MCID: LCL006
MIFTS: 65

Localized Scleroderma

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

Aliases & Classifications for Localized Scleroderma

MalaCards integrated aliases for Localized Scleroderma:

Name: Localized Scleroderma 12 20 58 36 15 71
Morphea 12 20 54 71
Localized Fibrosing Scleroderma 20 58
Localised Scleroderma 12 17
Scleroderma, Circumscribed or Localised 12
Scleroderma, Circumscribed or Localized 12
Circumscribed Scleroderma 12
Scleroderma, Localized 20
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


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 Innate Immune System and ERK Signaling. The drugs Sodium citrate and Tadalafil have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related phenotypes are thickened skin and arthralgia

GARD : 20 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 667)
# Related Disease Score Top Affiliating Genes
1 systemic scleroderma 31.9 TNF TGFB1 SELE IL6 IL4 IL13
2 scleroderma, familial progressive 31.6 COL1A1 CCN2 BMP6
3 keloid disorder 30.8 TGFB1 BMP6
4 diffuse scleroderma 30.7 TGFB1 COL1A1 CCN2
5 fasciitis 30.5 TNF MMP1 IL6 IL2 CD34
6 esophagitis 30.4 TNF IL6 IL4 IL13
7 scleredema adultorum 30.3 MMP1 DCN CD34
8 gastroesophageal reflux 30.3 TNF TGFB1 IL6 IL4
9 basal cell carcinoma 30.3 TNF MMP1 IL6 IL2 CD34
10 uveitis 30.2 TNF IL6 IL4 IL2 CXCL10
11 exanthem 30.2 TNF IL6 IL2
12 purpura 30.2 TNFSF13B TNF IL6 IL4
13 lichen planus 30.1 TNF IL6 IL4 IL2
14 hypertrophic scars 30.1 TGFB1 SMAD3 BMP6
15 osteomyelitis 30.1 TNF IL6 IL4 CXCL10
16 liver cirrhosis 30.1 TNF TGFB1 IL6 CD34 CCN2
17 pemphigus 30.0 TNFSF13B TNF IL6 IL4
18 pemphigoid 30.0 TNF TGFB1 IL4 IL13
19 tuberculoid leprosy 30.0 TGFB1 IL2 BMP6
20 central nervous system vasculitis 30.0 TNFSF13B TNF IL6 IL2
21 graft-versus-host disease 30.0 TNF IL6 IL2
22 chronic ulcer of skin 30.0 TNF MMP1 IL6
23 synovitis 30.0 VCAM1 TNF SELE MMP1 IL6
24 keratoconjunctivitis sicca 30.0 TNFSF13B TNF IL4
25 chlamydia 30.0 TNF IL6 IL4
26 cutaneous lupus erythematosus 29.9 TNFSF13B TNF SELE IL6
27 limb ischemia 29.9 TNF SELE IL6
28 chickenpox 29.9 TNF IL6 IL4 IL2
29 mixed connective tissue disease 29.9 TNFSF13B TNF IL6
30 bone resorption disease 29.9 TNF IL6 COL1A1
31 thyroiditis 29.9 TNF IL6 IL4 IL2 CXCL10
32 bronchiolitis obliterans 29.8 TNF TGFB1 IL6 IL13 BMP6
33 carpal tunnel syndrome 29.8 TGFB1 IL6 IL2 COL1A1 CCN2
34 toxoplasmosis 29.8 TNF TGFB1 IL6 IL4
35 vasculitis 29.8 VCAM1 TNFSF13B TNF SELE IL6
36 bullous pemphigoid 29.8 TNFSF13B TNF IL4 IL13
37 crohn's disease 29.7 TNF IL6 IL4 IL2 IL13
38 antiphospholipid syndrome 29.7 VCAM1 TNF SELE
39 bronchiolitis 29.7 TNF IL6 IL4 IL13 CXCL10
40 diphtheria 29.7 TNF IL4 IL2 IL13
41 alopecia areata 29.7 TNF IL6 IL4 IL2 IL13 CXCL10
42 peripheral nervous system disease 29.7 TNF IL6 IL4 IL2 CD34
43 leprosy 3 29.6 TNF IL6 IL4 IL2 IL13 CXCL10
44 lung disease 29.6 TNF TGFB1 IL6 IL4 IL13 CXCL10
45 connective tissue disease 29.6 TNFSF13B TNF SMAD3 IL6 IL4 IL2
46 nephrogenic systemic fibrosis 29.6 TGFB1 SMAD7 SMAD3 DCN CCN2
47 skin carcinoma 29.6 TNF MMP1 IL6 IL4 IL2 CD34
48 dermatitis 29.6 TNF IL6 IL4 IL2 IL13 CCL18
49 cryoglobulinemia 29.6 TNFSF13B TNF IL6 CXCL10
50 autoimmune disease 29.6 VCAM1 TNFSF13B TNF TGFB1 IL6 IL4

