Familial frontal fibrosing alopecia: A cross‐sectional study of 20 cases from nine families

Background/Objectives Frontal fibrosing alopecia (FFA) is a scarring alopecia whose prevalence is increasing. The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibr...

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Veröffentlicht in:Australasian journal of dermatology 2019-05, Vol.60 (2), p.e113-e118
Hauptverfasser: Porriño‐Bustamante, María Librada, López‐Nevot, Miguel Ángel, Aneiros‐Fernández, José, García‐Lora, Elena, Fernández‐Pugnaire, María Antonia, Arias‐Santiago, Salvador
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container_issue 2
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container_title Australasian journal of dermatology
container_volume 60
creator Porriño‐Bustamante, María Librada
López‐Nevot, Miguel Ángel
Aneiros‐Fernández, José
García‐Lora, Elena
Fernández‐Pugnaire, María Antonia
Arias‐Santiago, Salvador
description Background/Objectives Frontal fibrosing alopecia (FFA) is a scarring alopecia whose prevalence is increasing. The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported. Material and methods A cross‐sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed. Results Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo‐parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%). Conclusion This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non‐familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.
doi_str_mv 10.1111/ajd.12951
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The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported. Material and methods A cross‐sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed. Results Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo‐parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%). Conclusion This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non‐familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.</description><identifier>ISSN: 0004-8380</identifier><identifier>EISSN: 1440-0960</identifier><identifier>DOI: 10.1111/ajd.12951</identifier><identifier>PMID: 30430555</identifier><language>eng</language><publisher>Australia: Wiley Subscription Services, Inc</publisher><subject>Adult ; Age of Onset ; Aged ; Aged, 80 and over ; Alopecia ; Alopecia - classification ; Alopecia - genetics ; Alopecia - pathology ; Atrophy ; Autoimmune diseases ; Autoimmunity ; cicatricial alopecia ; Cross-Sectional Studies ; Dermoscopy ; Erythema ; Erythema - complications ; European Continental Ancestry Group ; familial ; Female ; Fibrosis ; frontal fibrosing alopecia ; Genetic Predisposition to Disease ; hair ; Hair Follicle - pathology ; Hair loss ; Humans ; Keratosis - complications ; Lymphocytes - pathology ; Male ; Middle Aged ; Patients ; scarring alopecia ; Sebaceous Glands - pathology ; Sex Distribution ; Spain</subject><ispartof>Australasian journal of dermatology, 2019-05, Vol.60 (2), p.e113-e118</ispartof><rights>2018 The Australasian College of Dermatologists</rights><rights>2018 The Australasian College of Dermatologists.</rights><rights>Copyright © 2019 The Australasian College of Dermatologists</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3531-800f099979de90ec934d1ad3c4855aaab24698bbd7b7a8c5c855032dee3b4d603</citedby><cites>FETCH-LOGICAL-c3531-800f099979de90ec934d1ad3c4855aaab24698bbd7b7a8c5c855032dee3b4d603</cites><orcidid>0000-0002-3471-4154</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://onlinelibrary.wiley.com/doi/pdf/10.1111%2Fajd.12951$$EPDF$$P50$$Gwiley$$H</linktopdf><linktohtml>$$Uhttps://onlinelibrary.wiley.com/doi/full/10.1111%2Fajd.12951$$EHTML$$P50$$Gwiley$$H</linktohtml><link.rule.ids>314,780,784,1417,27924,27925,45574,45575</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/30430555$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Porriño‐Bustamante, María Librada</creatorcontrib><creatorcontrib>López‐Nevot, Miguel Ángel</creatorcontrib><creatorcontrib>Aneiros‐Fernández, José</creatorcontrib><creatorcontrib>García‐Lora, Elena</creatorcontrib><creatorcontrib>Fernández‐Pugnaire, María Antonia</creatorcontrib><creatorcontrib>Arias‐Santiago, Salvador</creatorcontrib><title>Familial frontal fibrosing alopecia: A cross‐sectional study of 20 cases from nine families</title><title>Australasian journal of dermatology</title><addtitle>Australas J Dermatol</addtitle><description>Background/Objectives Frontal fibrosing alopecia (FFA) is a scarring alopecia whose prevalence is increasing. The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported. Material and methods A cross‐sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed. Results Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo‐parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%). Conclusion This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non‐familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.