Frontal fibrosing alopecia: Management

INTRODUCTION — Frontal fibrosing alopecia (FFA) is a lymphocytic cicatricial (scarring) alopecia that classically presents with the progressive development of a symmetric, band-like patch of alopecia involving the frontal hairline (picture 1A-D). FFA most frequently occurs in postmenopausal women and is postulated to be a subtype of lichen planopilaris based upon the presence of similar histopathologic findings. (See "Lichen planopilaris".)

Most patients with FFA desire treatment due to the chronic and progressive nature of the disease and the risk for permanent and disfiguring alopecia. However, data on the local and systemic therapies for FFA are limited, and high-quality trials are lacking, precluding conclusions on the best approach to therapy. Moreover, interpretation of the available data is complicated by the frequent use of combination therapy and the potential for spontaneous cessation of hairline recession.

The pathogenesis, clinical manifestations, and diagnosis of FFA are discussed separately. Overviews of lichen planopilaris and the evaluation of patients with hair loss are also provided separately.

●(See "Frontal fibrosing alopecia: Pathogenesis, clinical manifestations, and diagnosis".)

●(See "Lichen planopilaris".)

●(See "Evaluation and diagnosis of hair loss".)

GOALS — The primary goal of treatment is disease stabilization (the cessation of hairline recession and further hair loss). Additional desired effects include the resolution of other signs of disease activity, including perifollicular erythema, perifollicular hyperkeratosis, and associated symptoms. Although some studies have reported regrowth of hair with treatment, regrowth is usually limited or absent due to follicular scarring. (See 'General approach' below.)

ASSESSMENT OF RESPONSE — Different methods have been utilized to assess the response of FFA to treatment.

Assessment for cessation of hairline recession, the primary treatment goal, is essential and can be performed via serial measurements of the distance between the hairline and facial landmarks [1]. Typical landmarks include the glabella and bilateral outer canthi. In our experience, serial clinical photographs are a valuable supplement to measurements, providing visual documentation of disease progression.

The response of other findings suggestive of disease activity can be routinely assessed through the patient history and physical examination. The presence of symptoms (pruritus, burning, or pain) and physical signs of disease activity (perifollicular erythema or scale) should be assessed at follow-up visits.

Scoring systems have also been used to assess severity and response to treatment, primarily in the setting of specialty clinics and clinical studies. Examples include the Lichen Planopilaris Activity Index (LPPAI) and the Frontal Fibrosing Alopecia Severity Score (FFASS). The LPPAI includes assessment of symptoms (pruritus, pain, burning), signs (erythema, perifollicular erythema, perifollicular scale), anagen hair shedding (assessed via hair pull test), and spreading [2]. The FFASS includes assessment of hairline recession, eyebrow hair loss, perifollicular inflammation, pruritus, and pain [3].

WHO TO TREAT — It is generally accepted that treatment is appropriate for patients with signs supportive of active FFA, including progressing hair loss, clinical signs of inflammation (eg, perifollicular erythema or scale), or symptoms (eg, pruritus, burning, or pain).

The best approach to patients without evidence of progressing hair loss, clinical signs of inflammation, or symptoms is less clear because truly inactive disease is considered unlikely to benefit from medical treatments [4]. We typically offer treatment to all patients with FFA at the time of initial presentation because of the potential for subtle, unrecognized disease activity (eg, slow hair loss not evident to the patient).

If an absence of recent progression is confirmed (eg, via review of clinical measurements and clinical reports that support stable disease for at least 12 months) and other signs of active disease are absent, we do not initiate treatment specifically for FFA. Treatment for concomitant disorders that may also cause hair loss, such as androgenic alopecia, can still be initiated, and periodic clinical follow-up should occur to facilitate early detection of recurrences of disease activity.