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 top 50) (show all 54)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 thickened skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0001072
2 arthralgia 58 31 frequent (33%) Frequent (79-30%) HP:0002829
3 stiff skin 58 31 frequent (33%) Frequent (79-30%) HP:0030053
4 cutaneous sclerotic plaque 58 31 frequent (33%) Frequent (79-30%) HP:0031359
5 dental malocclusion 58 31 occasional (7.5%) Occasional (29-5%) HP:0000689
6 arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001369
7 flexion contracture 58 31 occasional (7.5%) Occasional (29-5%) HP:0001371
8 gastroesophageal reflux 58 31 occasional (7.5%) Occasional (29-5%) HP:0002020
9 myopathy 58 31 occasional (7.5%) Occasional (29-5%) HP:0003198
10 abnormality of vision 58 31 occasional (7.5%) Occasional (29-5%) HP:0000504
11 uveitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000554
12 hypopigmented skin patches 58 31 occasional (7.5%) Occasional (29-5%) HP:0001053
13 migraine 58 31 occasional (7.5%) Occasional (29-5%) HP:0002076
14 erythema 58 31 occasional (7.5%) Occasional (29-5%) HP:0010783
15 infra-orbital crease 58 31 occasional (7.5%) Occasional (29-5%) HP:0100876
16 upper limb asymmetry 58 31 occasional (7.5%) Occasional (29-5%) HP:0100560
17 fasciitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0100537
18 abnormality of upper lip 58 31 occasional (7.5%) Occasional (29-5%) HP:0000177
19 patchy alopecia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002232
20 hyperpigmentation of the skin 58 31 occasional (7.5%) Occasional (29-5%) HP:0000953
21 abnormality of the cheek 58 31 occasional (7.5%) Occasional (29-5%) HP:0004426
22 erythematous plaque 58 31 occasional (7.5%) Occasional (29-5%) HP:0025474
23 hemifacial atrophy 58 31 occasional (7.5%) Occasional (29-5%) HP:0011331
24 sclerosis of finger phalanx 58 31 occasional (7.5%) Occasional (29-5%) HP:0100899
25 short dental roots 58 31 occasional (7.5%) Occasional (29-5%) HP:0006336
26 abnormality of facial skeleton 58 31 occasional (7.5%) Occasional (29-5%) HP:0011821
27 progressive loss of facial adipose tissue 58 31 occasional (7.5%) Occasional (29-5%) HP:0009019
28 skeletal muscle atrophy 58 31 very rare (1%) Very rare (<4-1%) HP:0003202
29 hashimoto thyroiditis 58 31 very rare (1%) Very rare (<4-1%) HP:0000872
30 vitiligo 58 31 very rare (1%) Very rare (<4-1%) HP:0001045
31 arrhythmia 58 31 very rare (1%) Very rare (<4-1%) HP:0011675
32 deeply set eye 58 31 very rare (1%) Very rare (<4-1%) HP:0000490
33 proptosis 58 31 very rare (1%) Very rare (<4-1%) HP:0000520
34 stroke 58 31 very rare (1%) Very rare (<4-1%) HP:0001297
35 abnormality of the kidney 58 31 very rare (1%) Very rare (<4-1%) HP:0000077
36 vasculitis 58 31 very rare (1%) Very rare (<4-1%) HP:0002633
37 abnormality of the nose 58 31 very rare (1%) Very rare (<4-1%) HP:0000366
38 focal impaired awareness seizure 58 31 very rare (1%) Very rare (<4-1%) HP:0002384
39 abnormal blistering of the skin 58 31 very rare (1%) Very rare (<4-1%) HP:0008066
40 skin erosion 58 31 very rare (1%) Very rare (<4-1%) HP:0200041
41 raynaud phenomenon 58 31 very rare (1%) Very rare (<4-1%) HP:0030880
42 esophagitis 58 31 very rare (1%) Very rare (<4-1%) HP:0100633
43 abnormality on pulmonary function testing 58 31 very rare (1%) Very rare (<4-1%) HP:0030878
44 seizures 58 Very rare (<4-1%)
45 abnormality of the dentition 58 Occasional (29-5%)
46 abnormality of the cardiovascular system 58 Very rare (<4-1%)
47 autoimmunity 58 Occasional (29-5%)
48 facial asymmetry 58 Occasional (29-5%)
49 headache 58 Occasional (29-5%)
50 abnormality of the nervous system 58 Very rare (<4-1%)

