Histologic features of two 4-mm punch biopsies in formalin from the left lateral forehead at the hairline and mid-anterior crown showed “scarring alopecia, favor lichen planopilaris” with a comment, “The presence of scattered eosinophils makes lupus less likely.”
Frontal fibrosing alopecia (FFA) is a form of hair loss most often seen in postmenopausal Caucasian women. In a recent multicenter review of 355 patients with FFA, 98.5% were Caucasian, 97% were women, and 86% of the women were postmenopausal (Vañó-Galván et al., 2014). This has led some to suggest an androgen-driven cause hypothesis, but currently, the etiology is unknown. As the name suggests, it is a scarring alopecia. It is considered by most to be a variant of lichen planopilaris (LPP), a perifollicular inflammatory disease. The inflammation is characterized by a cell-mediated immune response that may be considered an autoimmune disease, as it is often associated with other autoimmune diseases such as thyroid abnormalities (Habif, 2010; MacDonald, Clarke, & Holmes, 2012).
The clinical presentation of FFA is characterized by alopecia along the frontal hairline that extends posteriorly with scarring. Patients may complain of an associated pain, stinging, or burning in the affected areas. On examination, perifollicular papules, erythema, and scaling may be present (Habif, 2010). The alopecia may or may not involve the eyebrows, eyelashes, and/or body hair. In a 2012 study of 60 patients with FFA, 73% had eyebrow loss, 25% had loss of body hair, and only 3% had lost eyelashes (MacDonald et al., 2012).
The histology of FFA is the same as LPP: In early stages, there is a lichenoid interface inflammation at the hair follicles and within the perifollicular dermis (Habif, 2010). Later, a perifollicular fibrosis is present (MacDonald et al., 2012). A 2010 histopathological study of 13 patients with FFA showed that the histology of areas of alopecia on the body showed the same features as on the scalp (Chew, Bashir, Wain, Fenton, & Stefanato, 2010).
Unfortunately, there is no consistently effective treatment for FFA. First-line therapy is often corticosteroids either topically, intralesionally, or orally (Habif, 2010). Other medications and medication combinations may be tried such as hydroxychloroquine, topical calcineurin inhibitors, or minoxidil; however, each has inconsistent results (MacDonald et al., 2012). In patients also presenting with androgenic alopecia in one study, finasteride with minoxidil was attempted with no significant improvement (Rallis, Gregoriou, Christofidou, & Rigopoulos, 2010). However, a systematic review found that oral 5-alpha reductase inhibitors had a good clinical response in 45% of patients and were the most effective treatment (Rácz, Gho, Moorman, Noordhoek Hegt, & Neumann, 2013). Currently, it is not obvious whether treatment affects long-term outcomes. A 2009 British study found that, in most cases, the disease stabilized over time regardless of treatment. However, the rate of progression of the disease and the amount of time before the disease was stable were variable (Tan & Messenger, 2009). Many patients eventually focus on camouflage of the disease with wigs (MacDonald et al., 2012).
FFA is an interesting, albeit frustrating, disease for the clinician. Although it can be diagnosed clinically by its location and by its comparable histology with LPP, the origin remains a mystery. The appearance is distressing to patients, and regrettably, there are no reliably effective treatments. Various treatment options may be tried, and eventually, patients may opt to use camouflage such as wigs to disguise the alopecia. In time, most cases will stabilize, with or without treatment. In this presentation of FFA, concomitant start of bioidentical hormones is an intriguing aspect. A review of the literature did not reveal any previous examples of this association at this time. More research is needed not only to determine etiology and treatment for FFA but also to investigate if bioidentical hormone replacement is a factor in progression or a potential therapeutic treatment option.
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: A unifying concept. Journal of the American Academy of Dermatology
, 63 (4), 653–660.
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: A review of 60 cases. Journal of the American Academy of Dermatology
, 67 (5), 955–961.
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, 71 (3), 415.e1–415.e15.
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and lichen planopilaris
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, 27 (12), 1461–1470.
Rallis E., Gregoriou S., Christofidou E., Rigopoulos D. (2010). Frontal fibrosing alopecia
: To treat or not to treat? Journal of Cutaneous Medicine and Surgery
, 14 (4), 161–166.
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, 160 (1), 75–79.
Vañó-Galván S., Molina-Ruiz A. M., Serrano-Falcón C., Arias-Santiago S., Rodrigues-Barata A. R., Garnacho-Saucedo G., Camacho F. M. (2014). Frontal fibrosing alopecia
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Keywords:Copyright © 2015 by the Dermatology Nurses' Association.
Frontal Fibrosing Alopecia; Lichen Planopilaris; Bioidentical Hormones; Autoimmune Alopecia