</description><subject>Adult</subject><subject>Age of Onset</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Alopecia</subject><subject>Alopecia - classification</subject><subject>Alopecia - genetics</subject><subject>Alopecia - pathology</subject><subject>Atrophy</subject><subject>Autoimmune diseases</subject><subject>Autoimmunity</subject><subject>cicatricial alopecia</subject><subject>Cross-Sectional Studies</subject><subject>Dermoscopy</subject><subject>Erythema</subject><subject>Erythema - complications</subject><subject>European Continental Ancestry Group</subject><subject>familial</subject><subject>Female</subject><subject>Fibrosis</subject><subject>frontal fibrosing alopecia</subject><subject>Genetic Predisposition to Disease</subject><subject>hair</subject><subject>Hair Follicle - pathology</subject><subject>Hair loss</subject><subject>Humans</subject><subject>Keratosis - complications</subject><subject>Lymphocytes - pathology</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Patients</subject><subject>scarring alopecia</subject><subject>Sebaceous Glands - pathology</subject><subject>Sex Distribution</subject><subject>Spain</subject><issn>0004-8380</issn><issn>1440-0960</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2019</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp1kMtKAzEUhoMotlYXvoAE3Ohi2pPbdOKuVOuFghtdypBJMpIylzrpIN35CD6jT2LaqS4Ez-aHw8cH_4_QKYEhCTdSCzMkVAqyh_qEc4hAxrCP-gDAo4Ql0ENH3i8ACCNCHKIeA85ACNFHLzNVusKpAudNXa026bKm9q56xaqol1Y7dYUnWIef__r49FavXF0Fzq9as8Z1jilgrbz1G0OJK1dZnG-l1h-jg1wV3p7scoCeZzdP07to_nh7P53MI80EI1ECkIOUciyNlWC1ZNwQZZjmiRBKqYzyWCZZZsbZWCVa6PAGRo21LOMmBjZAF5132dRvrfWrtHRe26JQla1bn1LCWEJjiGVAz_-gi7ptQqFAUcphzGhMAnXZUdvejc3TZeNK1axTAulm8zRsnm43D-zZzthmpTW_5M_IARh1wLsr7Pp_Uzp5uO6U3_koinU</recordid><startdate>201905</startdate><enddate>201905</enddate><creator>Porriño‐Bustamante, María Librada</creator><creator>López‐Nevot, Miguel Ángel</creator><creator>Aneiros‐Fernández, José</creator><creator>García‐Lora, Elena</creator><creator>Fernández‐Pugnaire, María Antonia</creator><creator>Arias‐Santiago, Salvador</creator><general>Wiley Subscription Services, Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-3471-4154</orcidid></search><sort><creationdate>201905</creationdate><title>Familial frontal fibrosing alopecia: A cross‐sectional study of 20 cases from nine families</title><author>Porriño‐Bustamante, María Librada ; 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The pathogenesis of this disease is not well known. Genetic, environmental, hormonal and autoimmunity related factors have been considered; however, only a few cases of familial frontal fibrosing alopecia have been reported. Material and methods A cross‐sectional study was performed at University Hospital in Granada (Spain). Twenty patients with frontal fibrosing alopecia belonging to nine different families were included, and clinical and dermoscopic features were analysed. Results Overall, 90% of the patients studied were women (mean age 61.4 years). About 50% of the patients had grade II frontal fibrosing alopecia at the time of diagnosis, whilst 35% had grades III or V. Mean recession was 2.83 cm in the frontal area and 1.99 cm in the temporo‐parietal area. Daughters presented a shorter recession area and earlier debut of the disease than mothers. Androgenetic alopecia was found in only two patients (10%). The dermoscopic signs most commonly found were perifollicular erythema (85%), hyperkeratosis (85%), and absence of vellus hair in the hairline (78.9%). Conclusion This study adds to the growing evidence that there is a genetic component to frontal fibrosing alopecia. The clinical pattern of frontal fibrosing alopecia was not different from that found in non‐familial cases, but the debut of the disease in daughters of mothers with frontal fibrosing alopecia may be earlier.</abstract><cop>Australia</cop><pub>Wiley Subscription Services, Inc</pub><pmid>30430555</pmid><doi>10.1111/ajd.12951</doi><tpages>6</tpages><orcidid>https://orcid.org/0000-0002-3471-4154</orcidid></addata></record>
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subjects Adult
Age of Onset
Aged
Aged, 80 and over
Alopecia
Alopecia - classification
Alopecia - genetics
Alopecia - pathology
Atrophy
Autoimmune diseases
Autoimmunity
cicatricial alopecia
Cross-Sectional Studies
Dermoscopy
Erythema
Erythema - complications
European Continental Ancestry Group
familial
Female
Fibrosis
frontal fibrosing alopecia
Genetic Predisposition to Disease
hair
Hair Follicle - pathology
Hair loss
Humans
Keratosis - complications
Lymphocytes - pathology
Male
Middle Aged
Patients
scarring alopecia
Sebaceous Glands - pathology
Sex Distribution
Spain
title Familial frontal fibrosing alopecia: A cross‐sectional study of 20 cases from nine families
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