UMLS symptoms related to Localized Scleroderma:


pruritus, exanthema

MGI Mouse Phenotypes related to Localized Scleroderma:

46 (show all 17)
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.43 CCN2 CD34 COL1A1 CXCL10 DCN IL13
2 homeostasis/metabolism MP:0005376 10.38 CCN2 CD34 COL1A1 CXCL10 DCN IL13
3 hematopoietic system MP:0005397 10.37 CD34 COL1A1 CXCL10 DCN IL13 IL2
4 growth/size/body region MP:0005378 10.36 BMP6 CCN2 COL1A1 DCN IL13 IL2
5 immune system MP:0005387 10.36 CD34 COL1A1 CXCL10 DCN IL13 IL2
6 cardiovascular system MP:0005385 10.31 CCN2 COL1A1 CXCL10 IL2 IL6 SELE
7 digestive/alimentary MP:0005381 10.28 CCN2 COL1A1 DCN IL13 IL2 IL4
8 endocrine/exocrine gland MP:0005379 10.26 CCN2 COL1A1 DCN IL13 IL2 IL4
9 integument MP:0010771 10.21 CCN2 CD34 COL1A1 DCN IL13 IL4
10 mortality/aging MP:0010768 10.21 CCN2 COL1A1 CXCL10 DCN IL13 IL2
11 craniofacial MP:0005382 10.15 CCN2 COL1A1 DCN IL4 SMAD3 SMAD7
12 liver/biliary system MP:0005370 10.06 CCN2 COL1A1 IL2 IL4 IL6 SELE
13 reproductive system MP:0005389 10.03 BMP6 COL1A1 DCN IL13 IL2 IL4
14 neoplasm MP:0002006 9.97 CD34 COL1A1 DCN IL2 IL6 MMP1
15 respiratory system MP:0005388 9.85 CCN2 COL1A1 DCN IL13 IL2 IL4
16 skeleton MP:0005390 9.65 BMP6 CCN2 COL1A1 DCN IL13 IL4
17 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 146)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sodium citrate Approved, Investigational Phase 3 68-04-2
2
Tadalafil Approved, Investigational Phase 3 171596-29-5 110635
3
Lidocaine Approved, Vet_approved Phase 2, Phase 3 137-58-6 3676
4
Sargramostim Approved, Investigational Phase 3 123774-72-1, 83869-56-1
5
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
6
Mycophenolic acid Approved Phase 3 24280-93-1 446541
7
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
8 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
9
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
10
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
11
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
12
Lenograstim Approved, Investigational Phase 3 135968-09-1
13
Macitentan Approved Phase 2, Phase 3 441798-33-0
14
Mesna Approved, Investigational Phase 3 3375-50-6 598
15
Citric acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
16
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
17 Pharmaceutical Solutions Phase 3
18 Citrate Phase 3
19 Phosphodiesterase Inhibitors Phase 3
20 Phosphodiesterase 5 Inhibitors Phase 3
21 Anticoagulants Phase 3
22 Calcium, Dietary Phase 3
23 Chelating Agents Phase 3
24 Vasodilator Agents Phase 3
25 Insulin, Globin Zinc Phase 3
26 insulin Phase 3
27 Alkylating Agents Phase 3
28 Sodium Channel Blockers Phase 2, Phase 3
29 Anti-Arrhythmia Agents Phase 2, Phase 3
30 Diuretics, Potassium Sparing Phase 2, Phase 3
31 Anesthetics, Local Phase 2, Phase 3
32 Immunosuppressive Agents Phase 3
33 Endothelin Receptor Antagonists Phase 2, Phase 3
34 Antilymphocyte Serum Phase 3
35 Thymoglobulin Phase 3
36 Methylprednisolone Acetate Phase 3
37 Endothelin A Receptor Antagonists Phase 2, Phase 3
38
Calcium Nutraceutical Phase 3 7440-70-2 271
39
Epoprostenol Approved Phase 2 61849-14-7, 35121-78-9 5280427 5282411
40
Treprostinil Approved, Investigational Phase 2 81846-19-7 54786 6918140
41
Diltiazem Approved, Investigational Phase 2 42399-41-7 39186
42
Petrolatum Approved, Investigational Phase 2 8009-03-8
43
Nitroglycerin Approved, Investigational Phase 2 55-63-0 4510
44
Abatacept Approved Phase 1, Phase 2 332348-12-6 10237
45
Dasatinib Approved, Investigational Phase 1, Phase 2 302962-49-8 3062316
46
Rilonacept Approved, Investigational Phase 1, Phase 2 501081-76-1 104924
47
Benzocaine Approved, Investigational Phase 2 1994-09-7, 94-09-7 2337
48
tannic acid Approved Phase 2 1401-55-4
49
Riociguat Approved Phase 2 625115-55-1
50
Pirfenidone Approved, Investigational Phase 2 53179-13-8 40632

