Frontal fibrosing alopecia: state of the art and future directions

Authors

  • Joana R. Lascasas Family Medicine UCSP Caminha, Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal http://orcid.org/0000-0003-3974-9636
  • Joana S. Peixoto Family Medicine USF Mais Saúde, Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal
  • Sofia C. Vedor Aesthetical Medicine, Centro Internacional de Estudios de Postgrado (CIEP), Distance University of Madrid, Cordoba, Spain

DOI:

https://doi.org/10.18203/issn.2455-4529.IntJResDermatol20204575

Keywords:

Alopecia, Lichen planopilaris, Follicular lichen planus, Scarring alopecia, Lymphocytic cicatricial alopecia

Abstract

Frontal fibrosing alopecia (FFA) is a primary lymphocytic cicatricial alopecia, characterized by recession of frontotemporal hairline with frequent involvement of eyebrows and affecting occasionally other body areas. Entitled an emerging epidemic, due to its rising incidence, FFA etiology remains unclear without proven effective therapies. This study reviews relevant publications on FFA, regarding pathogenesis, clinical findings, histology along with treatment and prognosis. A bibliographic search was conducted in the main international databases, using the term ‘FFA’. Guidelines, observational studies, randomized controlled trials, reviews, systematic reviews and meta-analysis regardless of publication date, presented in English, Portuguese or Spanish, were included in this review. 50 publications were fully analysed. The majority of FFA cases were observed in postmenopausal women, although both men and women from younger ages can be victims. From an etiological point of view, immune-mediated hypothesis is widely spread, as stem cells localized in the bulge region of the follicle are destroyed by an inflammatory infiltrate. However, environmental factors raised interest, since sunscreen use was related to a bigger incidence of FFA. Three clinical patterns have been described. Yet the failure to use standardized assessment methods in addition to the absence of prospective studies evaluating available therapies, do not concede comparable data. Spontaneous stabilization of disease can occur, but regrowth was only seen in scarce cases. Outcomes from randomized controlled trials and prospective studies are missing to draw further understanding of FFA.

Author Biographies

Joana R. Lascasas, Family Medicine UCSP Caminha, Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal

Integrated Master in Medicine at the Faculty of Medicine of University of Porto, Porto, Portugal (2010 - 2016)

General Training at Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal (2017) 

Internship Family Medicine UCSP Caminha, Unidade Local de Saúde do Alto Minho (2018 - now)

Postgraduation Student of Aesthetical Medicine, Centro Internacional de Estudios de Postgrado (CIEP), Cordoba
Distance University of Madrid, Spain (2019 - 2020)

7th Edition of the Advanced Aesthetical Course - Fillers and Botulinum Toxin, promoted by the Portuguese Association of Aesthetics, Porto, Portugal (july/ 2019)

Toxin Botulinum Course, promoted by the Portuguese Society of Aesthetical Medicine and Cosmetic Surgery, Hospital Escola Fernando Pessoa, Porto, Portugal (april/ 2019) 

Joana S. Peixoto, Family Medicine USF Mais Saúde, Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal

Integrated Master in Medicine at the Faculty of Medicine of University of Porto, Porto, Portugal (2010 - 2016)

General Training at Unidade Local de Saúde do Alto Minho, Viana do Castelo, Portugal (2017) 

Internship Family Medicine UCSP Caminha, Unidade Local de Saúde do Alto Minho (2018 - now)

Postgraduation Student of Aesthetical Medicine, Centro Internacional de Estudios de Postgrado (CIEP), Cordoba
Distance University of Madrid, Spain (2019 - 2020)

Toxin Botulinum Course, promoted by the Portuguese Society of Aesthetical Medicine and Cosmetic Surgery, Hospital Escola Fernando Pessoa, Porto, Portugal (april/ 2019) 

Sofia C. Vedor, Aesthetical Medicine, Centro Internacional de Estudios de Postgrado (CIEP), Distance University of Madrid, Cordoba, Spain

Integrated Master in Medicine at the Faculty of Medicine of University of Coimbra, Coimbra, Portugal (2010 - 2016)

General Training at Centro Hospitalar Universitário de Coimbra, Coimbra, Portugal (2017) 

