMAD3 TGFB1 TNF
39
Hepatitis C and Hepatocellular Carcinoma 1
11.41 IL6 MMP1 SMAD3 TGFB1
40
Transcription_Role of VDR in regulation of genes involved in osteoporosis 23
11.4 IL6 SMAD3 TNF
41
Photodynamic therapy-induced NF-kB survival signaling 1
Show member pathways
 11.35 IL6 MMP1 SELE TNF VCAM1
42
Validated transcriptional targets of AP1 family members Fra1 and Fra2 1
11.27 DCN IL6 MMP1
43
African trypanosomiasis 38
11.24 IL6 SELE TNF VCAM1
44
Cytokines and Inflammatory Response 1
11.17 IL6 TGFB1 TNF
45
AGE-RAGE signaling pathway in diabetic complications 38
11.1 IL6 SELE SMAD3 TGFB1 TNF VCAM1
46
Cellular roles of Anthrax toxin 1
11.04 TNF VCAM1
47
Transcription factor regulation in adipogenesis 1
11.04 IL6 TNF
48
Malaria 38
11.04 IL6 SELE TGFB1 TNF VCAM1
49
Overview of nanoparticle effects 1
10.97 IL6 TNF
50
TGF-B Signaling in Thyroid Cells for Epithelial-Mesenchymal Transition 1
10.93 SMAD3 TGFB1
Sources

GO Terms for Localized Scleroderma

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.61 BMP6 DCN DEFB1 DEFB4A IL6 MMP1
2 extracellular matrix GO:0031012 9.56 DCN MMP1 MMP13 TGFB1
3 Golgi lumen GO:0005796 9.46 DCN DEFB1 DEFB4A TGFB1
4 extracellular space GO:0005615 9.32 BMP6 DCN DEFB1 DEFB4A IL6 MMP13

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

(show all 44)
# Name GO ID Score Top Affiliating Genes
1 regulation of signaling receptor activity GO:0010469 9.96 BMP6 IL6 TGFB1 TNF
2 positive regulation of gene expression GO:0010628 9.94 IL6 SMAD3 TGFB1 TNF
3 aging GO:0007568 9.87 DCN TGFB1 VCAM1
4 response to hypoxia GO:0001666 9.86 SMAD3 TGFB1 VCAM1
5 response to lipopolysaccharide GO:0032496 9.85 DCN SELE VCAM1
6 leukocyte migration GO:0050900 9.84 MMP1 SELE TGFB1 TNF
7 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.83 IL6 TGFB1 TNF
8 inflammatory response GO:0006954 9.83 BMP6 IL6 SELE TGFB1 TNF
9 defense response GO:0006952 9.82 DEFB1 DEFB4A TNF
10 positive regulation of DNA-binding transcription factor activity GO:0051091 9.81 IL6 SMAD3 TNF
11 wound healing GO:0042060 9.79 DCN SMAD3 TGFB1
12 defense response to Gram-negative bacterium GO:0050829 9.79 DEFB1 DEFB4A IL6
13 positive regulation of peptidyl-serine phosphorylation GO:0033138 9.77 IL6 TGFB1 TNF
14 response to glucocorticoid GO:0051384 9.75 BMP6 IL6 TNF
15 SMAD protein signal transduction GO:0060395 9.73 BMP6 SMAD3 TGFB1
16 cytokine-mediated signaling pathway GO:0019221 9.72 IL6 MMP1 TGFB1 TNF VCAM1
17 endochondral ossification GO:0001958 9.69 BMP6 MMP13
18 negative regulation of myoblast differentiation GO:0045662 9.68 TGFB1 TNF
19 negative regulation of neurogenesis GO:0050768 9.68 IL6 TNF
20 positive regulation of protein complex assembly GO:0031334 9.68 TGFB1 TNF
21 cell-cell junction organization GO:0045216 9.67 SMAD3 TGFB1
22 calcium-mediated signaling using intracellular calcium source GO:0035584 9.67 DEFB1 VCAM1
23 positive regulation of chondrocyte differentiation GO:0032332 9.67 BMP6 SMAD3
24 extrinsic apoptotic signaling pathway GO:0097191 9.67 SMAD3 TGFB1 TNF
25 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.66 SMAD3 TGFB1
26 positive regulation of chemokine production GO:0032722 9.65 IL6 TNF
27 positive regulation of glial cell proliferation GO:0060252 9.64 IL6 TNF
28 lens fiber cell differentiation GO:0070306 9.63 SMAD3 TGFB1
29 negative regulation of mitotic cell cycle GO:0045930 9.63 SMAD3 TGFB1 TNF
30 positive regulation of SMAD protein signal transduction GO:0060391 9.62 BMP6 TGFB1
31 negative regulation of lipid storage GO:0010888 9.61 IL6 TNF
32 SMAD protein complex assembly GO:0007183 9.58 SMAD3 TGFB1
33 positive regulation of bone mineralization GO:0030501 9.58 BMP6 SMAD3 TGFB1
34 positive regulation of neuroinflammatory response GO:0150078 9.57 IL6 TNF
35 defense response to Gram-positive bacterium GO:0050830 9.56 DEFB1 DEFB4A IL6 TNF
36 positive regulation of extracellular matrix assembly GO:1901203 9.55 SMAD3 TGFB1
37 regulation of binding GO:0051098 9.52 SMAD3 TGFB1
38 regulation of striated muscle tissue development GO:0016202 9.51 SMAD3 TGFB1
39 positive regulation of mononuclear cell migration GO:0071677 9.48 TGFB1 TNF
40 immune response GO:0006955 9.43 BMP6 DEFB1 DEFB4A IL6 SMAD3 TNF
41 evasion or tolerance of host defenses by virus GO:0019049 9.37 SMAD3 TGFB1
42 leukocyte tethering or rolling GO:0050901 9.33 SELE TNF VCAM1
43 negative regulation of fat cell differentiation GO:0045599 8.92 IL6 SMAD3 TGFB1 TNF
44 positive regulation of transcription by RNA polymerase II GO:0045944 10.07 BMP6 DCN IL6 SMAD3 TGFB1 TNF

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

# Name GO ID Score Top Affiliating Genes
1 growth factor activity GO:0008083 9.5 BMP6 IL6 TGFB1
2 collagen binding GO:0005518 9.33 DCN MMP13 SMAD3
3 CCR6 chemokine receptor binding GO:0031731 9.26 DEFB1 DEFB4A
4 cytokine activity GO:0005125 9.26 BMP6 IL6 TGFB1 TNF
5 transforming growth factor beta receptor binding GO:0005160 8.8 BMP6 SMAD3 TGFB1
Sources

Sources for Localized Scleroderma

About this section
3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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