FFA
MCID: FRN020
MIFTS: 48

Frontal Fibrosing Alopecia (FFA)

Categories: Eye diseases, Rare diseases, Skin diseases

Aliases & Classifications for Frontal Fibrosing Alopecia

MalaCards integrated aliases for Frontal Fibrosing Alopecia:

Name: Frontal Fibrosing Alopecia 20 20 58 17
Lichen Planopilaris 20 58 54 17 32
Lichen Planus Follicularis 20 58 70
Follicular Lichen Planus 20 58
Lichen Follicularis 20 58
Ffa 20 58
Lpp 20 58
Postmenopausal Frontal Fibrosing Alopecia 70
Lichen Planopilaris Classic Type 20
Kossard Disease 20

Characteristics:

Orphanet epidemiological data:

58
frontal fibrosing alopecia
Age of onset: Adult;
lichen planopilaris
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

ICD10 32 L66.1
MESH via Orphanet 45 C535892
ICD10 via Orphanet 33 L66.1
UMLS via Orphanet 71 C0023645 C1274700
UMLS 70 C0023645 C1274700

Summaries for Frontal Fibrosing Alopecia

GARD : 20 Frontal fibrosing alopecia (FFA) is a form of lichen planopilaris that is characterized primarily by slowly progressive hair loss ( alopecia ) and scarring on the scalp near the forehead. In some cases, the eyebrows, eye lashes and/or other parts of the body may be involved, as well. Although it has been suggested that FFA may be due to hormonal changes or an autoimmune response, the exact cause of this condition is not yet known. There is currently no cure for FFA; however, treatment with certain types of medications may stop or slow hair loss in some cases.

MalaCards based summary : Frontal Fibrosing Alopecia, also known as lichen planopilaris, is related to alopecia areata and lipid metabolism disorder. An important gene associated with Frontal Fibrosing Alopecia is CYP21A2 (Cytochrome P450 Family 21 Subfamily A Member 2), and among its related pathways/superpathways are Cellular senescence (KEGG) and Phagosome. The drugs Apremilast and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include eye, liver and myeloid, and related phenotypes are hyperkeratosis and alopecia

Wikipedia : 73 Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal... more...

Related Diseases for Frontal Fibrosing Alopecia

Diseases related to Frontal Fibrosing Alopecia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 267)
# Related Disease Score Top Affiliating Genes
1 alopecia areata 30.6 HLA-B HLA-A
2 lipid metabolism disorder 29.9 PPARG CYP21A2
3 polycystic ovary syndrome 29.8 PPARG CYP21A2
4 herpes zoster 29.6 HLA-B HLA-A
5 celiac disease 1 29.4 HLA-B HLA-A CYP21A2
6 alopecia 11.3
7 pseudopelade of brocq 11.2
8 lipomatosis, multiple 11.1
9 pustulosis palmaris et plantaris 11.1
10 vacterl association 11.0
11 tetralogy of fallot 11.0
12 esophageal atresia 11.0
13 schizophrenia 11.0
14 pneumonic plague 10.9
15 plague 10.9
16 leukemia, acute myeloid 10.9
17 lichen planus 10.9
18 wilson-turner x-linked mental retardation syndrome 10.9
19 vacterl association, x-linked, with or without hydrocephalus 10.9
20 short-rib thoracic dysplasia 10 with or without polydactyly 10.9
21 infiltrating lipoma 10.9
22 prostate carcinoma in situ 10.9
23 cebpa-associated familial acute myeloid leukemia 10.9
24 etv6 thrombocytopenia and predisposition to leukemia 10.9
25 acute monoblastic leukemia 10.9
26 lichen planus pigmentosus 10.7
27 alopecia, androgenetic, 1 10.6
28 androgenic alopecia 10.6
29 keratosis 10.6
30 graham-little-piccardi-lassueur syndrome 10.6
31 pleomorphic lipoma 10.6
32 vitiligo-associated multiple autoimmune disease susceptibility 6 10.5
33 vitiligo-associated multiple autoimmune disease susceptibility 1 10.5
34 dowling-degos disease 1 10.5
35 hypothyroidism 10.5
36 quinquaud folliculitis decalvans 10.5
37 rosacea 10.4
38 cutaneous lupus erythematosus 10.4
39 oral lichen planus 10.4
40 discoid lupus erythematosus 10.4
41 lichen sclerosus et atrophicus 10.3
42 contact dermatitis 10.3
43 chronic cutaneous lupus erythematosus 10.3
44 skin disease 10.3
45 dermatitis herpetiformis, familial 10.2
46 thyroiditis 10.2
47 dermatitis herpetiformis 10.2
48 seborrheic dermatitis 10.2
49 hypertriglyceridemia, familial 10.2
50 hair whorl 10.2