Postgraduation Student of Aesthetical Medicine, Centro Internacional de Estudios de Postgrado (CIEP), Cordoba
Distance University of Madrid, Spain (2019 - 2020)

7th Edition of the Advanced Aesthetical Course - Fillers and Botulinum Toxin, promoted by the Portuguese Association of Aesthetics, Porto, Portugal (july/ 2019)

Toxin Botulinum Course, promoted by the Portuguese Society of Aesthetical Medicine and Cosmetic Surgery, Hospital Escola Fernando Pessoa, Porto, Portugal (april/ 2019) 

References

Iorizzo M, Tosti A. Frontal Fibrosing Alopecia: An Update on Pathogenesis, Diagnosis, and Treatment. Am J Clin Dermatol. 2019;20(3):379-390.

Mirmirani P, Tosti A, Goldberg L, Whiting D, Sotoodian B. Frontal Fibrosing Alopecia: An Emerging Epidemic. Ski Appendage Disord. 2019;5(2):90-93.

Aldoori N, Dobson K, Holden CR, McDonagh AJ, Harries M, Messenger AG. Frontal fibrosing alopecia: possible association with leave-on facial skin care products and sunscreens; a questionnaire study. Br J Dermatol. 2016;175(4):762-7.

Ma SA, Imadojemu S, Beer K, Seykora JT. Inflammatory features of frontal fibrosing alopecia. J Cutan Pathol. 2017;44(8):672-6.

Ramot Y, Bertolini M, Boboljova M, Uchida Y, Paus R. PPAR‐γ signalling as a key mediator of human hair follicle physiology and pathology. Exp Dermatol. 2020;29(3):312-21.

Gaspar NK. DHEA and frontal fibrosing alopecia: molecular and physiopathological mechanisms. An Bras Dermatol. 2016;91(6):776-80.

Karnik P, Tekeste Z, McCormick TS, Gilliam AC, Price VH, Cooper KD et al. Hair Follicle Stem Cell-Specific PPARγ Deletion Causes Scarring Alopecia. J Invest Dermatol. 2009;129(5):1243-57.

Robinson G, McMichael A, Wang SQ, Lim HW. Sunscreen and frontal fibrosing alopecia: A review. J Am Acad Dermatol. 2020;82(3):723-8.

Tavakolpour S, Mahmoudi H, Abedini R, Kamyab Hesari K, Kiani A, Daneshpazhooh M. Frontal fibrosing alopecia: An update on the hypothesis of pathogenesis and treatment. Int J Women’s Dermatol. 2019;5(2):116-23.

Mendoza-Milla C, Valero Jiménez A, Rangel C, Lozano A, Morales V, Becerril C et al. Dehydroepiandrosterone has strong antifibrotic effects and is decreased in idiopathic pulmonary fibrosis. Eur Respir J. 2013;42(5):1309-21.

Garcia-Robledo JE, Aragón CC, Nieto-Aristizábal I, Vásquez S, Montoya C, Tobón GJ. Frontal fibrosing alopecia: A new autoimmune entity? Med Hypotheses. 2019;124:13-6.

Vañó-Galván S, Molina-Ruiz AM, Serrano-Falcón C, Arias-Santiago S, Rodrigues-Barata AR, Garnacho-Saucedo G et al. Frontal fibrosing alopecia: A multicenter review of 355 patients. J Am Acad Dermatol. 2014;70(4):670-8.

Navarro-Belmonte MR, Navarro-López V, Ramírez-Boscà A, Martínez-Andrés MA, Molina-Gil C, González-Nebreda M et al. Case series of familial frontal fibrosing alopecia and a review of the literature. J Cosmet Dermatol. 2015;14(1):64-9.

Tziotzios C, Stefanato CM, Fenton DA, Simpson MA, McGrath JA. Frontal fibrosing alopecia: reflections and hypotheses on aetiology and pathogenesis. Exp Dermatol. 2016;25(11):847-52.

Zouboulis CC, Boschnakow A. Chronological ageing and photoageing of the human sebaceous gland. Clin Exp Dermatol. 2001;26(7):600-7.

Bolduc C, Sperling LC, Shapiro J. Primary cicatricial alopecia. J Am Acad Dermatol. 2016;75(6):1081-99.

da Silva Libório R, Trüeb R. Case report of connubial frontal fibrosing alopecia. Int J Trichol. 2018;10(2):76.