Interventional clinical trials:

(show top 50) (show all 101)
# 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 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)
5 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
6 Cyclophosphamide Versus Placebo in Scleroderma Lung Study Completed NCT00004563 Phase 3 Cyclophosphamide;Placebo
7 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
8 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
9 Effectiveness and Safety of Lidocaine for Scleroderma. Randomized Double-Blind Clinical Trial Completed NCT00740285 Phase 2, Phase 3 Lidocaine 2% without vessel constrictor
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 Upfront Autologous Hematopoietic Stem Cell Transplantation Versus Immunosuppressive Medication in Early Diffuse Cutaneous Systemic Sclerosis: an International Multicentre, Open-label, Randomized Con-trolled Trial Recruiting NCT04464434 Phase 3
13 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)
14 A Protocol Based Treatment for Debilitating Fibrosing Skin Disorders With (Anti-CD 20), Rituximab, Evaluating Safety and Efficacy Unknown status NCT00936546 Phase 2 Rituximab
15 Pilot Study Evaluating the Efficacy of a Topical PDE4 Inhibitor for Morphea Completed NCT03351114 Phase 2 Crisaborole
16 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
17 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
18 Effect of Bosentan in the Course of Scleroderma Renal Crisis Completed NCT01241383 Phase 2 Bosentan
19 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
20 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
21 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
22 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
23 A Pilot Study to Evaluate the Safety and Efficacy of Abatacept in Patients With Systemic Sclerosis Completed NCT00442611 Phase 1, Phase 2 Abatacept;Placebo
24 Psychosocial Interventions for Scleroderma Completed NCT00007267 Phase 2
25 Multicenter Phase II Trial of Oral Type I Bovine Collagen in Scleroderma Completed NCT00005675 Phase 2 Oral bovine type I collagen;Placebo
26 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
27 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
28 Mycophenolate vs. Oral Cyclophosphamide in Scleroderma Interstitial Lung Disease (Scleroderma Lung Study II) Completed NCT00883129 Phase 2 Mycophenolate mofetil;Cyclophosphamide;Placebo
29 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
30 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
31 A Randomized, Placebo-controlled Phase IIa Clinical Trial to Evaluate the Efficacy and Safety of Subcutaneous Dupilumab in Localized Scleroderma Recruiting NCT04200755 Phase 2 Dupilumab 300Mg Solution for Injection
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 Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells Active, not recruiting NCT02213705 Phase 1, Phase 2
35 Scleroderma Lung Study III (SLS III): Combining the Anti-fibrotic Effects of Pirfenidone (PFD) With Mycophenolate (MMF) for Treating Scleroderma-related Interstitial Lung Disease Active, not recruiting NCT03221257 Phase 2 Pirfenidone (PFD);Placebo (Plac);Mycophenolate Mofetil (MMF)
36 A Phase II, Randomised, Double Blind, Placebo-controlled Study of the Pharmacokinetics, Pharmacodynamic Effects, and Safety, of Oral FT011 in Participants With Diffuse Systemic Sclerosis Not yet recruiting NCT04647890 Phase 2 FT011;FT011;Placebo
37 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
38 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
39 A Proof of Concept Trial of Gleevec (Imatinib) in Active Diffuse Scleroderma Terminated NCT01545427 Phase 2 Imatinib mesylate
40 A Phase 1, Randomized, Double-blind, Placebo-controlled Study of the Safety and Tolerability of MEDI-551 in Scleroderma Completed NCT00946699 Phase 1
41 Safety & Suitability of Dabigatran to Inhibit Thrombin in Scleroderma Completed NCT02426229 Phase 1 dabigatran etexilate
42 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
43 Comparative Effectiveness Trial of Topical Calcipotriene, Clobetasol, and Tacrolimus in the Treatment of Pediatric Plaque Morphea Withdrawn NCT02680717 Phase 1 Clobetasol;Calcipotriene;Tacrolimus
44 Fractional Carbon Dioxide Laser Versus UVA1 Phototherapy for Treatment of Localized Scleroderma: A Clinical & Immunohistochemical Comparative Study Unknown status NCT02002897
45 The Effectiveness Of UVA1 Irradiation In The Treatment Of Skin Conditions With Altered Dermal Matrix: A Controlled, Cross-Over Study Completed NCT00476801
46 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 Completed NCT02222038
47 Something New Under the Sun: A Randomized, Double-Blinded, Controlled Trial of UVA1 Phototherapy in Morphea Completed NCT01799174
48 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
49 A Partially Nested RCT to Evaluate the Effectiveness of the Scleroderma Patient-centered Intervention Network COVID-19 Home-isolation Activities Together (SPIN-CHAT) Program to Reduce Anxiety Among At-Risk Scleroderma Patients Completed NCT04335279
50 Transcutaneous Electroacupuncture for Gastric Complications of Scleroderma Completed NCT03294616

