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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 10  |  Issue : 4  |  Page : 169-171  

Autologous hair transplantation in frontal fibrosing alopecia


1 Department of Dermatology, Santa Casa Hospital, Porto Alegre, Brazil
2 Center for Dermatology and Hair Diseases Professor Trueb, Zurich-Wallisellen, Switzerland

Date of Web Publication9-Oct-2018

Correspondence Address:
Prof. Ralph M Trüeb
Center for Dermatology and Hair Diseases, Bahnhofplatz 1A, CH-8304 Wallisellen
Switzerland
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijt.ijt_37_18

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   Abstract 


We report a patient with frontal fibrosing alopecia (FFA), in whom autologous hair transplantation was successfully performed despite evidence of active disease. Since the underlying pathology of FFA is usually lichen planopilaris, reservations, and caveats have been expressed with respect to the risk of köbnerization phenomena following hair transplantation surgery. An important question that arises is how the lichenoid tissue reaction pattern is generated around the hair follicles in FFA. Follicles with some form of damage or malfunction might express cytokine profiles that attract inflammatory cells to assist in damage repair or in the initiation of apoptosis-mediated organ deletion. Alternatively, an as yet unknown antigenic stimulus from the damaged or malfunctioning hair follicle might initiate a lichenoid tissue reaction in the immunogenetically susceptible individual. Therefore, it might be expected that the transplantation of whole healthy hair follicles might less give rise to an inflammatory reaction than the disease itself, as revealed in our case report of successful hair transplantation in FFA.

Keywords: Frontal fibrosing alopecia, hair transplantation, lichenoid tissue reaction


How to cite this article:
Scribel M, Dutra H, Trüeb RM. Autologous hair transplantation in frontal fibrosing alopecia. Int J Trichol 2018;10:169-71

How to cite this URL:
Scribel M, Dutra H, Trüeb RM. Autologous hair transplantation in frontal fibrosing alopecia. Int J Trichol [serial online] 2018 [cited 2019 May 22];10:169-71. Available from: http://www.ijtrichology.com/text.asp?2018/10/4/169/242921




   Introduction Top


Frontal fibrosing alopecia (FFA) represents a peculiar condition with a quasi-symmetrical, marginal alopecia along the frontal and temporal hairline with scarring. When FFA was first described by Axel Munthe in 1929,[1] it must have been sporadic and ignored, until Kossard's original report in 1994 of six postmenopausal women affected by the condition.[2] Since then, cases have multiplied worldwide exponentially, resulting in FFA eventually representing the most frequent cause of primary scarring alopecia these days. The pattern of hair loss and frequent involvement of eyebrows has a substantial psychological impact on patients, since the high forehead, the receding temporal hairline and the missing eyebrows are reminiscent of the hairstyles of the 15–16th centuries. Indeed, it has been proposed that the Duchess of Urbino may have suffered from the condition[3] while it is more probable that we are dealing with fashionable or cultural forms of frontal pseudoalopecia[4],[5] than a true pathologic condition, since the hair was then shaved around the hairline or plucked at the temples and the napes of the neck, and the condition later disappeared from the arts.

Obviously, there is a need for effective therapy, while the loss of hair is usually permanent, due to a lichen planopilaris (LPP)-like inflammation with fibrosis.[6] Due to the localization of the alopecia, a hair prosthesis is rather inept. Therefore, the last resort is hair transplantation surgery. Since the underlying pathology is usually LPP, reservations and caveats have been expressed with respect to the risk of köbnerization phenomena following hair transplantation surgery. Indeed, the emergence of FFA following both plastic surgery[7] and hair transplantation for androgenetic alopecia[8] has been reported in the literature. A way to circumvent this problem would be to postpone surgery until the disease has been quiescent for a proposed duration of 2 years, another to perform surgery with a short course of oral cyclosporine.[9]

We report a patient with FFA, in whom autologous hair transplantation was successfully performed despite evidence of active disease.


   Case Report Top


A 57-year-old female patient suffering from FFA since 9 years presented for a regular follow-up examination 2 years after successful autologous hair transplantation (follicular unit extraction) at the frontal hairline [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d. Dermoscopic examination revealed evidence of persistent follicular inflammation (perifollicular erythema and casts) at the site of FFA [Figure 2]a, whereas the hair transplants did not [Figure 2]b. The patient was initially treated with 1% pimecrolimus cream b.i.d. along the frontal hairline, and later with dermoscopically guided intralesional triamcinolone acetonide at the sites of active disease, as formerly described.[10],[11]
Figure 1: Frontal fibrosing alopecia (a) before, and (b-d) after autologous hair transplantation (performed by Beatrice Banholzer, Zurich, Switzerland)