Moreno-Arrones OM, Saceda-Corralo D, Fonda-Pascual P, Rodrigues-Barata AR, Buendía-Castaño D, Alegre-Sánchez A et al. Frontal fibrosing alopecia: clinical and prognostic classification. J Eur Acad Dermatol Venereol. 2017;31(10):1739-45.

Lacarrubba F, Micali G, Tosti A. Absence of vellus hair in the hairline: a videodermatoscopic feature of frontal fibrosing alopecia. Br J Dermatol. 2013;169(2):473-4.

MacDonald A, Clark C, Holmes S. Frontal fibrosing alopecia: A review of 60 cases. J Am Acad Dermatol. 2012;67(5):955-61.

Nanda S, De Bedout V, Hirt PA, Castillo DE, Mesquita T, Scott L et al. Increased Preauricular Wrinkles in Frontal Fibrosing Alopecia Compared to Age-Matched Controls: A Prospective Study of 64 Patients. Ski Appendage Disord. 2020;6(1):11-3.

Kanti V, Constantinou A, Reygagne P, Vogt A, Kottner J, Blume‐Peytavi U. Frontal fibrosing alopecia: demographic and clinical characteristics of 490 cases. J Eur Acad Dermatol Venereol. 2019;33(10):1976-83.

To D, Beecker J. Frontal Fibrosing Alopecia: Update and Review of Challenges and Successes. J Cutan Med Surg. 2018;22(2):182-9.

Doche I, Romiti R, Hordinsky MK, Valente NS. “Normal‐appearing” scalp areas are also affected in lichen planopilaris and frontal fibrosing alopecia: An observational histopathologic study of 40 patients. Exp Dermatol. 2020;29(3):278-81.

Starace M, Brandi N, Alessandrini A, Bruni F, Piraccini BM. Frontal fibrosing alopecia: a case series of 65 patients seen in a single Italian centre. J Eur Acad Dermatol Venereol. 2018:jdv.15372.

Karadag Köse Ö, Güleç AT. Evaluation of a Handheld Dermatoscope in Clinical Diagnosis of Primary Cicatricial Alopecias. Dermatol Ther (Heidelb). 2019;9(3):525-35.

Wong D, Goldberg LJ. The depth of inflammation in frontal fibrosing alopecia and lichen planopilaris: A potential distinguishing feature. J Am Acad Dermatol. 2017;76(6):1183-4.

Donati A, Gupta AK, Jacob C, Cavelier-Balloy B, Reygagne P. The Use of Direct Immunofluorescence in Frontal Fibrosing Alopecia. Ski Appendage Disord. 2017;3(3):125-8.

Vazquez-Herrera NE, Eber AE, Martinez-Velasco MA, Perper M, Cervantes J, Verne SH et al. Optical coherence tomography for the investigation of frontal fibrosing alopecia. J Eur Acad Dermatol Venereol. 2018;32(2):318-22.

Esteban-Lucía L, Molina-Ruiz AM, Requena L. Actualización en alopecia frontal fibrosante. Actas Dermosifiliogr. 2017;108(4):293-304.

Samrao A, Chew A-L, Price V. Frontal fibrosing alopecia: a clinical review of 36 patients. Br J Dermatol. 2010;163(6):1296-1300.

Saceda-Corralo D, Moreno-Arrones ÓM, Fonda-Pascual P, Pindado-Ortega C, Buendía-Castaño D, Alegre-Sánchez A et al. Development and validation of the Frontal Fibrosing Alopecia Severity Score. J Am Acad Dermatol. 2018;78(3):522-9.

Holmes S, Ryan T, Young D, Harries M. Frontal Fibrosing Alopecia Severity Index FFASI: a validated scoring system for assessing frontal fibrosing alopecia. Br J Dermatol. 2016;175(1):203-07.

Saceda-Corralo D, Moreno-Arrones OM, Fonda-Pascual P, Alegre-Sánchez A, Vañó-Galván S. Reply to: ‘Frontal Fibrosing Alopecia Severity Index (FFASI): a validated scoring system for assessing frontal fibrosing alopecia.’ Br J Dermatol. 2016;175(3):648.

