LSA
MCID: LCH016
MIFTS: 41

Lichen Sclerosus Et Atrophicus (LSA)

Categories: Rare diseases, Reproductive diseases, Skin diseases

Aliases & Classifications for Lichen Sclerosus Et Atrophicus

MalaCards integrated aliases for Lichen Sclerosus Et Atrophicus:

Name: Lichen Sclerosus Et Atrophicus 57 73 20 70 32
Lichen Sclerosus 20 54
Lsa 57 73
Lichen Sclerosis Et Atrophicus 20
Lichen Sclerosis 20

Characteristics:

OMIM®:

57 (Updated 05-Apr-2021)
Inheritance:
autosomal dominant


HPO:

31
lichen sclerosus et atrophicus:
Inheritance autosomal dominant inheritance


Classifications:



External Ids:

OMIM® 57 151590
ICD10 32 L90.0
MedGen 41 C0023652
UMLS 70 C0023652

Summaries for Lichen Sclerosus Et Atrophicus

GARD : 20 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 Pimecrolimus and Tacrolimus have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and thyroid, and related phenotypes are squamous cell carcinoma and carcinoma

Wikipedia : 73 Lichen sclerosus (LS) is a chronic, inflammatory skin disease of unknown cause which can affect any body... 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 295)
# Related Disease Score Top Affiliating Genes
1 phimosis 31.0 ELN ECM1
2 balanitis xerotica obliterans 31.0 ELN ECM1
3 celiac disease 1 30.3 TIA1 GZMB CD8A
4 skin disease 30.2 TIA1 ELN ECM1 CD8A
5 panniculitis 30.0 TIA1 GZMB CD8A
6 localized scleroderma 11.0
7 systemic scleroderma 10.9
8 lichen planus 10.8
9 balanitis 10.8
10 vulva squamous cell carcinoma 10.7
11 leukoplakia of penis 10.7
12 urethral stricture 10.7
13 keratosis 10.6
14 leukoplakia 10.6
15 acrodermatitis 10.6
16 penile cancer 10.6
17 malignant tumor of penis 10.5
18 vitiligo-associated multiple autoimmune disease susceptibility 6 10.5
19 vitiligo-associated multiple autoimmune disease susceptibility 1 10.5
20 graft-versus-host disease 10.5
21 acrodermatitis chronica atrophicans 10.5
22 primary biliary cholangitis 10.5
23 rapidly involuting congenital hemangioma 10.5
24 systemic lupus erythematosus 10.5
25 mycosis fungoides 10.5
26 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.5
27 balanoposthitis 10.5
28 connective tissue disease 10.5
29 lupus erythematosus 10.5
30 chronic graft versus host disease 10.5
31 vulva cancer 10.4
32 melanoma 10.4
33 basal cell carcinoma 10.4
34 dermatitis 10.4
35 skin atrophy 10.4
36 contact dermatitis 10.4
37 allergic contact dermatitis 10.4
38 granuloma annulare 10.4
39 alopecia 10.4
40 posttransplant acute limbic encephalitis 10.4
41 discoid lupus erythematosus 10.4
42 scleroderma, familial progressive 10.3
43 thyroiditis 10.3
44 in situ carcinoma 10.3
45 ige responsiveness, atopic 10.3
46 urinary tract infection 10.3
47 vulvar dystrophy 10.3
48 hypothyroidism 10.3
49 vasculitis 10.3
50 hashimoto thyroiditis 10.2

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®:

57 (Updated 05-Apr-2021)
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 (Updated 05-Apr-2021)

Drugs & Therapeutics for Lichen Sclerosus Et Atrophicus

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

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

Interventional clinical trials:

(show all 25)
# 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, 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
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 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
5 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%
6 Discovery and Validation of Biomarkers of Lichen Sclerosus Unknown status NCT03561428
7 Efficacy of Nd:Yttrium Aluminum Garnet Laser Treatment of Lichen Sclerosus Unknown status NCT03525522 Topical corticosteroid Diprosone
8 A Double Blind Placebo Controlled Trial of Autologous Platelet Rich Plasma (PRP) Intradermal Injections for the Treatment of Vulvar Lichen Sclerosus Completed NCT03045172 Placebo
9 Autologous Platelet Rich Plasma Intradermal Injections for Vulvar Lichen Sclerosus Completed NCT02794363
10 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 Completed NCT03665584
11 The Comfort and Adherence of Rivelin® Plain Patches in Vulva Lichen Sclerosus Patients Completed NCT03686800
12 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
13 Clinical Study of the CO2RE® Laser Device for Treatment of Vulvar Lichen Sclerosus Completed NCT04148651
14 Vulvar Lichen Sclerosus: Therapeutic Comparison Between Clobetasol Propionate, Photodynamic Therapy and Low-Intensity Laser Completed NCT02416531 Clobetasol propionate
15 Fractional / Pixel CO2 Laser Treatment of Vulvar Atrophy and Lichen Sclerosus Completed NCT03063684
16 Lichen Sclerosus - the Influence on Quality of Life Completed NCT03419377
17 Diagnostic Accuracy of "Three Rings Vulvoscopy" for Detection of Vulvar Dermatosis Completed NCT02732145
18 Effect of Treatment With the Ablative Fractional Carbodioxide Laser in Vulvar Lichen Sclerosis Recruiting NCT04107454
19 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
20 Effectiveness of Ablative Fractional 2940 nm Laser Treatment for Vulvar Lichen Sclerosus Recruiting NCT04134494
21 Laser Therapy in Women With Lichenoid Disorders: A Randomized Controlled Trial Recruiting NCT04697563
22 Safety and Efficacy of Er:YAG and Nd:YAG Laser Therapy in Gynecology: A Retrospective Case Series. Active, not recruiting NCT04073082
23 Evaluation of the Role of Vibrational Spectroscopy in the Assessment of Vulval Disease Suspended NCT02223975
24 Development of a Vulvar Scarring Grading Scale for Lichen Sclerosus Terminated NCT02880904
25 Open Ended Chart Review of Adult Women Presenting to the Vulvar Mucosal Specialty Clinic at Northwestern University Department of Dermatology Terminated NCT02881229

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, Thyroid, T Cells, Bone, Bone Marrow, Cervix

Publications for Lichen Sclerosus Et Atrophicus

Articles related to Lichen Sclerosus Et Atrophicus:

(show top 50) (show all 2165)
# Title Authors PMID Year
1
Autoantibodies to extracellular matrix protein 1 in lichen sclerosus. 61 54 57
12867112 2003
2
Interleukin 1 receptor antagonist gene polymorphism association with lichen sclerosus. 57 61
7927338 1994
3
Familial occurrence of lichen sclerosus et atrophicus. Case reports of a mother and daughter. 61 57
3827280 1987
4
Genetic aspects of vulvar lichen sclerosus. 61 57
6591797 1984
5
Downregulated CD44 and hyaluronan expression in vulvar intraepithelial neoplasia and squamous cell carcinomas. 54 61
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. 61 54
15927815 2005
9
Restored epidermal CD44 expression in lichen sclerosus et atrophicus and clinical improvement with topical application of retinaldehyde. 54 61
15787834 2005
10
Characterization of IgG autoantibodies to extracellular matrix protein 1 in lichen sclerosus. 61 54
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. 54 61
15068453 2004
13
The role of extracellular matrix protein 1 in human skin. 54 61
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. 61 54
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
Quality of Life in Vulvar Lichen Sclerosus Patients Treated With Long-Term Topical Corticosteroids. 61
33746196 2021
18
Characteristics Among Premenarchal Girls With Lichen Sclerosus. 61
33284147 2021
19
Sexual function is adversely affected in the majority of men presenting with penile lichen sclerosus. 61
33713357 2021
20
Successful response of vulvar lichen sclerosus with NB-UVB. 61
33486876 2021
21
Clinical and Dermoscopic Assessment of Vulvar Lichen Sclerosus After 5-Aminolevulinic Acid Photodynamic Therapy:A Prospective Study. 61
33246149 2021
22
Outcome of perineoplasty and de-adhesion in patients with vulvar Lichen sclerosus and sexual disorders. 61
33412460 2021
23
Proposition of a severity scale for lichen sclerosus: The "Clinical Lichen Sclerosus Score". 61
33426761 2021
24
Immunohistological analysis of CD34- positive dermal dendritic cells and microvessel density in the genital and extragenital lichen sclerosus. 61
33727063 2021
25
Lasers as an adjuvant for vulvar lichen sclerosus: a systematic review and meta-analysis. 61
33684499 2021
26
[Vulvar intraepithelial neoplasias]. 61
33744030 2021
27
[Paediatric vulval clinic]. 61
33591405 2021
28
Long term outcomes of one-stage augmentation anterior urethroplasty: a systematic review and meta-analysis. 61
32459452 2021
29
Lichen sclerosis-induced long segment anterior urethral stricture: The early outcome of one-stage repair using dorsolateral onlay buccal mucosa graft. 61
33764232 2021
30
Treatment Options in Vulvar Lichen Sclerosus: A Scoping Review. 61
33786234 2021
31
Conversations between women with vulval lichen sclerosus: a thematic analysis of online forums. 61
33596903 2021
32
Balanopreputial sac and urine microbiota in patients with male genital lichen sclerosus. 61
33084022 2021
33
Synchronous genitourinary lichen sclerosus signals a distinct urinary microbiome profile in men with urethral stricture disease. 61
32274566 2021
34
Acitretin therapy for vulvar lichen sclerosus complicated by recurrent squamous cell carcinoma. 61
33506084 2021
35
Frontal fibrosing alopecia and genital Lichen sclerosus: Single-center experience. 61
32590887 2021
36
Lichen Sclerosus and Phimosis - Discrepancies Between Clinical and Pathological Diagnosis and Its Consequences. 61
33248142 2021
37
Lichen Sclerosus on the Eyelids. 61
32986974 2021
38
Lichen sclerosus successfully treated with baricitinib plus psoralen and ultraviolet A. 61
33599052 2021
39
Lichen sclerosus and hidradenitis suppurativa: Two case reports of a new possible association. 61
32815154 2021
40
A case of acral lichen sclerosus et atrophicus. 61
33490341 2021
41
Flag-like hypermelanosis with superimposed lichen sclerosus et atrophicus: an unusual Koebner phenomenon? 61
32805750 2021
42
How Can We Treat Vulvar Carcinoma in Pregnancy? A Systematic Review of the Literature. 61
33671249 2021
43
Comparison of p53 immunohistochemical staining in differentiated vulvar intraepithelial neoplasia (dVIN) with that in inflammatory dermatoses and benign squamous lesions in the vulva. 61
32799363 2021
44
Adult Acquired Buried Penis: A Hidden Problem in Obese Men. 61
33680609 2021
45
Treatment of Vulvar Lichen Sclerosus et Atrophicus With Fractional Carbon Dioxide Laser Therapy: A Report of 4 Cases. 61
32679117 2021
46
Dermoscopy as a Tool for Assessing the Therapeutic Response in a Case of Extra-Genital Lichen Sclerosus et Atrophicus. 61
33768045 2021
47
Sunlight-induced unique finding of similar reaction to lichen sclerosus et atrophicus developing in scarring alopecia of systemic lupus erythematosus. 61
33035366 2021
48
The short-term efficacy and safety of fractional CO2 laser therapy for vulvovaginal symptoms in menopause, breast cancer, and lichen sclerosus. 61
33399322 2021
49
Increased prevalence of breast cancer in female patients with lichen sclerosus. 61
32325110 2021
50
Microablative fractional radiofrequency as a therapeutic option for vulvar lichen sclerosus: a pilot study. 61
33787656 2021

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
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-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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