GENERAL APPROACH — Treatments for FFA include a wide variety of medications that aim to suppress the damaging inflammatory process or that may work through other mechanisms. Because of the paucity of data to guide the treatment of FFA, approaches to treatment differ. Our approach to FFA is reviewed here; other approaches may be reasonable.

Treatment selection — Common initial therapies for FFA include local therapies (topical corticosteroids, topical calcineurin inhibitors, intralesional corticosteroid injections, topical minoxidil) and systemic therapies (oral 5-alpha reductase inhibitors [5-ARIs], hydroxychloroquine, and oral tetracyclines). Rarely, systemic glucocorticoids are prescribed in an attempt to slow rapid hair loss. (See 'Rapidly progressing disease' below.)

Our typical initial treatment regimen consists of the combination of the following:

●A super high- or high-potency topical corticosteroid applied daily

●Intermittent intralesional corticosteroid injections administered approximately every eight weeks

●Topical minoxidil 5% solution or foam applied daily

●Systemic therapy with a 5-ARI, hydroxychloroquine, or a tetracycline-class antibiotic for patients with progressing disease who desire aggressive treatment

Selection of a systemic therapy is based upon consideration of patient-specific contraindications and preferences. We tend to use a 5-ARI as the first systemic therapy for patients without contraindications to this form of therapy. (See 'Systemic therapies' below and '5-alpha reductase inhibitors' below.)

Approaches to the initiation of systemic therapy for FFA differ. Because of the potential for permanent hair loss, we have a low threshold for starting systemic therapy in patients who are experiencing active progression of hair loss and who desire aggressive treatment. Other clinicians opt to delay systemic therapy until there is an inadequate response to local treatment or reserve systemic therapy for patients presenting with certain features, such as prominent hairline recession, prominent perifollicular erythema, or prominent symptoms (eg, pruritus, burning, tenderness) [1,5].

Following stable resolution of signs of disease activity, topical and systemic treatment can be cautiously reduced to the lowest amount of therapy necessary to maintain improvement. (See 'Approach after achievement of stabilization' below.)

Patients who fail to respond well to an initial treatment course or cannot tolerate an initial treatment are typically transitioned to other combinations of the initial therapies. Refractory disease may be treated with oral retinoids, immunosuppressive drugs, or other therapies. (See 'Refractory disease' below.)

Local therapies — Common initial local therapies for FFA include topical corticosteroids, topical calcineurin inhibitors, intralesional corticosteroid injections, and topical minoxidil. Local treatments should be administered to the area of alopecia, adjacent hair-bearing regions, and symptomatic areas.

Topical corticosteroids and calcineurin inhibitors — Topical corticosteroids and topical calcineurin inhibitors (tacrolimus, pimecrolimus) are thought to suppress the damaging inflammatory process in FFA:

●Selection – Topical corticosteroids are often the initial topical immunosuppressants used for FFA, as they are widely available and often less expensive than topical calcineurin inhibitors. However, the potential for corticosteroid-induced skin atrophy can limit long-term use of topical corticosteroids. (See "Topical corticosteroids: Use and adverse effects", section on 'Adverse effects'.)

Topical calcineurin inhibitor use is often begun when patients develop signs of skin atrophy or as a measure to prevent atrophy when long-term, frequent use of topical corticosteroid therapy appears necessary for maintaining disease control. However, some clinicians consider topical calcineurin inhibitors the preferred initial topical immunosuppressants for FFA based upon a desire to avoid risk for corticosteroid-induced skin atrophy in cosmetically sensitive areas [6].

●Administration and efficacy – Topical corticosteroid therapy typically involves the application of a super high-potency or high-potency (groups 1 or 2) topical corticosteroid daily to the affected area (table 1). Daily treatment is continued until hair loss has been stabilized. (See 'Approach after achievement of stabilization' below.)

In our experience, at least partial clinical improvement in disease activity typically occurs within eight weeks. We discontinue treatment after this period if there is no improvement in hair loss or other signs of disease activity.

