Original Research

Frontal Fibrosing Alopecia Demographics: A Survey of 29 Patients

Author and Disclosure Information

Frontal fibrosing alopecia (FFA) is a form of scarring alopecia whose diagnosis is increasing globally. Although its etiology is unknown, FFA is thought to be a clinical subset of lichen planopilaris (LPP) that primarily affects postmenopausal women. Patients diagnosed with FFA between January 2006 and December 2013 at clinics of the Washington University Division of Dermatology (St. Louis, Missouri) were studied using patient surveys and chart notes to assess demographics, clinical features, medical history, and treatment. Twenty-nine patients were enrolled in the study, including 28 women and 1 man. The average age of disease onset was 55.4 years (range, 29–75 years). Many patients (55%) had a history of autoimmune diseases, including hypothyroidism (35%), mucocutaneous lichen planus (28%), psoriasis (7%), vitiligo (3%), systemic lupus erythematosus (3%), iritis (3%), Sjögren syndrome (3%), and ulcerative colitis (3%). Patients often identified a stressful inciting event prior to onset of hair loss. Patients tried an average of 3 different treatments for hair loss, with topical and intralesional steroids, hydroxychloroquine, topical calcineurin inhibitors, and excimer laser therapy being the most efficacious at limiting hair loss.

Practice Points

  • Frontal fibrosing alopecia (FFA) may be associated with other autoimmune conditions, and patients should be screened accordingly.
  • The most efficacious treatments for FFA include topical and intralesional steroids, hydroxychloroquine, calcineurin inhibitors, and excimer laser therapy.
  • A stressful precipitating event or metal dental implants/fillings are 2 possible environmental triggers for this condition.


 

References

Frontal fibrosing alopecia (FFA) is a form of lymphocytic cicatricial alopecia that presents as frontotemporal hairline recession, typically in postmenopausal women.1 The condition is considered to be a variant of lichen planopilaris (LPP) due to its similar histologic appearance.2 Loss of eyebrow1-11 and body5-11 hair also is commonly present in FFA, and histologic findings are identical to those for hair loss on the scalp,8,9 suggesting that FFA may be a form of generalized alopecia.

The pathogenesis of FFA is unknown, but several etiologies have been postulated. Some suggest that as a variant of LPP, FFA is a hair-specific autoimmune disorder characterized by a T cell–mediated immune reaction against epithelial hair follicle stem cells, leading to fibrosis and depletion of hair regeneration potential.12 In support of this theory, FFA has been associated with other autoimmune diseases including hypothyroidism,6,8,13-16 mucocutaneous lichen planus,8,15,17 vitiligo,15,18 Sjögren syndrome,19 and lichen sclerosus et atrophicus.15,20 Another hypothesis suggests that the proandrogenic state in postmenopausal women may be related to the disease process.1 This hypothesis is supported by the reported success of antiandrogen therapy with 5α-reductase inhibitors (5α-RIs) in stabilizing FFA.3-5,7 Finally, genetic16,21 and environmental factors related to smoking and socioeconomic status5 also have been postulated to be risk factors for FFA. A variety of treatments have shown varying success, including topical and intralesional corticosteroids, hydroxychloroquine, immunomodulators, antibiotics, and 5α-RIs.1,3-6,8,15,17,22 However, FFA is considered to be relatively difficult to treat and commonly progresses regardless of treatment before spontaneously stabilizing.2-4,6,8,10

Since its discovery in 1994,1 FFA has become increasingly prevalent, comprising 17% of new referrals for hair loss in one study (N=57).6 Although growing recognition of the condition likely plays a role in its increasing presentation, other unidentified factors may contribute to its expanding incidence. In this report, we describe the demographics, clinical features, and disease progression of 29 cases of FFA treated within our division using a series of surveys and chart reviews.

Methods

Upon receiving approval for the project from the institutional review board, we identified 29 patients who met the criteria for diagnosis of FFA through a chart review of all patients being treated for hair loss by clinics within the Washington University Division of Dermatology (St. Louis, Missouri). Diagnostic criteria for FFA included scarring alopecia in the frontotemporal distribution with associated perifollicular erythema or papules and, if performed, a scalp biopsy of the involved area of alopecia showing lymphocytic cicatricial alopecia, compatible with LPP. The diagnosis was confirmed by biopsy in 18 patients (62%), while the remainder of the diagnoses were made clinically. Most biopsy specimens were diagnosed by board-certified dermatopathologists at Washington University, with the remainder diagnosed by outside pathologists if the patient was initially diagnosed at another institution.

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