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.
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.