Similarly, topical calcineurin inhibitors (tacrolimus 0.1% ointment or compounded solution, pimecrolimus 1% cream) are applied daily. Topical calcineurin inhibitors may be used to decrease the frequency of topical corticosteroid use (eg, application on alternating weeks or application of the topical calcineurin inhibitor on weekdays and topical corticosteroid on weekends). Reducing the frequency of topical corticosteroid use may decrease risk for corticosteroid-induced skin atrophy.

Use of topical corticosteroids and topical calcineurin inhibitors is based upon data from retrospective studies and clinical experience [1]. Responses to treatment appear to vary. In one of the largest retrospective studies to evaluate these treatments, combination therapy with clobetasol propionate or betamethasone valerate with pimecrolimus 1% cream was associated with subjective improvement or disease stabilization in 31 of 48 treated patients (65 percent) followed for a median duration of 20 months [7].

●Adverse effects – Cutaneous atrophy, acneiform eruptions, suppression of the hypothalamic-pituitary axis, and other adverse effects of topical corticosteroids are reviewed in greater detail separately. (See "Topical corticosteroids: Use and adverse effects", section on 'Adverse effects'.)

Topical calcineurin inhibitors may be associated with transient burning, erythema, or pruritus after application. Although the US Food and Drug Administration issued warnings in 2005 regarding a possible link between topical calcineurin inhibitor use and cancer, a causal relationship has not been established. (See "Treatment of atopic dermatitis (eczema)".)

Intralesional corticosteroid injections — Intralesional injection of corticosteroids aims to deliver corticosteroids directly into the dermis to suppress perifollicular inflammation:

●Administration and efficacy – Intralesional corticosteroid injections are commonly employed in the treatment of inflammatory cicatricial alopecias. A disadvantage of this intervention is the discomfort associated with treatment. (See "Intralesional corticosteroid injection".)

Intralesional corticosteroid injections for FFA are typically performed with 2.5 to 10 mg/mL of triamcinolone acetonide and performed every four to eight weeks. Injections are placed throughout the area of alopecia and the hair-bearing posterior margin as well as sites demonstrating perifollicular erythema, scale, or symptoms related to FFA (eg, pruritus, burning, tenderness).

We generally treat with 5 or 10 mg/mL triamcinolone acetonide injections every eight weeks. At least partial improvement in disease activity is expected within two to three treatments. In patients who exhibit progressive improvement, we continue treatment until clinical signs of disease activity resolve. If no signs of improvement occur after a few treatments, we discontinue injections. (See 'Approach after achievement of stabilization' below.)

Because the risk for corticosteroid-induced skin atrophy increases with increasing concentrations, some clinicians do not exceed 2.5 mg/mL when treating cosmetically sensitive areas [6]. However, use of 10 mg/mL of triamcinolone acetonide for eyebrow FFA without the development of cutaneous atrophy has been reported [8].

Use of intralesional corticosteroid therapy for FFA is based upon retrospective studies and clinical experience [1]. One of the largest studies included 130 patients with FFA treated with intralesional corticosteroid injections every three to six months for an average of eight treatments [9]. Improvement (any regrowth of hair in the hairline) was reported in 44 patients (34 percent), and stabilization of disease (arrest of hairline recession) was noted in 64 patients (49 percent). Many patients also received other treatments.

●Adverse effects – In addition to skin atrophy, intralesional corticosteroid injections may result in hypopigmentation. Infrequent adverse effects include sterile abscesses and hypothalamic-pituitary axis suppression. (See "Intralesional corticosteroid injection", section on 'Adverse effects and pitfalls'.)

In the event of skin atrophy, intralesional triamcinolone injections should be stopped until the atrophy resolves, which usually takes up to a few months. Limiting the total amount of corticosteroid injected per treatment session may reduce risk for systemic adverse effects. We typically limit the total dose to no more than 20 mg of triamcinolone acetonide per treatment session.