Graphical network of the top 20 diseases related to Frontal Fibrosing Alopecia:



Diseases related to Frontal Fibrosing Alopecia

Symptoms & Phenotypes for Frontal Fibrosing Alopecia

Human phenotypes related to Frontal Fibrosing Alopecia:

58 31 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperkeratosis 58 31 hallmark (90%) Very frequent (99-80%) HP:0000962
2 alopecia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001596
3 papule 58 31 hallmark (90%) Very frequent (99-80%) HP:0200034
4 lichenification 58 31 hallmark (90%) Very frequent (99-80%) HP:0100725
5 abnormal fingernail morphology 58 31 frequent (33%) Frequent (79-30%) HP:0001231
6 skin ulcer 58 31 frequent (33%) Frequent (79-30%) HP:0200042
7 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
8 dermal atrophy 58 31 frequent (33%) Frequent (79-30%) HP:0004334
9 hepatitis 58 31 occasional (7.5%) Occasional (29-5%) HP:0012115
10 hypopigmented skin patches 58 31 occasional (7.5%) Occasional (29-5%) HP:0001053
11 pterygium 58 31 occasional (7.5%) Occasional (29-5%) HP:0001059
12 abnormal blistering of the skin 58 31 occasional (7.5%) Occasional (29-5%) HP:0008066
13 onycholysis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001806
14 neoplasm of the oral cavity 58 31 occasional (7.5%) Occasional (29-5%) HP:0100649
15 abnormal intestine morphology 58 31 occasional (7.5%) Occasional (29-5%) HP:0002242

GenomeRNAi Phenotypes related to Frontal Fibrosing Alopecia according to GeneCards Suite gene sharing:

26 (show all 22)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-103 10.65 HLA-A HLA-B
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-110 10.65 HLA-A HLA-B
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-156 10.65 HLA-A HLA-B
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-178 10.65 HLA-A HLA-B
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-19 10.65 HLA-A HLA-B
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-31 10.65 HLA-A HLA-B
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-7 10.65 HLA-A HLA-B
8 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.58 CYP21A2
9 Increased shRNA abundance (Z-score > 2) GR00366-A-105 9.58 CYP21A2
10 Increased shRNA abundance (Z-score > 2) GR00366-A-139 9.58 CYP21A2 PPARG
11 Increased shRNA abundance (Z-score > 2) GR00366-A-145 9.58 CYP21A2 PPARG
12 Increased shRNA abundance (Z-score > 2) GR00366-A-161 9.58 CYP21A2
13 Increased shRNA abundance (Z-score > 2) GR00366-A-17 9.58 PPARG
14 Increased shRNA abundance (Z-score > 2) GR00366-A-184 9.58 CYP21A2
15 Increased shRNA abundance (Z-score > 2) GR00366-A-185 9.58 CYP21A2
16 Increased shRNA abundance (Z-score > 2) GR00366-A-203 9.58 CYP21A2 PPARG
17 Increased shRNA abundance (Z-score > 2) GR00366-A-206 9.58 CYP21A2
18 Increased shRNA abundance (Z-score > 2) GR00366-A-208 9.58 CYP21A2
19 Increased shRNA abundance (Z-score > 2) GR00366-A-213 9.58 CYP21A2
20 Increased shRNA abundance (Z-score > 2) GR00366-A-36 9.58 PPARG
21 Increased shRNA abundance (Z-score > 2) GR00366-A-63 9.58 CYP21A2
22 Increased shRNA abundance (Z-score > 2) GR00366-A-8 9.58 CYP21A2

