ABSTRACT
Frontal fibrosing alopecia (FFA) is a patterned primary cicatricial alopecia that was first described in 1994. Once rare, the incidence of FFA has increased dramatically, representing the current most common cause of cicatricial alopecia worldwide. FFA typically begins in postmenopausal women with symmetrical, progressive recession of the frontotemporal hairline together with bilateral loss of the eyebrows. FFA has a distinctive clinical phenotype, which remains a challenge. The histology is identical to lichen planopilaris (LPP), but only a small number of patients have coincidental LPP, usually of the scalp. The vast majority of patients have no evidence of lichen planus elsewhere, and the symmetry and patterned nature of the hair loss are unusual for LPP. Familial cases of FFA are reported, and gene associations have been identified in population studies; however, the pathophysiology remains controversial. Without treatment, FFA is slowly progressive, and although many treatments have been prescribed, the response is often disappointing. We review the pathogenesis, epidemiology, clinical features, histology, and treatment of FFA.