Topical minoxidil — Topical minoxidil is a common treatment for female and male pattern hair loss that is also frequently used in patients with FFA. The mechanism of action for minoxidil is unknown; effects on male and female pattern hair loss are thought to result from prolongation of the anagen (growth) phase of hair follicles, shortening of the telogen (resting) phase, and induction of enlargement of miniaturized follicles [10] (see "Female pattern hair loss (androgenetic alopecia in females): Management", section on 'Topical minoxidil' and "Male pattern hair loss (androgenetic alopecia in males): Management"):

●Administration and efficacy – Although often used, data to confirm the efficacy of minoxidil for FFA are lacking. Proposed rationales for the use of topical minoxidil include cosmetic improvement related to efficacy for coexisting female or male pattern hair loss and the potential for antifibrotic effects of minoxidil to reduce follicular scarring [11]. Some clinicians limit use of minoxidil to patients with evidence of concomitant female or male pattern hair loss [5]. (See "Female pattern hair loss (androgenetic alopecia in females): Pathogenesis, clinical features, and diagnosis", section on 'Clinical manifestations' and "Male pattern hair loss (androgenetic alopecia in males): Pathogenesis, clinical features, and diagnosis", section on 'Clinical features'.)

We typically advise patients to apply minoxidil 5% foam or solution. Women apply minoxidil 5% once daily. Men apply minoxidil 5% twice daily. A noticeable reduction in hair loss is expected within the first four to six months of treatment. Regrowth of hair lost secondary to FFA is not expected. (See 'Approach after achievement of stabilization' below.)

●Adverse effects – Examples of adverse effects of minoxidil include skin irritation manifesting as erythema and pruritus, initial hair shedding, and facial hypertrichosis. (See "Minoxidil (topical): Drug information".)

Systemic therapies — Common initial systemic therapies for FFA include 5-ARIs, hydroxychloroquine, and tetracyclines. There are insufficient data to confirm the efficacy of any of these treatments. The selection of these agents over other systemic drugs used for FFA is based upon reports that suggest benefit and the relatively well-tolerated nature of these therapies.

Infrequently, systemic glucocorticoid therapy is used in an attempt to stabilize rapidly progressive disease. However, the potential for serious adverse effects precludes routine use of this therapy. (See 'Rapidly progressing disease' below.)

General approach — Individuals who are not of childbearing potential may be treated with 5-ARIs, hydroxychloroquine, or a tetracycline. Treatment with 5-ARIs is contraindicated during pregnancy, and caution is indicated for use in patients who may become pregnant. (See '5-alpha reductase inhibitors' below.)

Literature support for benefit of these therapies is primarily limited to retrospective studies, and data are insufficient to confirm efficacy of any of these therapies [1]. Selection among these three interventions is based upon consideration of patient-specific contraindications and preferences.

5-alpha reductase inhibitors — Finasteride and dutasteride are used for FFA, although the mechanism through which 5-ARIs might improve FFA is unclear. Dutasteride inhibits both 5-alpha reductase types 1 and 2, making it a more potent 5-ARI than finasteride, which inhibits only 5-alpha reductase type 2. However, greater efficacy of dutasteride for FFA has not been proven.

Some authors have challenged the use of 5-ARIs for FFA based upon limited evidence for efficacy and the unclear rationale for benefit [12]. It has been proposed that improvement in hair loss may be attributable to positive effects on coexisting androgenetic alopecia:

●Administration and efficacy – Regimens for 5-alpha reductase therapy vary, and the preferred regimens are unclear. Avoidance of 5-ARI therapy during pregnancy is essential. Treatment should not be given to premenopausal individuals of childbearing potential who are unwilling or unable to comply with reliable birth control methods.

Dosing for 5-ARIs varies, and the optimal regimen for FFA is unclear. Examples of initial regimens we utilize include 2.5 to 5 mg per day of finasteride and 0.5 mg per day of dutasteride. We treat for at least four months prior to assessing efficacy (improvement in hair loss) and continue treatment in patients who appear to respond. (See 'Approach after achievement of stabilization' below and 'Refractory disease' below.)