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Figure 2: Dermoscopic view on (a) active frontal fibrosing alopecia (perifollicular erythema and follicular casts), and (b) hair transplants without evidence of inflammation despite the active disease

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   Discussion Top


Kossard himself interpreted FFA as a frontal variant of LPP on the basis of his histopathological studies.[6] LPP is regarded to be a T-cell-mediated autoimmune reaction that triggers apoptosis of the follicular epithelial cells. This autoimmune process is thought to be in response to some antigenic challenge, but a specific antigen has yet not been identified. Harries et al.[12] provide the first evidence that LPP may result from an immune privilege collapse of the hair follicle's epithelial stem cell niche. Where a causal or triggering agent is identified, this is termed a lichenoid reaction rather than lichen planus. The etiology of FFA has remained elusive, though some observations may provide clues to its pathogenesis. The predilection for postmenopausal women and the pattern of alopecia have suggested hormonal factors, which so far have not been identifiable. Moreover, the condition has meanwhile also been described in premenopausal women and in men,[13],[14],[15],[16],[17] though with a significantly lesser frequency. The observation of both familial FFA[18],[19],[20] and more recently, connubial FFA[21] suggest both genetic and environmental factors, related to either androgenetic alopecia[22] or to the use of cosmetics and sunscreens,[23],[24] though the latter has been challenged.[25]

An important question that arises is how the lichenoid tissue reaction pattern is generated around the hair follicles in FFA. Follicles with some form of damage or malfunction might express cytokine profiles that attract inflammatory cells to assist in damage repair or in the initiation of apoptosis-mediated organ deletion. Alternatively, an as yet unknown antigenic stimulus from the damaged or malfunctioning hair follicle might initiate a lichenoid tissue reaction in the immunogenetically susceptible individual.[26] Remarkably, in healthy murine skin clusters of perifollicular macrophages have been described as perhaps indicating the existence of a physiological program of immunologically controlled hair follicle degeneration by which malfunctioning follicles are removed by programmed organ deletion.[27] It has been proposed that various forms of clinically perceptible, permanent alopecia might represent pathological exaggeration of this type of programmed organ deletion,[27] resulting in a lichenoid tissue reaction pattern and scarring. Therefore, it might be expected that the transplantation of whole healthy hear follicles might less give rise to an inflammatory reaction than the disease itself, as revealed in our case report of successful hair transplantation in FFA.

In any case, care should be given that the donor area for transplantation is checked for evidence of follicular inflammation,[28] follicular unit test grafting should precede the procedure,[29],[30] and the patient should be carefully followed up posttransplantation, while at the same time active disease in the proximity of the transplants is actively treated, preferably with a topical calcineurin inhibitor[31] that does not impair wound healing.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

This case report represents an integral part of Mariana Scribel's traineeship in Dermato-Trichology in May 2018 at the Center for Dermatology and Hair Diseases Professor Trüeb.



 
   References Top

1.
Trüeb RM. A comment on frontal fibrosing alopecia (Axel Munthe's syndrome). Int J Trichology 2016;8:203-5.  Back to cited text no. 1
    
2.
Kossard S. Postmenopausal frontal fibrosing alopecia. Scarring alopecia in a pattern distribution. Arch Dermatol 1994;130:770-4.  Back to cited text no. 2
    
3.
Serrano-Falcón C, Serrano-Ortega S. Did the duchess of urbino have frontal fibrosing alopecia? Actas Dermosifiliogr 2008;99:737-8.  Back to cited text no. 3
    
4.
Fernandez-Flores A. Frontal pseudoalopecia in history: Part 1 – Fashionable forms. Clin Dermatol 2012;30:548-52.  Back to cited text no. 4
    
5.
Fernandez-Flores A. Frontal pseudoalopecia in history: Part 2-cultural forms. Clin Dermatol 2013;31:131-4.  Back to cited text no. 5
    
6.
Kossard S, Lee MS, Wilkinson B. Postmenopausal frontal fibrosing alopecia: A frontal variant of lichen planopilaris. J Am Acad Dermatol 1997;36:59-66.  Back to cited text no. 6
    
7.
Chiang YZ, Tosti A, Chaudhry IH, Lyne L, Farjo B, Farjo N, et al. Lichen planopilaris following hair transplantation and face-lift surgery. Br J Dermatol 2012;166:666-370.  Back to cited text no. 7
    
8.
Kossard S, Shiell RC. Frontal fibrosing alopecia developing after hair transplantation for androgenetic alopecia. Int J Dermatol 2005;44:321-3.  Back to cited text no. 8
    
9.
Mirmirani P, Willey A, Price VH. Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol 2003;49:667-71.  Back to cited text no. 9
    
