The Frontal Fibrosing Alopecia Syndrome: How a Single Word Name Change Might Change So Much

Frontal fibrosing alopecia (FFA) was first described in 1994 by dermatologist Dr. Steven Kossard [1]. Since then, there have been intensive worldwide efforts to understand the cause of the condition and how best to treat it. Herein, I propose that we change the name of the condition to frontal fibrosing alopecia syndrome. Although I am convinced that much good would come from this name change for clinicians, researchers, and patients, my sincere hope is that this article will open the much-needed discussion. Many of our well-known medical diseases have gone through one or more name changes. Even FFA was originally named postmenopausal FFA [1]. The adjective postmenopausal was quickly dropped following the realization that premenopausal women—and men—can also be affected. A syndrome is a group of signs and symptoms that occur together and characterize a particular condition. As with many well-known syndromes in medicine, patient characteristics can greatly vary. For example, patients with polycystic ovary syndrome (PCOS) have many shared features, but no two patients are identical. Patients with FFA do not typically share identical features (Table 1). One patient might be premenopausal with facial papules, rosacea, hyperandrogenism, and dry eyes secondary to meibomian gland dysfunction. The next patient might have eyebrow loss, profound forehead skin atrophy, early menopause, and androgen deficiency. Common patterns Linear (type 1), diffuse (type 2) and pseudo-fringe (type 3) L. Trauma-induced FFA The proposed 2018 diagnostic criteria [2] for FFA (Table 2) or the more recent 2021 International FFA Cooperative Group (IFFACG) guidelines recognize the wide variety of presentations that all lead to a final diagnosis of FFA. Over the years, I have come to realize that these criteria work well for most patients with FFA—but not every patient. The 44-year-old female patient whose sister has FFA would herself not meet any of the current FFA criteria when she presents with axillary hair loss, rapid body hair loss, early menopause, facial papules, lichen planus pigmentosus, and lichen sclerosus. Does she still have FFA? According to the Vañó-Galván et al. (2018) criteria or the 2021 IFFACG guidelines, she would not—at least not yet. However, I would argue that she indeed has the features necessary to diagnose the proposed FFA syndrome. Wider recognition of FFA as a syndrome is important—especially as we seek to understand disease pathogenesis fully and capture the diagnosis at the earliest possible stage. Part 1: (2018) Vañó-Galván et al. Frontal Fibrosing Alopecia Diagnostic Criteria. Diagnosis requires 2 major criteria or 1 major criterion and 2 minor criteria. (Modified from Vañó-Galván et al. [2]). Major criteria Minor criteria Much disagreement exists in our community regarding many aspects of FFA diagnosis and treatment. For example, we disagree on how best to treat FFA. A recent article by Vañó-Galván et al. proposing that dutasteride might best be viewed as a first-line treatment for FFA [3] was quickly followed by an opposing article by Holmes et al. [4] outlining why dutasteride should not be considered a first-line option. Our current narrow view of FFA as a single disease entity only adds to the challenges of studying this complex condition. If we were more open to recognizing FFA as a syndrome with extremely varied clinical presentations, we might be more open to the possibility that different treatments might work better in different presentations. A new focus on FFA as a syndrome might open us all to consider the possibility that there might be more than one first-line treatment option for various presentations. For example, it's difficult to argue against the use of isotretinoin as a first-line treatment for FFA associated with prominent facial papules. Isotretinoin, however, might not be an appropriate option for a patient with dry eyes secondary to severe meibomian gland dysfunction or Sjögren's syndrome. Similarly, dutasteride might be considered a reasonable first-line option for many FFA patients but is not an ideal option for a patient with low libido associated with severe androgen deficiency. Our current tunnel view of FFA as a single disease entity influences many practitioners to think first and foremost about what is happening to the hairline and leads many to ignore the 30 or more signs and symptoms associated with this syndrome (Table 1). A new focus on FFA as a syndrome would bring needed attention to these issues. This is important not only in diagnosis but also as we consider how best to define treatment success. In my view, we need to define better what constitutes a successful treatment outcome. The current methods for evaluating treatment success have limitations—and often rely on tools that specifically assess disease activity or severity rather than assess treatment outcomes. For example, our current methodologies for evaluating FFA disease activity (such as the use of the Lichen Planopilaris Activity Index or LPPAI [5]) are quite focused on assessing whether certain hairline parameters “disappear” over multiple follow-up appointments (i.e., itching, redness, positive pull tests) without enough attention as to whether certain parameters “appear” over time—like new hair! We need to consider incorporating an expanded number of clinical parameters beyond a simple assessment limited to the hairline. Imagine two patients using drugs A and B, respectively (Table 2, Part 2). Both have achieved a 65.8% reduction in their LPPAI with the use of these drugs. Should we say drugs A and B are equally effective in treating FFA? It is tempting. But, if I point out that patient B using treatment B achieved an outcome that patient A could not—new hairline and eyebrow growth, fewer facial papules, and a reduced appearance of facial veins—a clear winner emerges. Treatment B is superior, but this could not be captured with the use of an assessment tool like the LPPAI. Tools like the FFA Severity Scale and FFA Severity Index are also not designed to address treatment responses. We need assessment scales that allow us to precisely score improvement in the hairline, eyebrows, eyelashes, and body hair density, as well as improvement in facial papules, lichen planus pigmentosus, cutaneous atrophy, facial veins, and many more outcomes. I suspect that renaming FFA to FFA syndrome will open up a new dialogue between patients and practitioners. The current name captures only a narrow spectrum of the several dozen issues that patients may experience. However, if the word syndrome was added, I would argue that the doors would be opened to a new appreciation of the disease. Many patients with FFA tell me that their stories do not always align with the stories of other FFA patients. In a world where patients with rare diseases like FFA can be connected with ease through various social media platforms, these differences can sometimes be a source of confusion. The addition of the word syndrome adds new understanding as to the reasons why two patients with FFA may not have identical stories. It's time for a new name for FFA—especially one that captures the incredible and ever-expanding array of findings that are part of the disease. While many have proposed that FFA might be renamed lichen planopilaris of Kossard in honor of Dr. Kossard's great contributions, it seems that an even simpler naming revision might do even more to help. Dr. Jeffrey Donovan has received honoraria from Pfizer and Vichy, has participated on advisory boards at Pfizer for payment, participates on the Board of Directors for the Scarring Alopecia Foundation, has received royalties from UpToDate, and is the active Director of the Evidence-Based Hair Training Program.

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