Evidence to support use of 5-ARIs comes from retrospective studies and case reports [13]. In one of the largest studies, a retrospective study that included 111 patients treated with a 5-ARI (often in conjunction with other therapies), treatment with finasteride (2.5 to 5 mg per day) was associated with improvement (any regrowth of hair in the hairline) or stabilization of disease (arrest of hairline recession) in 47 and 53 percent of 102 treated patients, respectively [9]. Among 18 patients given dutasteride (0.5 mg per week), 44 percent had improvement and 56 percent achieved stabilization.

Another retrospective study of 242 females with FFA that included 106 patients treated with only dutasteride (0.5 mg three times per week) and topical clobetasol 0.05% foam (twice weekly) found 28 (37 percent) had achieved stabilization of disease after 12 months [14]. The remaining patients experienced continued recession.

●Adverse effects – Adverse effects of 5-ARIs include abnormalities of genitalia in the male fetus when taken during pregnancy, sexual dysfunction, mood disturbances, and hypotension. Safety data in women are minimal [15]. (See "Finasteride: Drug information" and "Dutasteride: Drug information".)

There is uncertainty regarding the relationship between 5-ARI therapy and high-grade prostate cancer. This is reviewed in detail separately. (See "Chemoprevention strategies in prostate cancer", section on '5-Alpha reductase inhibitors'.)

Hydroxychloroquine — Hydroxychloroquine is postulated to benefit FFA through immunomodulatory properties:

●Administration and efficacy – Hydroxychloroquine may be given at a dose of 200 mg twice daily. However, because the maximum daily dose should not exceed 5 mg/kg to minimize the risk for hydroxychloroquine-induced retinopathy, lower doses are necessary for patients weighing less than 80 kg [16]. (See "Antimalarial drugs in the treatment of rheumatic disease", section on 'Dose adjustment for weight'.)

In our experience, responses to hydroxychloroquine often become evident within the first four months of treatment. If there is no response within this period, we proceed to other treatments. (See 'Approach after achievement of stabilization' below and 'Refractory disease' below.)

Use of hydroxychloroquine is based upon retrospective study data [1]. For example, in a retrospective study that included 54 patients treated with hydroxychloroquine (200 to 400 mg per day) alone or in conjunction with other therapies, 8 (15 percent) improved (any regrowth of hair in the hairline), 32 (59 percent) stabilized (arrest of hairline recession), and 12 (22 percent) worsened (progression of hairline recession) [9].

●Adverse effects – Hydroxychloroquine can cause retinopathy. Baseline and follow-up ophthalmologic examinations are advised for treated patients. (See "Antimalarial drugs in the treatment of rheumatic disease", section on 'Monitoring for toxicity'.)

Examples of additional adverse effects include gastrointestinal distress, skin eruptions, and hematologic toxicity. (See "Antimalarial drugs in the treatment of rheumatic disease", section on 'Adverse effects'.)

Tetracyclines — Benefit of tetracyclines for FFA is postulated to result from anti-inflammatory properties:

●Administration and efficacy – Common treatment regimens for FFA include doxycycline (100 mg twice daily), minocycline (100 mg twice daily), and tetracycline (500 mg twice daily). Initial signs of response to tetracyclines usually become evident within two months. If there is no improvement within four months, we stop treatment and proceed to other therapies. (See 'Approach after achievement of stabilization' below and 'Refractory disease' below.)

Efficacy data on tetracyclines are limited [1]. Treatment with tetracyclines was documented as demonstrating similar efficacy outcomes as treatment with hydroxychloroquine in a retrospective study that included 65 patients given doxycycline, tetracycline, or minocycline and 32 patients treated with hydroxychloroquine; however, the proportion of responders was not reported [17].