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, Eye, Skeletal Muscle, Kidney, Endothelial

Publications for Localized Scleroderma

Articles related to Localized Scleroderma:

(show top 50) (show all 2002)
# Title Authors PMID Year
1
Autoantibodies against matrix metalloproteinase-1 in patients with localized scleroderma. 61 54
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. 54 61
17448296 2007
4
Suggested mechanisms of action of UVA phototherapy in morphea: a molecular study. 54 61
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. 54 61
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. 54 61
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. 54 61
7510487 1994
12
Expression of osteonectin, decorin, and transforming growth factor-beta 1 genes in fibroblasts cultured from patients with systemic sclerosis and morphea. 54 61
2023219 1991
13
Pediatric-onset solitary morphea profunda. 61
32955115 2021
14
Biomarker profiles of endothelial activation and dysfunction in rare systemic autoimmune diseases: implications for cardiovascular risk. 61
32810267 2021
15
Morphea following treatment with pembrolizumab for melanoma with metastatic lymph nodes: case report and review of literature. 61
33323722 2021
16
An Evaluation of the Performance of Current Morphea Subtype Classifications. 61
33595618 2021
17
Nasal and Oral Subunits Reconstruction of Localized Scleroderma Deformities. 61
33606439 2021
18
Morbidity and Disability in Juvenile Localized Scleroderma: The Case for Early Recognition and Systemic Immunosuppressive Treatment. 61
33548257 2021
19
Generalized morphea with extensive telangiectasia on the palms. 61
32902882 2021
20
Digital immunohistological dissection of immune privilege collapse in syringotropic autoimmune diseases: Implication for the pathogenesis. 61
33183905 2021
21
Ultrasonography, MRI and classic radiography of skin and MSK involvement in juvenile scleroderma. 61
33500800 2021
22
Aesthetic and therapeutic outcome of fat grafting for localized Scleroderma treatment: From basic study to clinical application. 61
33486881 2021
23
Morphea-like Carcinoma En Cuirasse Revealing a Bilateral Breast Cancer. 61
33359773 2021
24
Early morphea during treatment with ibrutinib in a patient with chronic lymphocytic leukemia. 61
32221651 2021
25
Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review. 61
33449302 2021
26
A case report on the use of topical cysteamine 5% cream in the management of refractory postinflammatory hyperpigmentation (PIH) resistant to triple combination cream (hydroquinone, topical corticosteroids, and retinoids). 61
32997864 2021
27
The Development and Initial Validation of PUMC Localized Scleroderma Facial Aesthetic Index: A Pilot Study. 61
33452541 2021
28
Histopathologic Spectrum of Morphea. 61
33337624 2021
29
3D automated quantification of epidermal thickness and dermal heterogeneity in high-definition optical coherence tomography: Illustrative utility in morphea. 61
33511640 2021
30
Cutaneous acral myofibroma with PDGFRB mutation in a patient with linear morphea en coup de sabre. 61
33495995 2021
31
Morphea in a Crohn's disease patient undergoing ustekinumab treatment. 61
33495147 2021
32
Histopathological Coexistence of Extragenital Lichen Sclerosus and Morphea in a Single Lesion. 61
33489622 2020
33
Dermoscopy in Monitoring and Predicting Therapeutic Response in General Dermatology (Non-Tumoral Dermatoses): An Up-To-Date Overview. 61
33030661 2020
34
Parry-Romberg syndrome: a mini review. 61
33348939 2020
35
Adult-onset generalized morphea associated with osteomyelitis. 61
32881032 2020
36
Masquerading presentation of morphea as hypopigmented patch mimicking indeterminate leprosy. 61
33259054 2020
37
Nivolumab-induced plaque morphea in a malign melanoma patient. 61
33355973 2020
38
Characteristics of Japanese patients with eosinophilic fasciitis: A brief multicenter study. 61