10.
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;163:1142-4.  Back to cited text no. 10
    
11.
Trüeb RM, Rezende HD, Diaz MF. Dynamic trichoscopy. JAMA Dermatol 2018. doi: 10.1001/jamadermatol.2018.1175.  Back to cited text no. 11
    
12.
Harries MJ, Meyer K, Chaudhry I, E Kloepper J, Poblet E, Griffiths CE, et al. Lichen planopilaris is characterized by immune privilege collapse of the hair follicle's epithelial stem cell niche. J Pathol 2013;231:236-47.  Back to cited text no. 12
    
13.
Samrao A, Chew AL, Price V. Frontal fibrosing alopecia: A clinical review of 36 patients. Br J Dermatol 2010;163:1296-300.  Back to cited text no. 13
    
14.
MacDonald A, Clark C, Holmes S. Frontal fibrosing alopecia: A review of 60 cases. J Am Acad Dermatol 2012;67:955-61.  Back to cited text no. 14
    
15.
Ladizinski B, Bazakas A, Selim MA, Olsen EA. Frontal fibrosing alopecia: A retrospective review of 19 patients seen at Duke university. J Am Acad Dermatol 2013;68:749-55.  Back to cited text no. 15
    
16.
Banka N, Mubki T, Bunagan MJ, 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;53:1324-30.  Back to cited text no. 16
    
17.
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;70:670-8.  Back to cited text no. 17
    
18.
Junqueira Ribeiro Pereira AF, Vincenzi C, Tosti A. Frontal fibrosing alopecia in two sisters. Br J Dermatol 2010;162:1154-5.  Back to cited text no. 18
    
19.
Dlova N, Goh CL, Tosti A. Familial frontal fibrosing alopecia. Br J Dermatol 2013;168:220-2.  Back to cited text no. 19
    
20.
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;14:64-9.  Back to cited text no. 20
    
21.
da Silva Libório R, Trüeb RM. Case report of connubial frontal fibrosing alopecia. Int J Trichology 2018;10:76-9.  Back to cited text no. 21
    
22.
Gaffney DC, Sinclair RD, Yong-Gee S. Discoid lupus alopecia complicated by frontal fibrosing alopecia on a background of androgenetic alopecia. Br J Dermatol 2013;169:217-8.  Back to cited text no. 22
    
23.
Aldoori N, Dobson K, Holden CR, McDonagh AJ, Harries M, Messenger AG, et al. Frontal fibrosing alopecia: Possible association with leave-on facial skin care products and sunscreens; a questionnaire study. Br J Dermatol 2016;175:762-7.  Back to cited text no. 23
    
24.
Debroy Kidambi A, Dobson K, Holmes S, Carauna D, Del Marmol V, Vujovic A, et al. Frontal fibrosing alopecia in men: An association with facial moisturizers and sunscreens. Br J Dermatol 2017;177:260-1.  Back to cited text no. 24
    
25.
Seegobin SD, Tziotzios C, Stefanato CM, Bhargava K, Fenton DA, McGrath JA, et al. Frontal fibrosing alopecia: There is no statistically significant association with leave-on facial skin care products and sunscreens. Br J Dermatol 2016;175:1407-8.  Back to cited text no. 25
    
26.
Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: Patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch Dermatol 2000;136:205-11.  Back to cited text no. 26
    
27.
Eichmüller S, van der Veen C, Moll I, Hermes B, Hofmann U, Müller-Röver S, et al. Clusters of perifollicular macrophages in normal murine skin: Physiological degeneration of selected hair follicles by programmed organ deletion. J Histochem Cytochem 1998;46:361-70.  Back to cited text no. 27
    
28.
Nirmal B, Somiah S, Sacchidanand SA, Biligi DS, Palo S. Evaluation of perifollicular inflammation of donor area during hair transplantation in androgenetic alopecia and its comparison with controls. Int J Trichology 2013;5:73-6.  Back to cited text no. 28
    
29.
Nusbaum BP, Nusbaum AG. Frontal fibrosing alopecia in a man: Results of follicular unit test grafting. Dermatol Surg 2010;36:959-62.  Back to cited text no. 29
    
30.
Jiménez F, Poblet E. Is hair transplantation indicated in frontal fibrosing alopecia? The results of test grafting in three patients. Dermatol Surg 2013;39:1115-8.  Back to cited text no. 30
    
31.
Katoulis A, Georgala, Bozi E, Papadavid E, Kalogeromitros D, Stavrianeas N, et al. Frontal fibrosing alopecia: Treatment with oral dutasteride and topical pimecrolimus. J Eur Acad Dermatol Venereol 2009;23:580-2.  Back to cited text no. 31
    


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