●Adverse effects – Tetracyclines, particularly tetracycline and doxycycline, can cause photosensitivity. Examples of additional adverse effects of tetracyclines include gastrointestinal distress, esophagitis, and pseudotumor cerebri. Minocycline may also cause vertigo, skin discoloration, serum sickness, and a lupus-like syndrome. Tetracyclines can cause discoloration of developing teeth and should not be administered to pregnant individuals.

APPROACH AFTER ACHIEVEMENT OF STABILIZATION — Following successful achievement of disease stabilization, treatment can be tapered slowly, as tolerated, usually over several months. We typically do not attempt tapering of systemic therapies until evidence of hair loss progression has been absent for at least four to six months.

Tapering of topical and intralesional therapies typically involves reducing the frequency of application. For example, topical corticosteroid use may be reduced to every-other-day application for eight weeks, followed by application three times weekly. If disease activity does not recur after two to three months of thrice weekly use, treatment can be discontinued. Tapering of systemic therapies involves dose and/or frequency reductions.

Close clinical follow-up should continue during tapering; we typically reassess patients two to three months after treatment reductions. Recurrence of disease activity prompts a return to the previously effective regimen.

RAPIDLY PROGRESSING DISEASE — Oral glucocorticoids are not recommended for the routine treatment of FFA but are infrequently used in an attempt to stabilize rapidly progressive disease (eg, >1 cm hairline recession over a two-month period). Treatment with oral prednisone 40 mg per day for one week and then tapered by 5 mg per week for eight weeks has been suggested by some authors and is an approach that we consider reasonable [1]. Other approaches have also been utilized. In one series, treatment with oral prednisone 25 or 50 mg per day for one month seemed to slow hair loss in two patients with rapid hair loss [18].

Systemic glucocorticoid therapy is associated with risk for a wide variety of serious adverse effects and should be approached with caution. Long-term treatment with systemic glucocorticoids is not advised, and patients should be transitioned to other treatments. (See "Major adverse effects of systemic glucocorticoids" and 'General approach' above.)

REFRACTORY DISEASE — Patients who fail to achieve disease stabilization with initial treatment options may try on other combinations of these therapies. For example, if a patient achieves only a partial response with a 5-alpha reductase inhibitor (5-ARI) given as initial treatment, doxycycline could be added as an adjunctive therapy. If no additional improvement occurs after the addition of doxycycline, doxycycline could be stopped and replaced with hydroxychloroquine. At least partial responses to 5-ARIs, hydroxychloroquine, or tetracyclines are expected within four months; we typically discontinue treatments that do not seem to have any benefit within this period.

The best approach to patients for whom this is insufficient is unclear. In this scenario, we typically discontinue the failed systemic therapies and prescribe an oral retinoid or immunosuppressive therapy. As with other therapies for FFA, efficacy of these interventions is unproven. In our experience, at least partial responses are usually evident within four months in patients who will respond to retinoid and immunosuppressive therapies.

Oral retinoids — Oral retinoids, such as isotretinoin and acitretin, may be beneficial. In one retrospective study, 22 of 29 patients (76 percent) treated with isotretinoin (20 mg per day) and 8 of 11 patients (73 percent) treated with acitretin (20 mg per day) had no further progression of disease after 12 months of treatment, and similar proportions had no progression of disease 8 to 12 months after the discontinuation of treatment [19]. In the same study, 6 of 14 patients (43 percent) treated with finasteride (5 mg per day) achieved these endpoints.

The mechanisms through which retinoids might improve FFA are unclear; anti-inflammatory effects and promotion of normalization of antigen expression of follicular keratinocytes have been proposed [18].

Oral retinoids are teratogenic, and individuals of childbearing potential must avoid pregnancy during treatment. Pregnancy should also be avoided for one month after isotretinoin therapy and for three years after acitretin therapy. (See "Isotretinoin: Drug information" and "Acitretin: Drug information".)