32860239 2020
39
Nodular morphea keloidal type: A rare case with paradigmatic histopathology significantly accompanied by a flawless surgical scar. 61
33258515 2020
40
Unilateral Linear Induration of the Skin: A Case Report of an Unusual Presentation of Scleredema. 61
33488921 2020
41
Feasibility of Using Elastography Ultrasound in Pediatric Localized Scleroderma (Morphea). 61
32951932 2020
42
The importance of development standards for anchoring vignettes: an illustrative example from pediatric localized scleroderma quality of life. 61
32654054 2020
43
Preliminary Evidence on Abatacept Safety and Efficacy in Refractory Juvenile Localized Scleroderma. 61
33369667 2020
44
Biological effects of a new ultraviolet A1 prototype based on light-emitting diodes on the treatment of localized scleroderma. 61
32592187 2020
45
The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review. 61
32876907 2020
46
Linear Scleroderma and Primary Sclerosing Cholangitis: An Undescribed Association. 61
33487709 2020
47
Dermatologic sequelae of breast cancer: From disease, surgery, and radiation. 61
33226140 2020
48
Clinical characteristics and histopathological changes of morphea: A single-center, retrospective study of 137 patients. 61
33188871 2020
49
Morphea patients with mucocutaneous involvement: A cross sectional study from the Morphea in Adults and Children (MAC) cohort. 61
33249064 2020
50
Mycophenolate mofetil treatment of an H syndrome patient with a SLC29A3 mutation. 61
33029882 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 77)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
14.08 VCAM1 TNFSF13B TNF TGFB1 MMP1 IL6
2
Show member pathways
13.87 TNFSF13B TNF TGFB1 SMAD3 IL6 IL4
3
Show member pathways
13.76 TNFSF13B TNF TGFB1 SMAD3 IL6 IL4
4
Show member pathways
13.58 TNFSF13B TNF TGFB1 SMAD7 SMAD3 IL6
5
Show member pathways
13.46 TNFSF13B TNF TGFB1 IL6 IL4 IL2
6
Show member pathways
13.43 VCAM1 TNFSF13B TNF TGFB1 MMP1 IL6
7
Show member pathways
13.25 TNFSF13B TNF TGFB1 SMAD3 IL6 IL4
8
Show member pathways
13.13 VCAM1 TGFB1 IL6 IL4 IL2 CCN2
9
Show member pathways
13.12 TGFB1 IL6 IL2 COL1A1 CCN2 BMP6
10
Show member pathways
13.1 TNF TGFB1 SMAD3 IL6 IL2 CXCL10
11 12.94 TGFB1 SMAD3 MMP1 IL6 IL4 IL2
12
Show member pathways
12.94 TNFSF13B TNF TGFB1 IL6 IL4 IL2
13
Show member pathways
12.8 TNFSF13B TNF TGFB1 IL6 IL2 CCN2
14
Show member pathways
12.79 VCAM1 TGFB1 MMP1 DCN COL1A1
15
Show member pathways
12.69 TNF TGFB1 SMAD3 IL6 IL4 IL2
16
Show member pathways
12.66 TNF TGFB1 IL6 IL4 IL2
17 12.66 VCAM1 TNFSF13B TNF IL4 IL2 CXCL10
18
Show member pathways
12.45 IL6 IL4 IL2 IL13
19
Show member pathways
12.44 TGFB1 IL6 IL2 CCN2 BMP6
20
Show member pathways
12.44 TNF MMP1 IL6 IL4 IL13 CXCL10
21 12.43 TNF TGFB1 SMAD3 IL6 IL2
22
Show member pathways
12.42 TNF IL6 IL2 CXCL10 CCL18
23
Show member pathways
12.38 TNF IL6 IL4 IL2
24 12.35 TNF TGFB1 DCN COL1A1
25
Show member pathways
12.34 TNF TGFB1 IL6 IL4 IL2 IL13
26 12.3 TNF TGFB1 SMAD3 MMP1 IL6
27 12.29 TGFB1 SMAD3 CCN2 BMP6
28 12.27 TGFB1 SMAD7 SMAD3 CCN2 BMP6
29
Show member pathways
12.25 TNF IL6 IL4 CXCL10
30 12.14 TGFB1 SMAD7 SMAD3 MMP1
31 12.14 TNF TGFB1 SMAD3 IL6
32 12.11 TNF IL6 IL4 IL2 CD34
33 12.09 VCAM1 TNF SELE IL6 CXCL10
34 12.03 TGFB1 SMAD3 IL6 COL1A1
35 12.01 TNF TGFB1 IL6 COL1A1
36 11.99 TNF IL6 IL4 CD34
37 11.99 TNFSF13B TNF TGFB1 IL6 IL4 IL2
38 11.98 TNFSF13B TNF TGFB1 MMP1 IL6
39 11.97 VCAM1 TGFB1 IL6 DCN CD34 BMP6
40
Show member pathways
11.96 TGFB1 SMAD3 IL6 IL4
41 11.94 TNF TGFB1 SMAD7 SMAD3 DCN BMP6
42 11.92 VCAM1 TNF TGFB1 MMP1 IL6 IL4
43 11.91 TNF IL4 IL2 IL13 CD34
44
Show member pathways
11.87 TNF TGFB1 IL6 IL2
45 11.87 SELE MMP1 IL6 IL4 IL2 IL13
46 11.86 TNF TGFB1 IL6 IL2 CD34
47
Show member pathways
11.81 TNF IL4 IL2
48 11.81 TGFB1 IL6 IL2 CCN2 BMP6
49 11.8 VCAM1 TNF TGFB1 SMAD3 SELE IL6
50 11.78 TGFB1 SMAD7 SMAD3

