LPP
MCID: LCH011
MIFTS: 50

Lichen Planopilaris (LPP)

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Lichen Planopilaris

MalaCards integrated aliases for Lichen Planopilaris:

Name: Lichen Planopilaris 53 59 55
Lichen Planus Follicularis 53 59 73
Follicular Lichen Planus 53 59
Lichen Follicularis 53 59
Lpp 53 59
Postmenopausal Frontal Fibrosing Alopecia 73
Lichen Planopilaris Classic Type 53
Frontal Fibrosing Alopecia 53
Kossard Disease 53

Characteristics:

Orphanet epidemiological data:

59
lichen planopilaris
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 59  
Rare skin diseases


Summaries for Lichen Planopilaris

NIH Rare Diseases : 53 Lichen planopilaris (LPP) is an inflammatory condition that causes patchy hair loss, mainly on the scalp. It is a form of lichen planus that most often affects women in young adulthood. There are 3 forms of LPP, distinguished by specific patterns and locations of symptoms: classic LPP, frontal fibrosing alopecia, and Lassueur Graham-Little Piccardi syndrome. Signs and symptoms may include patches of hair loss, scaling and redness around hair follicles, and pain, burning, tenderness, or itching on the scalp. Tiny, red pimples (papules) may appear around clusters of hairs. Rarely, blistering occurs. In some cases, other areas of the skin or the mucous membranes are affected. Hair loss may be permanent if the disease causes permanent scarring (cicatricial alopecia). Treatment options may include steroids, isotretinoin or hydroxychloroquine. Hair transplant may be effective in some cases.

MalaCards based summary : Lichen Planopilaris, also known as lichen planus follicularis, is related to epidermolysis bullosa and frontal fibrosing alopecia. An important gene associated with Lichen Planopilaris is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways/superpathways are Cytoskeleton remodeling Neurofilaments and MHC class II antigen presentation. The drugs Apremilast and Lidocaine have been mentioned in the context of this disorder. Affiliated tissues include skin, thyroid and lung, and related phenotypes are hepatitis and hyperkeratosis

Related Diseases for Lichen Planopilaris

Diseases related to Lichen Planopilaris via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 145)
# Related Disease Score Top Affiliating Genes
1 epidermolysis bullosa 30.0 COL17A1 KRT14 KRT5
2 frontal fibrosing alopecia 12.0
3 pseudopelade of brocq 11.6
4 vacterl association 11.5
5 esophageal atresia 11.5
6 pneumonic plague 11.4
7 esophageal atresia/tracheoesophageal fistula 11.4
8 tracheoesophageal fistula 11.4
9 lichen planus pemphigoides 11.4
10 celiac disease 1 11.3
11 pustulosis palmaris et plantaris 11.3
12 tetralogy of fallot 11.3
13 lipomatosis, multiple 11.2
14 congenital anomalies of kidney and urinary tract 2 11.1
15 leukemia, acute myeloid 11.1
16 paraphilia disorder 11.1
17 pedophilia 11.1
18 enamel erosion 11.1
19 hair disease 11.1
20 infiltrating lipoma 11.1
21 lichen disease 11.1
22 prostate carcinoma in situ 11.1
23 seborrheic dermatitis 11.1
24 acute monoblastic leukemia 11.1
25 acute t cell leukemia 10.9
26 alopecia 10.8
27 lichen planus 10.5
28 lupus erythematosus 10.4
29 alopecia, androgenetic, 1 10.3
30 androgenic alopecia 10.3
31 discoid lupus erythematosus 10.3
32 graham-little-piccardi-lassueur syndrome 10.3
33 hypertrichosis 10.2
34 rosacea 10.2
35 depression 10.2
36 nevus, epidermal 10.2
37 dermatitis herpetiformis, familial 10.2
38 dermatitis 10.2
39 folliculitis 10.2
40 thyroiditis 10.2
41 dermatitis herpetiformis 10.2
42 psoriasis 10.2
43 quinquaud's decalvans folliculitis 10.2
44 plague 10.1
45 benign breast adenomyoepithelioma 10.1 KRT14 KRT5
46 breast adenomyoepithelioma 10.1 KRT14 KRT5
47 epidermolysis bullosa simplex, autosomal recessive 1 10.1 KRT14 KRT5
48 large cell acanthoma 10.1 KRT14 KRT5
49 apocrine sweat gland neoplasm 10.1 KRT14 KRT5
50 epidermolysis bullosa simplex, localized 10.1 KRT14 KRT5

Graphical network of the top 20 diseases related to Lichen Planopilaris:



Diseases related to Lichen Planopilaris

Symptoms & Phenotypes for Lichen Planopilaris

Human phenotypes related to Lichen Planopilaris:

59 32 (show all 16)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hepatitis 59 32 occasional (7.5%) Occasional (29-5%) HP:0012115
2 hyperkeratosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000962
3 abnormal blistering of the skin 59 32 occasional (7.5%) Occasional (29-5%) HP:0008066
4 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
5 abnormality of the fingernails 59 32 frequent (33%) Frequent (79-30%) HP:0001231
6 alopecia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001596
7 skin ulcer 59 32 frequent (33%) Frequent (79-30%) HP:0200042
8 hypopigmented skin patches 59 32 occasional (7.5%) Occasional (29-5%) HP:0001053
9 papule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200034
10 pterygium 59 32 occasional (7.5%) Occasional (29-5%) HP:0001059
11 lichenification 59 32 hallmark (90%) Very frequent (99-80%) HP:0100725
12 onycholysis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001806
13 neoplasm of the oral cavity 59 32 occasional (7.5%) Occasional (29-5%) HP:0100649
14 dermal atrophy 59 32 frequent (33%) Frequent (79-30%) HP:0004334
15 abnormality of the intestine 59 Occasional (29-5%)
16 abnormal intestine morphology 32 occasional (7.5%) HP:0002242

Drugs & Therapeutics for Lichen Planopilaris

Drugs for Lichen Planopilaris (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 38)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Apremilast Approved, Investigational Phase 4 608141-41-9 11561674
2
Lidocaine Approved, Vet_approved Phase 4 137-58-6 3676
3 Immunologic Factors Phase 4
4 Anti-Inflammatory Agents, Non-Steroidal Phase 4
5 Immunosuppressive Agents Phase 4
6 Analgesics Phase 4,Early Phase 1
7 Analgesics, Non-Narcotic Phase 4
8 Antirheumatic Agents Phase 4
9 Anti-Infective Agents Phase 4
10 Anti-Bacterial Agents Phase 4
11 Peripheral Nervous System Agents Phase 4,Early Phase 1
12 Anti-Inflammatory Agents Phase 4
13 Angiogenesis Modulating Agents Phase 4
14 Angiogenesis Inhibitors Phase 4
15 Central Nervous System Depressants Phase 4,Early Phase 1
16 Anesthetics, Local Phase 4
17 Anesthetics Phase 4
18 Diuretics, Potassium Sparing Phase 4
19 Sodium Channel Blockers Phase 4
20 Anti-Arrhythmia Agents Phase 4
21
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
22 Alkylating Agents Phase 2
23 Antineoplastic Agents, Alkylating Phase 2
24
Gabapentin Approved, Investigational Early Phase 1 60142-96-3 3446
25
gamma-Aminobutyric acid Approved, Investigational Early Phase 1 56-12-2 119
26 Anti-Anxiety Agents Early Phase 1
27 Excitatory Amino Acids Early Phase 1
28 calcium channel blockers Early Phase 1
29 Antiparkinson Agents Early Phase 1
30 GABA Agents Early Phase 1
31 Neurotransmitter Agents Early Phase 1
32 Excitatory Amino Acid Antagonists Early Phase 1
33 Tranquilizing Agents Early Phase 1
34 Pharmaceutical Solutions Early Phase 1
35 Calcium, Dietary Early Phase 1
36 Anticonvulsants Early Phase 1
37 Psychotropic Drugs Early Phase 1
38 Antimanic Agents Early Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Trial Evaluating the Efficacy of Apremilast for the Treatment of Frontal Fibrosing Alopecia Recruiting NCT03422640 Phase 4 Apremilast
2 Pain Outcomes Following Intralesional Corticosteroid Injections Not yet recruiting NCT03630198 Phase 4 Corticosteroid with lidocaine;Corticosteroid with normal saline
3 Valchlor in the Treatment of Lichen Planopilaris Recruiting NCT03417141 Phase 2 Valchlor
4 Expression of Fas Protein in Skin Biopsies of Participants With Scarring Alopecia Completed NCT00691769
5 Role of Neurogenic Inflammation and Topical 6% Gabapentin Therapy in Symptomatic Scarring Alopecia Recruiting NCT03346668 Early Phase 1 Topical gabapentin
6 Design and Validation of a New Assessment Tool for Lichen Planopilaris Active, not recruiting NCT03082560
7 Trichoscopy in Diagnosis of Immunobollous Diseases Not yet recruiting NCT03478072
8 Platelet Rich Plasma for Frontal Fibrosing Alopecia Not yet recruiting NCT03335228 Not Applicable

Search NIH Clinical Center for Lichen Planopilaris

Genetic Tests for Lichen Planopilaris

Anatomical Context for Lichen Planopilaris

MalaCards organs/tissues related to Lichen Planopilaris:

41
Skin, Thyroid, Lung, Kidney, Ovary, Prostate, B Cells

Publications for Lichen Planopilaris

Articles related to Lichen Planopilaris:

(show top 50) (show all 173)
# Title Authors Year
1
Ocular Surface Findings in Patients With Lichen Planopilaris. ( 29757849 )
2018
2
Platelet rich plasma for the treatment of lichen planopilaris. ( 29807058 )
2018
3
Ultraviolet filters in hair-care products: a possible link with frontal fibrosing alopecia and lichen planopilaris. ( 28994172 )
2018
4
Lichen planopilaris: A retrospective study of 32 cases in an Australian tertiary referral hair clinic. ( 29297588 )
2018
5
Clinical Description of Frontal Fibrosing Alopecia with Concomitant Lichen Planopilaris. ( 29765970 )
2018
6
Lichen Planopilaris with Pustules: A Diagnostic Challenge. ( 29765960 )
2018
7
Ulcerating Lichen Planopilaris - Successful Treatment by Surgery. ( 29483996 )
2018
8
Lichen planopilaris and frontal fibrosing alopecia cannot be differentiated by histopathology. ( 29369400 )
2018
9
Therapeutic management of classic lichen planopilaris: a systematic review. ( 29520159 )
2018
10
Lichen planopilaris associated with pembrolizumab in a patient with metastatic melanoma. ( 29387763 )
2018
11
Lichen Planopilaris and Frontal Fibrosing Alopecia as Model Epithelial Stem Cell Diseases. ( 29661566 )
2018
12
The Timing and Distribution of Non-Scalp Hair Loss in Patients with Lichen Planopilaris and Frontal Fibrosing Alopecia: A Survey-Based Study. ( 29559401 )
2018
13
Facial Papules in a Patient With Lichen Planopilaris. ( 29299591 )
2018
14
Differential expression of mTOR signaling pathway proteins in lichen planopilaris and frontal fibrosing alopecia. ( 30278995 )
2018
15
IL-17-positive mast cell infiltration in the lesional skin of lichen planopilaris: possible role of mast cells in inducing inflammation and dermal fibrosis in cicatricial alopecia. ( 30379356 )
2018
16
Lichen Planopilaris Caused by Wig Attachment: A Case of Koebner Phenomenon in Frontal Fibrosing Alopecia. ( 30386077 )
2018
17
"Normal-appearing" scalp areas are also affected in lichen planopilaris and frontal fibrosing alopecia: an observational histopathologic study of 40 patients. ( 30403306 )
2018
18
Evidence for neurogenic inflammation in lichen planopilaris and frontal fibrosing alopecia pathogenic mechanism. ( 30408256 )
2018
19
A comparative immunohistochemical study of epidermal and dermal/perifollicular Langerhans cell concentration in discoid lupus erythematosus and lichen planopilaris: a cross-sectional study. ( 30376791 )
2018
20
Medical comorbidities in patients with lichen planopilaris, a retrospective case-control study. ( 29682736 )
2018
21
Association of Lichen Planopilaris With Dyslipidemia. ( 29998306 )
2018
22
Lichen planopilaris beyond scalp: a case series with dermoscopy-histopathology correlation. ( 30073666 )
2018
23
Familial facial lichen planopilaris and satisfactory response to isotretinoin. ( 30133952 )
2018
24
Pediatric ocular lichen planus and lichen planopilaris: One new case and a review of the literature. ( 30168195 )
2018
25
Comorbid conditions in lichen planopilaris: A retrospective data analysis of 334 patients. ( 30175224 )
2018
26
Lichen planopilaris with Koebner phenomenon. ( 30258967 )
2018
27
Scalp porocarcinoma and lichen planopilaris. ( 30261574 )
2018
28
Tofacitinib for the treatment of lichen planopilaris: A case series. ( 30264512 )
2018
29
Facial Lichen Planopilaris: A Separate Entity or Not? A Case Series. ( 30386080 )
2018
30
Lichen planopilaris: Retrospective study on the characteristics and treatment of 291 patients. ( 30411987 )
2018
31
Novel Treatment Using Low-Dose Naltrexone for Lichen Planopilaris. ( 29141063 )
2017
32
Isolated Body Hair Loss: An Unusual Presentation of Lichen Planopilaris. ( 28232914 )
2017
33
Reply to: "Plasmacytoid dendritic cell content, clustering, and distribution pattern are useful parameters in differentiating lupus alopecia from lichen planopilaris". ( 28089018 )
2017
34
Hair transplantation for the treatment of lichen planopilaris and frontal fibrosing alopecia: A report of two cases. ( 28736812 )
2017
35
Lichen, Planopilaris ( 29262050 )
2017
36
Lichen planopilaris developed during childhood. ( 28954108 )
2017
37
Effectiveness Of Low-Level Laser Therapy In Lichen Planopilaris. ( 29198781 )
2017
38
Epithelial-to-Mesenchymal Stem Cell Transition in a Human Organ: Lessons from Lichen Planopilaris. ( 29106928 )
2017
39
Reply to "Clusters of CD123(+) plasmacytoid dendritic cells help distinguish lupus alopecia from lichen planopilaris": Plasmacytoid dendritic cell content, clustering, and distribution pattern are useful parameters in differentiating lupus alopecia from lichen planopilaris. ( 28089017 )
2017
40
Lichen planopilaris in a Latin American (Chilean) population: demographics, clinical profile and treatment experience. ( 28748570 )
2017
41
The role of hydroxychloroquine in the treatment of lichen planopilaris: A retrospective case series and review. ( 28168851 )
2017
42
Coexistence of lichen planus follicularis tumidus and Hashimoto's thyroiditis. ( 28954124 )
2017
43
The depth of inflammation in frontal fibrosing alopecia and lichen planopilaris: A potential distinguishing feature. ( 28522040 )
2017
44
Treatment of Lichen planopilaris: Methotrexate or Cyclosporine A therapy? ( 28944688 )
2017
45
Clinical Efficacy and Safety of Methotrexate versus Hydroxychloroquine in Preventing Lichen Planopilaris Progress: A Randomized Clinical Trial. ( 28584619 )
2017
46
Prevalence of hormonal and endocrine dysfunction in patients with lichen planopilaris (LPP): A retrospective data analysis of 168 patients. ( 28088992 )
2017
47
Invisible Lichen Planopilaris Unmasked by Dermatoscopy. ( 28839393 )
2017
48
Oral lichen planus preceding concomitant lichen planopilaris. ( 27544399 )
2016
49
Commentary on A New Subtype of Lichen Planopilaris Affecting Vellus Hairs and Clinically Mimicking Androgenetic Alopecia. ( 27661431 )
2016
50
The Use of Anti-Keratin 903 Antibodies to Visualize Colloid Bodies and Diagnose Lichen Planopilaris. ( 26913845 )
2016

