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Frontal fibrosing alopecia: Bewitched, bothered, and bewildered


DII small banner By Warren R. Heymann, MD
Feb. 13, 2017


frontal fibrosing alopecia
Women given the diagnosis of frontal fibrosing alopecia (FFA) with different severity grades. A, FFA grade I/V in a premenopausal 30-year-old woman, with a less than 1-cm wide area of cicatricial skin produced by the recession of the frontal and temporal hairline. B, FFA grade II/V in a postmenopausal 63-year-old woman, with a 2-cm wide area of cicatricial skin produced by the recession of the frontal hairline, associated with total eyebrow loss. C, FFA grade III/V in a postmenopausal 61-year-old woman, with a 4-cm wide area of cicatricial skin produced by the recession of the frontal and temporal hairline, associated with partial eyebrow loss. D, FFA V/V (“clown alopecia”) in a postmenopausal 77-year-old woman, with a more than 7-cm wide area of cicatricial skin produced by the recession of the frontal and temporal hairline.
Credit: JAAD
It is not your imagination — undoubtedly you have made the diagnosis of frontal fibrosing alopecia (FFA) more frequently in recent years. FFA is considered a subtype of lichen planopilaris (LPP) based on the identical histology of a lymphocytic cicatrical alopecia localizing near the infundibulum and isthmus (at the hair bulge). It is seen predominantly, but not exclusively, in postmenopausal women. The characteristic presentation is a progressive frontotemporal hairline recession with perifollicular erythema and hyperkeratosis, accompanied by symptoms of pruritus and burning. Progressive loss of the eyebrows is often associated with the disease, as is loss of body hair (1). FFA is frequently accompanied by atrophy within the frontal hairline (2). In a study of 355 patients with FFA, androgenetic alopecia (AGA) was observed in 136 of 343 women (40%) and 8 of 12 men (67%) (3).

The pathogenesis of FFA remains an enigma. There may be a genetic predisposition, as familial cases have been reported, demonstrating an autosomal dominant pattern with incomplete penetrance. Environmental factors such as sunscreens and moisturizers have been implicated (4). Because of the perimenopausal time of onset, hormonal influences have been investigated. In a study of 168 patients with LPP, FFA, or LPP/FAA overlap, androgen excess was identified in 31.5% (n = 53) of the 168 patients with LPP and all subtypes (P < .001). Interestingly, androgen deficiency was identified in 32.1% (n = 17) of the 53 patients with FFA (P < .001) (5). Ultimately, the inflammatory response comes down to proinflammatory cytokines such as interferons, with an increased apoptotic response, and collapse of the relative immune privilege of the hair follicle (1). Looking forward, Tziotzios et al state: “Identifying a refined genetic or protein micro-entity as a biomarker would be indispensable to disease prognostication and could help optimize therapeutic strategies: circulating microRNA and metabolomic profiling may also be an appropriate approach but may be challenging to validate, given the slowly progressive nature of FFA requiring a prolonged and carefully controlled study.” (6)

Therapeutically, virtually every approach taken for LPP has been utilized for FFA (intralesional steroids, topical steroids, systemic steroids, retinoids, antimalarials, tetracyclines, mycophenolate mofetil, cyclosporine, tacrolimus, thalidomide, laser, pioglitazone), however, it is the 5 alpha-reductase inhibitors (finasteride and dutasteride) that have been considered most effective in stabilizing, and even improving, FFA. In a review of 114 patients with FFA, a good response was noted in 45% of those receiving the 5 alpha-reductase inhibitors and in 30% of those treated with hydroxycholoroquine (1). Although one can question if it just improving an associated AGA, if you look at a case such as Donovan’s (2), you might think otherwise. If the study by Ranasinghe et al (5) noting androgen deficiency in FFA is confirmed, the utility of 5 alpha-reductase inhibitors would raise even more questions about their mechanism of action in FFA, as one would consider them to be efficacious in the context of androgen excess.

I am bewitched by FFA’s increasing incidence, patients are bothered by their appearance, and all are bewildered by understanding its pathomechanism, which we need to decipher to develop optimal therapy. I look forward to hearing our patients sing “Happy Days Are Here Again!”

1. Rácz E, et al. Treatment of frontal fibrosing alopecia and lichen planopilaris.: A systematic review. J Eur Acad Dermatol Venereol 2013; 27: 1461-70.
2. Donovan JC. Finasteride-mediated hair regrowth and reversal of atrophy in a patient with frontal fibrosing alopecia. JAAD Case Reports 2015; 353-5.
3. Vañó- Galván S, et al. Frontal fibrosing alopecia: A multicenter review of 355 patients. J Am Acad Dermatol 2014; 70: 670-8.
4. Debroy-Kidambi A, et al. Frontal fibrosing alopecia in men – an association with facial moisturizers and sunscreens. Br J Dermatol 2017 Jan 23 [Epub ahead of print].
5. Ranasinghe GC, et al. Prevalence of hormonal and endocrine dysfunction in patients with lichen planopilaris (LPP): A retrospective data analysis of 168 patients. J Am Acad Dermatol 2017; 76: 314-20.
6. Tziotzios C, et al. Frontal fibrosing alopecia: Reflections and hypotheses an aetiology and pathogenesis. Exp Dermatol 2016; 25: 847-52.

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