Martínez-Velasco MA, Vázquez-Herrera NE, Misciali C, Vincenzi C, Maddy AJ, Asz-Sigall D et al. Frontal Fibrosing Alopecia Severity Index: A Trichoscopic Visual Scale That Correlates Thickness of Peripilar Casts with Severity of Inflammatory Changes at Pathology. Ski Appendage Disord. 2018;4(4):277-80.

Rácz E, Gho C, Moorman PW, Noordhoek Hegt V, Neumann HAM. Treatment of frontal fibrosing alopecia and lichen planopilaris: a systematic review. J Eur Acad Dermatol Venereol. 2013;27(12):1461-70.

Saceda-Corralo D, Moreno-Arrones ÓM, Fonda-Pascual P, Pindado-Ortega C, Hermosa-Gelbard Á, Rodrigues-Barata AR et al. Steroid-induced changes noted on trichoscopy of patients with frontal fibrosing alopecia. J Am Acad Dermatol. 2018;79(5):956-7.

Strazzulla LC, Avila L, Li X, Lo Sicco K, Shapiro J. Prognosis, treatment, and disease outcomes in frontal fibrosing alopecia: A retrospective review of 92 cases. J Am Acad Dermatol. 2018;78(1):203-5.

Gkini M-A, Riaz R, Jolliffe V. A retrospective analysis of efficacy and safety of intralesional triamcinolone injections in the treatment of frontal fibrosing alopecia either as monotherapy or as a concomitant therapy. Int J Trichol. 2018;10(4):162.

Donovan JC, Samrao A, Ruben BS, Price VH. Eyebrow regrowth in patients with frontal fibrosing alopecia treated with intralesional triamcinolone acetonide. Br J Dermatol. 2010;163(5):1142-44.

Banka N, Mubki T, Bunagan MJK, McElwee K, Shapiro J. Frontal fibrosing alopecia: a retrospective clinical review of 62 patients with treatment outcome and long-term follow-up. Int J Dermatol. 2014;53(11):1324-30.

Mahmoudi H, Rostami A, Tavakolpour S, Nili A, Teimourpour A, Salehi Farid A et al. Oral isotretinoin combined with topical clobetasol 0.05% and tacrolimus 0.1% for the treatment of frontal fibrosing alopecia: a randomized controlled trial. J Dermatolog Treat. 2020:1-7.

Rakowska A, Agnieszka G, Olszewska M, Rudnicka L. Efficacy of Isotretinoin and Acitretin in Treatment of Frontal Fibrosing Alopecia: Retrospective Analysis of 54 Cases. J Drugs Dermatol. 2017;(16):988-92.

Murad A, Bergfeld W. 5-alpha-reductase inhibitor treatment for frontal fibrosing alopecia: an evidence-based treatment update. J Eur Acad Dermatol Venereol. 2018;32(8):1385-90.

Chiang C, Sah D, Cho BK, Ochoa BE, Price VH. Hydroxychloroquine and lichen planopilaris: Efficacy and introduction of Lichen Planopilaris Activity Index scoring system. J Am Acad Dermatol. 2010;62(3):387-92.

Marmor MF, Kellner U, Lai TYY, Melles RB, Mieler WF. Recommendations on Screening for Chloroquine and Hydroxychloroquine Retinopathy (2016 Revision). Ophthalmol. 2016;123(6):1386-94.

Strazzulla LC, Avila L, Lo Sicco K, Shapiro J. Novel Treatment Using Low-Dose Naltrexone for Lichen Planopilaris. J Drugs Dermatol. 2017;16(11):1140-2.

Yang CC, Khanna T, Sallee B, Christiano AM, Bordone LA. Tofacitinib for the treatment of lichen planopilaris: A case series. Dermatol Ther. 2018;31(6):e12656.

Plante J, Eason C, Snyder A, Elston D. Tofacitinib in the treatment of lichen planopilaris: a retrospective review. J Am Acad Dermatol. 2020:S0190-9622(20)30979.

Jiménez F, Poblet E. Is Hair Transplantation Indicated in Frontal Fibrosing Alopecia? The Results of Test Grafting in Three Patients. Dermatol Surg. 2013;39(7):1115-8.

Downloads

Published

2020-10-22

Issue

Section

Review Articles