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 9.83 TNFSF13B TNF TGFB1 MMP1 IL6 IL4
2 external side of plasma membrane GO:0009897 9.65 VCAM1 TNF IL13 CXCL10 CD34
3 extracellular matrix GO:0031012 9.55 TGFB1 MMP1 DCN COL1A1 CCN2
4 extracellular space GO:0005615 9.5 VCAM1 TNFSF13B TNF TGFB1 SELE IL6
5 heteromeric SMAD protein complex GO:0071144 9.32 SMAD7 SMAD3

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

(show top 50) (show all 64)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II GO:0000122 10.2 TNF TGFB1 SMAD7 SMAD3 SKOR1 IL4
2 positive regulation of transcription, DNA-templated GO:0045893 10.18 TNF TGFB1 SMAD3 IL6 IL4 COL1A1
3 positive regulation of cell proliferation GO:0008284 10.1 TGFB1 IL6 IL4 IL2 CXCL10 CCN2
4 positive regulation of transcription by RNA polymerase II GO:0045944 10.1 TNF TGFB1 SMAD7 SMAD3 IL6 IL4
5 extracellular matrix organization GO:0030198 10.04 VCAM1 TNF MMP1 DCN COL1A1
6 positive regulation of cell migration GO:0030335 10.04 TGFB1 SMAD3 IL4 CXCL10 COL1A1
7 positive regulation of ERK1 and ERK2 cascade GO:0070374 10.03 TNF TGFB1 CCN2 CCL18
8 positive regulation of protein phosphorylation GO:0001934 10.02 TNF TGFB1 IL2 CCN2
9 aging GO:0007568 10 VCAM1 TGFB1 DCN CCN2
10 response to lipopolysaccharide GO:0032496 9.98 VCAM1 SELE IL13 DCN CXCL10
11 negative regulation of inflammatory response GO:0050728 9.96 SMAD3 IL4 IL2 IL13
12 wound healing GO:0042060 9.93 TGFB1 SMAD3 DCN COL1A1
13 regulation of immune response GO:0050776 9.93 VCAM1 TNFSF13B SMAD3 IL4 COL1A1 CD34
14 BMP signaling pathway GO:0030509 9.92 TGFB1 SMAD7 SMAD3 BMP6
15 leukocyte migration GO:0050900 9.91 TNF TGFB1 SELE MMP1 COL1A1 CD34
16 response to glucocorticoid GO:0051384 9.9 TNF IL6 BMP6
17 cellular response to mechanical stimulus GO:0071260 9.9 TGFB1 IL13 COL1A1 BMP6
18 response to mechanical stimulus GO:0009612 9.89 IL13 DCN COL1A1
19 positive regulation of smooth muscle cell proliferation GO:0048661 9.89 TNF IL6 IL13
20 positive regulation of interleukin-1 beta production GO:0032731 9.88 TNF SMAD3 IL6
21 positive regulation of protein complex assembly GO:0031334 9.88 TNF TGFB1 MMP1
22 SMAD protein signal transduction GO:0060395 9.88 TGFB1 SMAD7 SMAD3 BMP6
23 positive regulation of protein secretion GO:0050714 9.87 TGFB1 IL13 BMP6
24 cellular response to transforming growth factor beta stimulus GO:0071560 9.87 TGFB1 SMAD7 SMAD3 COL1A1
25 T cell activation GO:0042110 9.86 TGFB1 SMAD3 IL4