Variations for Lichen Planopilaris

Expression for Lichen Planopilaris

Search GEO for disease gene expression data for Lichen Planopilaris.

Pathways for Lichen Planopilaris

Pathways related to Lichen Planopilaris according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.44 KRT14 KRT5
2 11.38 HLA-DQB1 HLA-DRB1
3 11.31 HLA-DQB1 HLA-DRB1
4 11.21 HLA-DQB1 HLA-DRB1
5 11.07 KRT14 KRT5
6 10.81 HLA-DQB1 HLA-DRB1
7 10.37 HLA-DQB1 HLA-DRB1

GO Terms for Lichen Planopilaris

Cellular components related to Lichen Planopilaris according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 keratin filament GO:0045095 9.43 KRT14 KRT5
2 trans-Golgi network membrane GO:0032588 9.4 HLA-DQB1 HLA-DRB1
3 endocytic vesicle membrane GO:0030666 9.37 HLA-DQB1 HLA-DRB1
4 ER to Golgi transport vesicle membrane GO:0012507 9.32 HLA-DQB1 HLA-DRB1
5 transport vesicle membrane GO:0030658 9.26 HLA-DQB1 HLA-DRB1
6 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DQB1 HLA-DRB1
7 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQB1 HLA-DRB1
8 MHC class II protein complex GO:0042613 8.62 HLA-DQB1 HLA-DRB1

Biological processes related to Lichen Planopilaris according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.46 HLA-DQB1 HLA-DRB1
2 epithelial cell differentiation GO:0030855 9.43 KRT14 PPARG
3 epidermis development GO:0008544 9.43 COL17A1 KRT14 KRT5
4 interferon-gamma-mediated signaling pathway GO:0060333 9.4 HLA-DQB1 HLA-DRB1
5 antigen processing and presentation GO:0019882 9.37 HLA-DQB1 HLA-DRB1
6 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.32 HLA-DQB1 HLA-DRB1
7 humoral immune response mediated by circulating immunoglobulin GO:0002455 9.16 HLA-DQB1 HLA-DRB1
8 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.96 HLA-DQB1 HLA-DRB1
9 hemidesmosome assembly GO:0031581 8.8 COL17A1 KRT14 KRT5

Molecular functions related to Lichen Planopilaris according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 9.16 KRT14 KRT5
2 peptide antigen binding GO:0042605 8.96 HLA-DQB1 HLA-DRB1
3 MHC class II receptor activity GO:0032395 8.62 HLA-DQB1 HLA-DRB1

Sources for Lichen Planopilaris

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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