Drugs & Therapeutics for Frontal Fibrosing Alopecia

Drugs for Frontal Fibrosing Alopecia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Apremilast Approved, Investigational Phase 4 608141-41-9 11561674
2 Antirheumatic Agents Phase 4
3 Anti-Inflammatory Agents Phase 4
4 Anti-Inflammatory Agents, Non-Steroidal Phase 4
5 Analgesics Phase 4
6 Analgesics, Non-Narcotic Phase 4
7
Mechlorethamine Approved, Investigational Phase 2 51-75-2 4033
8
Ethanol Approved Phase 2 64-17-5 702
9
Naltrexone Approved, Investigational, Vet_approved Phase 2 16590-41-3 5360515
10 Alkylating Agents Phase 2
11 Immunologic Factors Phase 2
12 Immunoglobulins Phase 2
13 Antibodies Phase 2
14 Antibodies, Monoclonal Phase 2
15 Narcotics Phase 2
16 Narcotic Antagonists Phase 2
17
Triamcinolone Approved, Vet_approved 124-94-7 31307
18 Pharmaceutical Solutions
19 Triamcinolone diacetate
20 Triamcinolone hexacetonide
21 triamcinolone acetonide

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Open Label Trial Evaluating the Efficacy of Apremilast for the Treatment of Frontal Fibrosing Alopecia Unknown status NCT03422640 Phase 4 Apremilast
2 Valchlor in the Treatment of Lichen Planopilaris: A Single Arm, Open- Label, Exploratory Study Completed NCT03417141 Phase 2 Mechlorethamine 0.016% Top Gel
3 A Proof of Concept Study to Evaluate the Efficacy, Safety and Tolerability of Secukinumab 300 mg Over 32 Weeks in Adult Patients With Biopsy-proven Forms of Lichen Planus Not Adequately Controlled With Topical Therapies - PRELUDE Recruiting NCT04300296 Phase 2 Cutaneous lichen planus secukinumab 300 mg Q4W;Cutaneous lichen planus placebo;Mucosal lichen planus placebo;Lichen planopilaris placebo;Mucosal lichen planus secukinumab 300 mg Q4W;Lichen planopilaris secukinumab 300 mg Q4W
4 Oral Low-Dose Naltrexone in the Treatment of Lichen Planopilaris and Frontal Fibrosing Alopecia; an Uncontrolled Open-label Prospective Study Active, not recruiting NCT04409041 Phase 2 Low-Dose Naltrexone
5 Prospective Double-blind, Placebo Controlled Study to Assess the Efficacy of Intralesional Corticoid on the Treatment of Frontal Fibrosing Alopecia Unknown status NCT02467101
6 Design and Validation of a New Assessment Tool for Lichen Planopilaris, the Boston Grade of Activity in Lichen Planopilaris (Boston GOAL) Active, not recruiting NCT03082560
7 Assessment of the Safety and Efficacy of Intralesional Platelet Rich Plasma in Reducing Scalp Symptoms and Promoting Hair Growth in Frontal Fibrosing Alopecia Not yet recruiting NCT03335228

Search NIH Clinical Center for Frontal Fibrosing Alopecia

Genetic Tests for Frontal Fibrosing Alopecia

Anatomical Context for Frontal Fibrosing Alopecia

MalaCards organs/tissues related to Frontal Fibrosing Alopecia:

40
Eye, Liver, Myeloid, Heart, Lung, Skin, Bone Marrow

Publications for Frontal Fibrosing Alopecia

Articles related to Frontal Fibrosing Alopecia:

(show top 50) (show all 765)
# Title Authors PMID Year
1
Frontal fibrosing alopecia: reflections and hypotheses on aetiology and pathogenesis. 61 20
27198858 2016
2
Case series of familial frontal fibrosing alopecia and a review of the literature. 61 20
25614294 2015
3
Lichen planopilaris treated with a peroxisome proliferator-activated receptor gamma agonist. 61 54
20026843 2009
4
Scarring alopecia and the PPAR-gamma connection. 54 61
19369934 2009
5
Lichen planopilaris in men: a retrospective clinicopathologic study of 19 patients. 61
33128471 2021
6
Comment on: Risk factors for frontal fibrosing alopecia: A case-control study in a multiracial population. 61
33253842 2021
7
Regarding methodologic concerns in clinical studies on frontal fibrosing alopecia. 61
33253845 2021
8
Prevalence estimates for lichen planopilaris and frontal fibrosing alopecia in a New York City health care system. 61
33137442 2021
9
Localized grey hair repigmentation (canities reversal) in patients with frontal fibrosing alopecia. 61
33651461 2021
10
Dysbiosis and Enhanced Beta-Defensin Production in Hair Follicles of Patients with Lichen Planopilaris and Frontal Fibrosing Alopecia. 61
33800045 2021
11
Trichoscopic features of mild frontal fibrosing alopecia. 61
32885489 2021
12
Epidemiologic and clinical features of pattern III frontal fibrosing alopecia (pseudo fringe type): A multicenter series of 38 patients. 61
32497694 2021
13
Features of classic lichen planopilaris and frontal fibrosing alopecia in reflectance confocal microscopy: A preliminary study. 61
32743819 2021
14
Suitability of high-frequency ultrasonography (20 MHz) in evaluation of various forms of primary cicatricial alopecia in relation to trichoscopy - pilot study. 61
33751668 2021
15
Risk factors for frontal fibrosing alopecia: A case-control study in a multiracial population. 61
32835739 2021
16
Frontal fibrosing alopecia in a woman with vulvar erosive lichen planus. 61
33771365 2021
17
Oral minoxidil treatment for hair loss: A review of efficacy and safety. 61
32622136 2021
18
Review of oral minoxidil as treatment of hair disorders: in search of the perfect dose. 61
33660357 2021
19
Co-localization of alopecia areata and lichen planopilaris in a patient receiving immunosuppressants: A rare case. 61
33167077 2021
20
The spectrum of pediatric scarring alopecia: A retrospective review of 27 patients seen at Mayo Clinic. 61
33644931 2021
21
Common causes of hair loss - clinical manifestations, trichoscopy and therapy. 61
33290611 2021
22
It has the Erythema of a Lichen Planopilaris, it has the Hyperkeratosis of a Lichen Planopilaris, but it is Not a Lichen Planopilaris: About the "Lichen Planopilaris-Like" Form of Folliculitis Decalvans. 61
32769552 2021
23
Erosive pustular dermatosis in a patient with lichen planopilaris treated with aminolevulinic acid photodynamic therapy. 61
33556619 2021
24
"Follicular prominency", a new clinical sign in lichen planopilaris: Fire under the ashes. 61
33580985 2021
25
Adverse cutaneous reactions secondary to hydroxychloroquine in patients with dermatomyositis, lupus erythematosus, and lichen planopilaris. 61
33607179 2021
26
Frontal fibrosing alopecia and genital Lichen sclerosus: Single-center experience. 61
32590887 2021
27
Reply to ''Coexistence of frontal fibrosing alopecia and alopecia areata: 3 new cases and review of the literature'': plasmacytoid dendritic cell as possible link. 61
33590490 2021
28
Alopecia Areata Mimicking Frontal Fibrosing Alopecia. 61
33754093 2021
29
The association of frontal fibrosing alopecia with skin and hair care products: A survey-based case series of 56 patients seen at the Mayo Clinic. 61
32437720 2021
30
Eyebrow Regrowth in Patients with Frontal Fibrosing Alopecia Treated with Low-Dose Oral Minoxidil. 61
33796556 2021
31
Prostaglandin analogue for treatment of eyebrow loss in frontal fibrosing alopecia: three cases with different outcomes. 61
32780892 2021
32
Letter to the editor: Frontal fibrosing alopecia: An autoimmune manifestation? 61
33321246 2021
33
Beard loss in men with frontal fibrosing alopecia. 61
33484769 2021
34
Yellow dots in frontal fibrosing alopecia. 61
32683726 2021
35
Colour Doppler ultrasound study in patients with frontal fibrosing alopecia. 61
33455050 2021
36
Hair Transplantation in Frontal Fibrosing Alopecia and Lichen Planopilaris: A Systematic Review. 61
32045028 2021
37
Histological evidence for epidermal and dermal atrophy of the alopecic band in treatment-naïve patients with Frontal Fibrosing Alopecia. 61
32594595 2021
38
Concomitant occurrence of frontal fibrosing alopecia and trichotemnomania: The importance of trichoscopy. 61
33580936 2021
39
Eyelash loss in frontal fibrosing alopecia: Microscopic features of two cases. 61
33160730 2021
40
Comedone-Like Lesions as a Manifestation of Lichen Planopilaris beyond the Scalp: A Case Report with Dermoscopic Features and Literature Review. 61
33790753 2021
41
Lichen planopilaris with significant post-inflammatory pigmentary alteration. 61
33560796 2021
42
A case of frontal fibrosing alopecia in a 19th century portrait? 61
33434364 2021
43
Lichen-planopilaris-like scarring pattern in a patient with alopecia and pityriasis rubra pilaris. 61
32949041 2021
44
Occipital Fibrosing Alopecia in a Young Male: A Case Report. 61
33614725 2021
45
Nilotinib-induced keratosis pilaris associated with cicatricial alopecia resembling frontal fibrosing alopecia. 61
33236495 2021
46
Lichen planopilaris restricted to the vitiliginous skin on the leg. 61
33244857 2021
47
Cicatricial pattern hair loss is not a variant of lichen planopilaris. 61
32376428 2020
48
Commentary on: "Cicatricial pattern hair loss is not a variant of lichen planopilaris". 61
32380216 2020
49
Isotretinoin for the treatment of facial lichen planopilaris: A new indication for an old drug, a case series study. 61
33363772 2020
50
Diffuse variants of scalp lichen planopilaris: Clinical, trichoscopic, and histopathologic features of 40 patients. 61
31706933 2020