Immunosuppressants — Methotrexate (15 to 25 mg per week) and mycophenolate mofetil (0.5 to 1 g twice daily) are occasionally used for refractory FFA based upon the immunosuppressive effects of these drugs. In one retrospective study, one of three patients treated with methotrexate achieved stabilization of disease [20]. In another retrospective study, one of five patients treated with mycophenolate mofetil responded (achievement of an 85 percent reduction in the Lichen Planopilaris Activity Index [LPPAI] score) and two had partial responses (25 to 85 percent reduction in the LPPAI score) after six months [21].

Methotrexate and mycophenolate mofetil are associated with risk for serious adverse effects. Adverse effects of these agents are reviewed separately. (See "Major side effects of low-dose methotrexate" and "Mycophenolate: Overview of use and adverse effects in the treatment of rheumatic diseases", section on 'Adverse effects'.)

Other interventions — Multiple other agents have been reported as beneficial for FFA. However, limited experience and/or risk for adverse effects preclude recommendations for routine use. Examples include pioglitazone (a peroxisome proliferator-activated receptor-gamma agonist) [22], naltrexone (an opioid antagonist with anti-inflammatory properties) [23], and tofacitinib (a Janus kinase inhibitor) [24]. Pioglitazone has been studied more extensively in lichen planopilaris; however, efficacy of this drug for lichen planopilaris is also unclear. (See "Lichen planopilaris", section on 'Other therapies'.)

Procedural interventions have included treatment with an excimer laser or a carbon dioxide laser [25,26].

USE OF SUNSCREENS — Although sunscreen use has been proposed as a risk factor for FFA, data are insufficient to confirm a causal relationship. Given the insufficient evidence and the known benefits of sunscreen use for skin cancer prevention, we do not advise our patients to cease use of sunscreens [27]. (See "Frontal fibrosing alopecia: Pathogenesis, clinical manifestations, and diagnosis", section on 'Pathogenesis'.)

Hair regrowth in a patient with a clinical diagnosis of FFA after cessation of sunscreen use on the forehead has been reported [28]. The patient was also receiving other treatments.

COSMETIC MEASURES

Nonsurgical measures — Some patients with hair loss secondary to FFA may desire options for camouflaging hair loss, such as hairpieces, wigs, and other products. Referral to a cosmetologist or other professional experienced with such products and techniques can be beneficial for these individuals.

Hair transplantation — Hair transplantation for the purpose of restoring hair in areas in patients with FFA should be approached with caution because of risk for poor long-term outcomes [29,30]. Although a retrospective study of 51 patients with FFA who underwent hair transplantation found that 42 (82 percent) reported satisfaction with the procedure, mean graft survival rates after one, two, three, and five years were 87 percent (n = 51), 71 percent (n = 51), 60 percent (n = 38), and 41 percent (n = 12), respectively [29]. The development of FFA following hair transplantation and face lift procedures has also been reported [31].

The optimal time for performance of hair transplantation is unclear. In general, hair transplantation is not advised for patients with cicatricial alopecia that has not been stabilized for at least one year because of the potential for the loss of transplanted hair upon reactivation of disease [32,33]. In addition, medical therapy for FFA is typically continued after hair transplantation in an attempt to minimize risk for recurrence of disease activity. Medical therapy can be tapered gradually as stability is maintained after the transplant procedure. Intermittent (eg, twice weekly) use of a high-potency topical corticosteroid or use of a topical calcineurin inhibitor has been suggested [34].

PROGNOSIS — Data on the course of FFA without treatment are limited, and the course appears to vary among patients. The general course appears to involve a period of progressive hair loss followed by spontaneous disease stabilization after a widely variable period of time [1,35]. The author's experience suggests that disease activity for a period of around five years may be common.

Although partial hair regrowth has been reported with treatment, it is generally not expected given the cicatrizing nature of FFA. (See 'Goals' above.)