26 extrinsic apoptotic signaling pathway GO:0097191 9.86 TNF TGFB1 SMAD3
27 inflammatory response GO:0006954 9.86 TNF TGFB1 SELE IL6 IL13 CXCL10
28 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 9.85 TNF TGFB1 SMAD3
29 ureteric bud development GO:0001657 9.85 TGFB1 SMAD7 SMAD3
30 negative regulation of fat cell differentiation GO:0045599 9.85 TNF TGFB1 SMAD3 IL6
31 positive regulation of bone mineralization GO:0030501 9.84 TGFB1 SMAD3 BMP6
32 negative regulation of mitotic cell cycle GO:0045930 9.84 TNF TGFB1 SMAD3
33 positive regulation of epithelial to mesenchymal transition GO:0010718 9.84 TGFB1 SMAD3 IL6 COL1A1
34 microglial cell activation GO:0001774 9.82 TNF IL4 IL13
35 positive regulation of B cell proliferation GO:0030890 9.81 TNFSF13B IL4 IL2 IL13
36 positive regulation of gene expression GO:0010628 9.81 TNF TGFB1 SMAD3 IL6 IL4 IL13
37 regulation of transforming growth factor beta receptor signaling pathway GO:0017015 9.8 TGFB1 SMAD7 SMAD3
38 negative regulation of myoblast differentiation GO:0045662 9.8 TNF TGFB1 CXCL10
39 positive regulation of interleukin-10 production GO:0032733 9.8 IL6 IL4 IL13 CD34
40 leukocyte tethering or rolling GO:0050901 9.79 VCAM1 TNF SELE
41 positive regulation of immunoglobulin production GO:0002639 9.78 IL6 IL2 IL13
42 positive regulation of interleukin-17 production GO:0032740 9.77 TGFB1 IL6 IL2
43 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.77 TNF IL6 IL4 IL2 IL13
44 negative regulation of production of miRNAs involved in gene silencing by miRNA GO:1903799 9.73 TNF TGFB1
45 positive regulation of vascular permeability GO:0043117 9.73 TGFB1 BMP6
46 positive regulation of isotype switching to IgG isotypes GO:0048304 9.73 IL4 IL2
47 positive regulation of mast cell degranulation GO:0043306 9.73 IL4 IL13
48 negative regulation of lipid storage GO:0010888 9.73 TNF IL6
49 response to laminar fluid shear stress GO:0034616 9.72 TGFB1 SMAD7
50 positive regulation of T cell proliferation GO:0042102 9.72 VCAM1 TNFSF13B IL6 IL4 IL2

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 9.43 TGFB1 IL6 IL4 IL2 CCN2 BMP6
2 collagen binding GO:0005518 9.33 SMAD7 SMAD3 DCN
3 cytokine activity GO:0005125 9.32 TNFSF13B TNF TGFB1 IL6 IL4 IL2
4 type I transforming growth factor beta receptor binding GO:0034713 9.26 TGFB1 SMAD7

Sources for Localized Scleroderma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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