LSA
MCID: LCH016
MIFTS: 41

Lichen Sclerosus Et Atrophicus (LSA)

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Lichen Sclerosus Et Atrophicus

MalaCards integrated aliases for Lichen Sclerosus Et Atrophicus:

Name: Lichen Sclerosus Et Atrophicus 56 74 52 71 32
Lichen Sclerosus 52 54
Lsa 56 74
Lichen Sclerosis Et Atrophicus 52
Lichen Sclerosis 52

Characteristics:

OMIM:

56
Inheritance:
autosomal dominant


HPO:

31
lichen sclerosus et atrophicus:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM 56 151590
ICD10 32 L90.0
MedGen 41 C0023652
UMLS 71 C0023652

Summaries for Lichen Sclerosus Et Atrophicus

NIH Rare Diseases : 52 Lichen sclerosus is a chronic skin disorder that is more common in women, most often affecting the external part of the vagina (vulva) or the area around the anus. In men, it typically affects the tip of the penis. It can occur at any age but is usually seen in women over age 50. Some people have no symptoms, while others may experience itchiness (sometimes severe), discomfort, or blistering. It often lasts for years and can cause permanent scarring. The underlying cause of lichen sclerosus is not fully understood but it is thought to relate to an autoimmune process. Treatment may include topical steroids or other types of topical creams and/or surgery.

MalaCards based summary : Lichen Sclerosus Et Atrophicus, also known as lichen sclerosus, is related to phimosis and balanitis xerotica obliterans. An important gene associated with Lichen Sclerosus Et Atrophicus is ECM1 (Extracellular Matrix Protein 1), and among its related pathways/superpathways are NF-kappaB Signaling and Downstream signaling in naive CD8+ T cells. The drugs Clobetasol and Hormones have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and t cells, and related phenotypes are squamous cell carcinoma and carcinoma

Wikipedia : 74 Lichen sclerosus (LS) is a chronic, inflammatory skin disease of unknown cause, commonly appearing as... more...

More information from OMIM: 151590

Related Diseases for Lichen Sclerosus Et Atrophicus

Diseases related to Lichen Sclerosus Et Atrophicus via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 297)
# Related Disease Score Top Affiliating Genes
1 phimosis 30.9 ELN ECM1
2 balanitis xerotica obliterans 30.9 ELN ECM1
3 celiac disease 1 30.3 TIA1 GZMB CD8A
4 panniculitis 30.0 TIA1 GZMB CD8A
5 skin disease 29.8 TIA1 ELN ECM1 CD8A
6 localized scleroderma 11.1
7 systemic scleroderma 11.0
8 lichen planus 10.8
9 squamous cell carcinoma 10.8
10 balanitis 10.8
11 vulva squamous cell carcinoma 10.8
12 leukoplakia of penis 10.8
13 urethral stricture 10.7
14 keratosis 10.6
15 acrodermatitis 10.6
16 leukoplakia 10.6
17 penile cancer 10.6
18 vitiligo-associated multiple autoimmune disease susceptibility 6 10.6
19 vitiligo-associated multiple autoimmune disease susceptibility 1 10.6
20 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.6
21 graft-versus-host disease 10.6
22 acrodermatitis chronica atrophicans 10.6
23 primary biliary cirrhosis 10.6
24 liver cirrhosis 10.6
25 lupus erythematosus 10.6
26 malignant tumor of penis 10.6
27 sexual disorder 10.6
28 mycosis fungoides 10.5
29 balanoposthitis 10.5
30 connective tissue disease 10.5
31 chronic graft versus host disease 10.5
32 rapidly involuting congenital hemangioma 10.5
33 autoimmune disease 10.5
34 systemic lupus erythematosus 10.5
35 vulva cancer 10.5
36 basal cell carcinoma 10.5
37 dermatitis 10.5
38 skin atrophy 10.5
39 contact dermatitis 10.5
40 allergic contact dermatitis 10.5
41 granuloma annulare 10.5
42 47,xyy 10.5
43 posttransplant acute limbic encephalitis 10.5
44 discoid lupus erythematosus 10.5
45 scleroderma, familial progressive 10.4
46 thyroiditis 10.4
47 in situ carcinoma 10.4
48 ige responsiveness, atopic 10.4
49 vulvar dystrophy 10.4
50 pustulosis of palm and sole 10.4