Prognostic factors for FFA are not established. The findings of a retrospective study that included 106 women with FFA treated with dutasteride and topical corticosteroids suggest that the pattern of hair loss may be an indicator of prognosis [14]. Eyebrow loss, eyelash loss, and facial papules are additional proposed poor prognostic factors [36].

FOLLOW-UP — Patients with FFA may benefit from close follow-up to assess responses to treatment. We typically see patients every 6 to 12 weeks until the disease is stabilized for at least 12 months. Subsequent clinical evaluations may occur every 6 to 12 months provided the patients remain asymptomatic and without sigs of progression of hair loss. More frequent evaluation may be necessary for patients continuing systemic maintenance treatments that require monitoring. Patients with stabilized disease who no longer require treatment can be instructed to return for re-evaluation if they experience recurrence of symptoms or hair loss.

RESOURCES FOR SUPPORT — Hair loss secondary to FFA can be a significant psychologic stressor. Patients may benefit from access to support resources, such as the Cicatricial Alopecia Research Foundation.

SUMMARY AND RECOMMENDATIONS

●Frontal fibrosing alopecia (FFA) is a lymphocytic cicatricial (scarring) alopecia that classically presents with progressive regression of the frontal hairline (picture 1A-D). FFA may represent a subtype of lichen planopilaris. (See 'Introduction' above.)

●The primary goal of treatment of FFA is disease stabilization (cessation of progression of hair loss). Additional beneficial effects include resolution of other signs of disease activity, including symptoms (pruritus, pain, burning) and perifollicular erythema and scale. Regrowth of hair lost secondary to FFA is not expected. (See 'Goals' above.)

●Assessment for disease stabilization can be performed through serial measurements of hairline recession and clinical photographs. The response of other findings suggestive of disease activity can be assessed through the patient history and physical examination. (See 'Assessment of response' above.)

●For patients with FFA and active progression of hair loss or other findings suggestive of active disease (symptoms or perifollicular erythema or scale), we suggest treatment rather than observation (Grade 2C). For asymptomatic patients with a confirmed absence of recent progression of hair loss (eg, clinical documentation supportive of stable disease for at least 12 months) who also lack other findings suggestive of active disease, we suggest not initiating treatment specifically for FFA (Grade 2C). Treatment for concomitant disorders that may also cause hair loss, such as androgenic alopecia, can still be initiated. Close clinical follow-up for recurrence of disease activity should be implemented. (See 'Who to treat' above.)

●For patients with active progression of hair loss, we suggest a combination of local and systemic treatment rather than local treatment alone (Grade 2C). Our typical initial regimen includes a high-potency topical corticosteroid, periodic intralesional corticosteroid injections, topical minoxidil, and a systemic therapy (5-alpha reductase inhibitor [5-ARI], hydroxychloroquine, or tetracycline class antibiotic). Selection among these systemic therapies is based upon consideration of patient-specific contraindications and preferences. Patients presenting with rapid progression of hair loss may benefit from a course of systemic glucocorticoid therapy. (See 'General approach' above and 'Rapidly progressing disease' above.)

●For patients who continue to have progression of hair loss despite standard systemic treatments (5-ARI, hydroxychloroquine, and/or tetracycline class antibiotics), we suggest oral retinoid or immunosuppressive therapy (Grade 2C). Common initial options include acitretin, isotretinoin, methotrexate, and mycophenolate mofetil. Other treatments have also been utilized. (See 'Refractory disease' above.)

●Sunscreen use has been proposed as a risk factor for FFA; however, data are insufficient to confirm a causal relationship. We suggest that patients with FFA do not avoid sunscreen use (Grade 2C). (See 'Use of sunscreens' above.)

●Hair transplantation has been utilized in an attempt to restore hair in areas of permanent alopecia. Hair transplantation for FFA should be approached with caution. In general, hair transplantation is not advised for patients with FFA that has not been stabilized for at least one year.