Variations for Frontal Fibrosing Alopecia

Expression for Frontal Fibrosing Alopecia

Search GEO for disease gene expression data for Frontal Fibrosing Alopecia.

Pathways for Frontal Fibrosing Alopecia

GO Terms for Frontal Fibrosing Alopecia

Cellular components related to Frontal Fibrosing Alopecia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum membrane GO:0005789 9.58 HLA-B HLA-A CYP21A2
2 early endosome membrane GO:0031901 9.37 HLA-B HLA-A
3 recycling endosome membrane GO:0055038 9.32 HLA-B HLA-A
4 phagocytic vesicle membrane GO:0030670 9.26 HLA-B HLA-A
5 ER to Golgi transport vesicle membrane GO:0012507 9.16 HLA-B HLA-A
6 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-B HLA-A
7 MHC class I protein complex GO:0042612 8.62 HLA-B HLA-A

Biological processes related to Frontal Fibrosing Alopecia according to GeneCards Suite gene sharing:

(show all 11)
# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.67 PPARG HLA-B HLA-A
2 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-dependent GO:0002479 9.48 HLA-B HLA-A
3 interferon-gamma-mediated signaling pathway GO:0060333 9.46 HLA-B HLA-A
4 type I interferon signaling pathway GO:0060337 9.43 HLA-B HLA-A
5 antigen processing and presentation GO:0019882 9.4 HLA-B HLA-A
6 antigen processing and presentation of peptide antigen via MHC class I GO:0002474 9.37 HLA-B HLA-A
7 positive regulation of T cell mediated cytotoxicity GO:0001916 9.32 HLA-B HLA-A
8 detection of bacterium GO:0016045 9.26 HLA-B HLA-A
9 antigen processing and presentation of exogenous peptide antigen via MHC class I, TAP-independent GO:0002480 9.16 HLA-B HLA-A
10 antigen processing and presentation of endogenous peptide antigen via MHC class I via ER pathway, TAP-independent GO:0002486 8.96 HLA-B HLA-A
11 protection from natural killer cell mediated cytotoxicity GO:0042270 8.62 HLA-B HLA-A

Molecular functions related to Frontal Fibrosing Alopecia according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-B HLA-A
2 TAP binding GO:0046977 8.62 HLA-B HLA-A

Sources for Frontal Fibrosing Alopecia

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