Graphical network of the top 20 diseases related to Lichen Sclerosus Et Atrophicus:



Diseases related to Lichen Sclerosus Et Atrophicus

Symptoms & Phenotypes for Lichen Sclerosus Et Atrophicus

Human phenotypes related to Lichen Sclerosus Et Atrophicus:

31
# Description HPO Frequency HPO Source Accession
1 squamous cell carcinoma 31 HP:0002860
2 carcinoma 31 HP:0030731

Symptoms via clinical synopsis from OMIM:

56
Skin:
lichen sclerosus et atrophicus
ivory-colored, polygonal, flat-topped lichenoid papules
papules may coalesce into atrophic plaques

Oncology:
vulvar lsa may predispose to squamous cell carcinoma

Clinical features from OMIM:

151590

Drugs & Therapeutics for Lichen Sclerosus Et Atrophicus

Drugs for Lichen Sclerosus Et Atrophicus (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 37)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Clobetasol Approved, Experimental, Investigational Phase 3 25122-41-2, 25122-46-7 32798 5311051
2 Hormones Phase 3
3 glucocorticoids Phase 3
4 Hormone Antagonists Phase 3
5 Anti-Inflammatory Agents Phase 3
6
Tacrolimus Approved, Investigational Phase 2 104987-11-3 445643 439492 6473866
7
Pimecrolimus Approved, Investigational Phase 2 137071-32-0 17753757 6447131
8
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
9
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
10
Progesterone Approved, Vet_approved Phase 2 57-83-0 5994
11 Immunosuppressive Agents Phase 2
12 Immunologic Factors Phase 2
13 Calcineurin Inhibitors Phase 2
14 Dermatologic Agents Phase 2
15 Analgesics, Non-Narcotic Phase 2
16 Analgesics Phase 2
17 Antirheumatic Agents Phase 2
18 Anti-Inflammatory Agents, Non-Steroidal Phase 2
19 Hydrocortisone 17-butyrate 21-propionate Phase 2
20 Hydrocortisone-17-butyrate Phase 2
21 Hydrocortisone hemisuccinate Phase 2
22 Progestins Phase 2
23
Methylene blue Approved, Investigational Phase 1 61-73-4
24
Ropivacaine Approved Phase 1 84057-95-4 71273 175805
25
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 1 1177-87-3
26
Dexamethasone Approved, Investigational, Vet_approved Phase 1 50-02-2 5743
27
Betamethasone Approved, Vet_approved 378-44-9 9782
28
Petrolatum Approved, Investigational 8009-03-8
29
Cortisone Experimental 53-06-5 222786
30 Respiratory System Agents
31 Anti-Asthmatic Agents
32 Betamethasone Valerate 2152-44-5
33 Betamethasone-17,21-dipropionate
34 Betamethasone sodium phosphate
35 Betamethasone benzoate
36 Acidophilus
37 Estrogens

Interventional clinical trials:

(show all 29)
# Name Status NCT ID Phase Drugs
1 A Randomized Trial of Clobetasol Proprionate Versus Fractionated CO2 Laser for the Treatment of Lichen Sclerosus (CuRLS) Completed NCT02573883 Phase 3 Clobetasol Propionate 0.05% ointment
2 A Double Blind Phase II Study Comparing Safety and Efficacy of Tacrolimus Versus Topical Clobetasol Propionate in the Treatment of Vulvar Lichen Sclerosus. Completed NCT00757874 Phase 2 Tacrolimus cream;Clobetasol cream
3 Pilot Study of Vulvar Lichen Sclerosus (VLS) Treatment Using Adipose Tissue Associated With Autologous Platelet-rich Plasma (PRP). Completed NCT03961126 Phase 2 Injection of autologous fatty tissue associated with autologous platelet-rich plasma.;Corticosteroids (clobetasol 0.05%)
4 A Double-Blind, Parallel-group Trial of Topical Pimecrolimus Cream 1% (Elidel®) Versus Clobetasol 0.05% Cream for the Treatment of Vulvar Lichen Sclerosus Completed NCT00393263 Phase 2 pimecrolimus and clobetasol;clobetasol 0.05% cream
5 Evaluation of the Antipruritic Effect of Elidel (Pimecrolimus) in Non-atopic Pruritic Disease Completed NCT00507832 Phase 2 Pimecrolimus;Hydrocortisone;Pimecrolimus
6 Efficacy of a Topic Therapy With Progesterone Compared to the Conventional Therapy With Clobetasol Propionate in Patients With Vulvar Lichen Sclerosus. A Double Blind, Randomized Phase II Pilot Study. Terminated NCT01126255 Phase 2 Clobetasol propionate 0.05%;Progesterone 8%
7 Evaluation of the Efficacy of Mixed Methylene Blue Compound Injection for the Treatment of Nonneoplastic Epithelial Disorders of Vulva. Unknown status NCT03200808 Phase 1 mixed methylene blue compound injection
8 Efficacy of Nd:Yttrium Aluminum Garnet Laser Treatment of Lichen Sclerosus Unknown status NCT03525522 Topical corticosteroid Diprosone
9 Discovery and Validation of Biomarkers of Lichen Sclerosus Unknown status NCT03561428
10 Interest of the Confocal Microscope in the Diagnosis of Epidermoid Carcinoma of the Vulva and Their Precursors Unknown status NCT02735109
11 Lichen Sclerosus - the Influence on Quality of Life Completed NCT03419377
12 Autologous Platelet Rich Plasma Intradermal Injections for Vulvar Lichen Sclerosus Completed NCT02794363
13 The Comfort and Adherence of Rivelin® Plain Patches in Vulva Lichen Sclerosus Patients Completed NCT03686800
14 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
15 A Double Blind Placebo Controlled Trial of Autologous Platelet Rich Plasma (PRP) Intradermal Injections for the Treatment of Vulvar Lichen Sclerosus Completed NCT03045172 Placebo
16 Vulvar Lichen Sclerosus: Therapeutic Comparison Between Clobetasol Propionate, Photodynamic Therapy and Low-Intensity Laser Completed NCT02416531 Clobetasol propionate
17 Diagnostic Accuracy of "Three Rings Vulvoscopy" for Detection of Vulvar Dermatosis Completed NCT02732145
18 Fractional / Pixel CO2 Laser Treatment of Vulvar Atrophy and Lichen Sclerosus Completed NCT03063684
19 Clinical Study of the CO2RE® Laser Device for Treatment of Vulvar Lichen Sclerosus Recruiting NCT04148651
20 Effect of Treatment With the Ablative Fractional Carbodioxide Laser in Vulvar Lichen Sclerosis Recruiting NCT04107454
21 Prospective, Randomized, Active-controlled Investigator Initiated Clinical Trial to Demonstrate That the Nd:YAG/Er:YAG Dual Laser Therapy is Effective to Treat Vulvar Lichen Sclerosus and Similar to Standard Treatment With Steroid Cream Recruiting NCT03926299 Clobetasol propionate 0.05% ointment
22 Effectiveness of Ablative Fractional 2940 nm Laser Treatment for Vulvar Lichen Sclerosus Recruiting NCT04134494
23 Safety and Efficacy of Er:YAG and Nd:YAG Laser Therapy in Gynecology: A Retrospective Case Series. Active, not recruiting NCT04073082
24 Development of a Vulvar Scarring Grading Scale for Lichen Sclerosus Active, not recruiting NCT02880904
25 Open Ended Chart Review of Adult Women Presenting to the Vulvar Mucosal Specialty Clinic at Northwestern University Department of Dermatology Active, not recruiting NCT02881229
26 A Double Blinded Sham Controlled Trial of Fractional CO2 Laser Treatment Using the DEKA SmartXide Touch Laser System (MonaLisa Touch) for the Treatment of Vulvar Lichen Sclerosus Active, not recruiting NCT03665584
27 Implication of Human Papillomavirus (HPV) in Lichen Physiopathology in Human Active, not recruiting NCT02761122
28 Is Low Level Laser Therapy (LLLT) Effective in Reducing Pain Experienced by Women With Provoked Vestibulodynia? Not yet recruiting NCT04234542
29 Evaluation of the Role of Vibrational Spectroscopy in the Assessment of Vulval Disease Suspended NCT02223975

Search NIH Clinical Center for Lichen Sclerosus Et Atrophicus

Genetic Tests for Lichen Sclerosus Et Atrophicus

Anatomical Context for Lichen Sclerosus Et Atrophicus

MalaCards organs/tissues related to Lichen Sclerosus Et Atrophicus:

40
Skin, Breast, T Cells, Thyroid, Testes, Bone, Cervix

Publications for Lichen Sclerosus Et Atrophicus

Articles related to Lichen Sclerosus Et Atrophicus:

(show top 50) (show all 2035)
# Title Authors PMID Year
1
Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. 56 54 61
12867112 2003
2
Interleukin 1 receptor antagonist gene polymorphism association with lichen sclerosus. 56 61
7927338 1994
3
Familial occurrence of lichen sclerosus et atrophicus. Case reports of a mother and daughter. 56 61
3827280 1987
4
Genetic aspects of vulvar lichen sclerosus. 61 56
6591797 1984
5
Downregulated CD44 and hyaluronan expression in vulvar intraepithelial neoplasia and squamous cell carcinomas. 61 54
19900133 2010
6
A case of lichen sclerosus of the scalp associated with autoantibodies to extracellular matrix protein 1. 61 54
20026864 2009
7
CD44 and hyaluronate expression in follicular mucinosis. 54 61
16466510 2006
8
Three-dimensional imaging reveals major changes in skin microvasculature in lipoid proteinosis and lichen sclerosus. 54 61
15927815 2005
9
Restored epidermal CD44 expression in lichen sclerosus et atrophicus and clinical improvement with topical application of retinaldehyde. 61 54
15787834 2005
10
Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. 54 61
15347336 2004
11
Development of antigen-specific ELISA for circulating autoantibodies to extracellular matrix protein 1 in lichen sclerosus. 54 61
15173881 2004
12
Translational benefits from research on rare genodermatoses. 61 54
15068453 2004
13
The role of extracellular matrix protein 1 in human skin. 61 54
14723723 2004
14
Alterations in fibrillin as well as collagens I and III and elastin occur in vulval lichen sclerosus. 61 54
11683283 2001
15
Decrease in epidermal CD44 expression as a potential mechanism for abnormal hyaluronate accumulation in superficial dermis in lichen sclerosus et atrophicus. 54 61
11121141 2000
16
Expression of retinoic acid receptors in non-neoplastic epithelial disorders of the vulva and normal vulvar skin. 61 54
10782404 2000
17
Fat Grafting Improves Fibrosis and Scarring in Vulvar Lichen Sclerosus: Results From a Prospective Cohort Study. 61
32205767 2020
18
Adult Vulvar Lichen Sclerosus: Can Experts Agree on the Assessment of Disease Severity? 61
32205764 2020
19
Anogenital High-Grade Squamous Intraepithelial Lesion Comorbid With Vulvar Lichen Sclerosus and Lichen Planus. 61
32324694 2020
20
Vulvar Lichen Sclerosus: Outcomes Important to Patients in Assessing Disease Severity. 61
32569254 2020
21
Extragenital lichen sclerosus successfully treated by topical maxacalcitol/betamethasone butyrate propionate. 61
32155665 2020
22
Clinicopathologic Diagnostic Criteria for Vulvar Lichen Planus. 61
32205763 2020
23
Proposition of standardized protocol for photodynamic therapy for vulvar lichen sclerosus. 61
32420789 2020
24
Vulvar basal cell carcinoma with vulvar lichen sclerosus: a rare association. 61
32545938 2020
25
An epidemiologic overview of a tertiary referral practice for male paediatric lichen sclerosus. 61
32549740 2020
26
Incidence of lichen sclerosus and subsequent causes of death: a nationwide Finnish register study. 61
32065721 2020
27
Acrosyringeal variant of extragenital lichen sclerosus et atrophicus. 61
32533734 2020
28
Risk calculator for prediction of treatment-related urethroplasty failure in patients with penile urethral strictures. 61
32002746 2020
29
Comparison of Serum Vitamin D Levels in Relation to Bowel and Bladder Symptoms in Women with Vulvar Diseases. 61
30747611 2020
30
Vulvar diseases: Conditions in adults and children. 61
31712170 2020
31
An arm-based network meta-analysis on treatments for vulvar lichen sclerosus and a call for development of core outcome sets. 61
31697910 2020
32
Restoration of the Lost Obscured Coronal Sulcus: A Novel Surgical Technique for Penile Lichen Sclerosus with Severe Preputial Adhesions. 61
32536486 2020
33
Scleredema of Buschke associated with lichen sclerosus: Three cases. 61
30289118 2020
34
Elastophagocytosis and interstitial granulomatous infiltrate are more common in extragenital vs genital lichen sclerosus. 61
32399972 2020
35
Novel therapeutic approaches and targets for treatment of vulvar lichen sclerosus. 61
32416670 2020
36
Outcome domains in Lichen Sclerosus. 61
32471015 2020
37
Lichen Sclerosus: More Than Meets the Eye. 61
32087208 2020
38
Preventing the long-term consequences of lichen sclerosus. 61
32376435 2020
39
Reply to Dr. Bunker's letter about lichen sclerosus. 61
32376436 2020
40
Association of Topical Corticosteroids With Reduced Vulvar Squamous Cell Carcinoma Recurrence in Patients With Vulvar Lichen Sclerosus. 61
32374363 2020
41
The Vulval Disease Quality of Life Index in women with vulval lichen sclerosus correlates with clinician and symptom scores. 61
31729009 2020
42
Surgical management and outcomes of adult acquired buried penis with and without lichen sclerosus: a comparative analysis. 61
32378139 2020
43
Clinical outcomes and adherence to topical corticosteroid therapy in women with vulvar lichen sclerosus: A retrospective cohort study. 61
32387654 2020
44
Vulvar intraepithelial neoplasia: Classification, epidemiology, diagnosis, and management. 61
32417455 2020
45
Balanitis Xerotica Obliterans Associated With Differentiated Penile Intraepithelial Neoplasia and "Bramble-Bush" Elastic Fibers Accumulation: A Finding Not Specific for Penicillamine Intake. 61
32472770 2020
46
A critical outcome analysis of Asopa single-stage dorsal inlay substitution urethroplasty for penile urethral stricture. 61
31321508 2020
47
Development and Validation of a Clinical Grading Scale to Assess the Vulvar Region: The Vulvar Architecture Severity Scale. 61
32382752 2020
48
Introital Obliteration as a Consequence of Lichen Sclerosus. 61
30772315 2020
49
Fractionated CO2 Laser as Therapy in Recalcitrant Lichen Sclerosus. 61
32068618 2020
50
Lichen sclerosus-associated nevus on glans penis mimicking melanoma. 61
32258310 2020

Variations for Lichen Sclerosus Et Atrophicus

Expression for Lichen Sclerosus Et Atrophicus

Search GEO for disease gene expression data for Lichen Sclerosus Et Atrophicus.

Pathways for Lichen Sclerosus Et Atrophicus

Pathways related to Lichen Sclerosus Et Atrophicus according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.85 GZMB CD8A CD44
2
Show member pathways
11.04 GZMB CD8A
3 11.03 GZMB CD8A CD44
4 10.39 ELN ECM1 CD44

GO Terms for Lichen Sclerosus Et Atrophicus

Biological processes related to Lichen Sclerosus Et Atrophicus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 T cell activation GO:0042110 8.62 CD8A CD44

Sources for Lichen Sclerosus Et Atrophicus

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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