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

1. Introduction

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.

2. Name Changes Are an Expected Part of a Refined Understanding of Diseases

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.

3. FFA Is a Syndrome—With Remarkably Varied Presentations

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.

TABLE 1.

Entities comprising the proposed frontal fibrosing alopecia syndrome (FFA Syndrome).

FFA variants
Classic FFA Grades 1‐5 (severity) Common patterns ○ Linear (type 1), diffuse (type 2) and pseudo‐fringe (type 3)
B Uncommon/Atypical FFA variants ○ Androgenetic Alopecia‐like ○ Cockade‐like pattern ○ Clown Alopecia pattern ○ Occipital patterns (ophiasis‐like, lateral, patchy, pseudo‐fringe) ○ Upsilon pattern ○ Pustular
C FFA with associated cutaneous or nail changes FFA associated with classic scalp lichen planopilaris FFA associated with fibrosing alopecia in a pattern distribution FFA associated with Graham‐Little‐Piccardi‐Lassueur Syndrome FFA associated with facial papules FFA associated with nuchal papules and ectatic veins FFA associated with severe cutaneous atrophy/depression of the frontal veins FFA associated with rosacea FFA associated with follicular red dots (glabella/ forehead, eyebrows, hips, cheeks, chest) FFA associated with androgenetic alopecia FFA associated with irritant/allergic contact dermatitis FFA associated with psoriasis/seborrheic dermatitis FFA associated with excessive facial sweating FFA associated with lichen planus pigmentosus FFA associated with reversal of canities FFA associated with cutaneous lichen planus/ FFA associated with nail lichen planus FFA associated with atopy
D FFA initially presenting as isolated non‐scalp hair loss presentations FFA presenting with isolated eyebrow loss (no scalp hair loss) FFA presenting with rapid loss of body hair (no scalp hair loss)
E FFA with mucosal changes FFA with lichen sclerosus FFA with genital lichen planus FFA with meibomian gland dysfunction FFA with oral lichen planus
F FFA with comorbid autoimmune diseases FFA with (i) alopecia areata, (ii) cutaneous/systemic lupus, (iii) vitiligo, (iv) Sjogren's syndrome, (v) rheumatoid arthritis, (vi) inflammatory bowel disease, (vii) others
G FFA with comorbid endocrine diseases FFA with thyroid disorders FFA with androgen excess/ FFA with androgen deficiency FFA associated with vitamin D deficiency FFA associated with metabolic dysfunction/dyslipidemia FFA post‐hysterectomy/FFA with ovarian insufficiency
H FFA with early age of onset FFA with premenopausal onset FFA with premenopausal onset PLUS ovarian insufficiency FFA with adolescent or pre‐pubertal onset
I FFA in special groups FFA in males FFA in skin of color
J FFA with family history Familial FFA
K Medication‐induced FFA FFA associated with use of medications such as: tamoxifen, raloxifene, checkpoint inhibitors, prostate cancer medications, estrogens
L Trauma‐induced FFA FFA developing post: (i) hair transplant, (ii) facelift surgery, or (iii) laser ablative resurfacing

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.

TABLE 2.

Example of 2018 Proposed Diagnostic Criteria for FFA and Limitations in LPPAI.

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 Clinical appearance of scarring alopecia of the frontal, frontotemporal or temporal scalp, in the absence of follicular keratotic papules on the body Diffuse bilateral eyebrow loss Minor criteria 3 Typical trichoscopic features of the disease: perifollicular erythema and/or follicular hyperkeratosis 4 Histopathologic features consistent with LPP/FFA 5 Hair loss at sites other than the frontal hairline: occipital area, facial hair, sideburns, or body hair 6 Noninflammatory facial papules
Part 2: Example of limitation of using percent changes in Lichen Planopilaris Activity Index (LPPAI) to evaluate FFA treatment efficacy.
Medication	LPPAI before treatment	LPPAI after treatment	Regrowth in eyebrows?	Regrowth in hairline?	Percent reduction in LPPAI	Most effective treatment
A	3.8	1.3	No	No	65.8%
B	3.8	1.3	Yes	Yes	65.8%	B

Abbreviations: FFA, frontal fibrosing alopecia; LPP, Lichen planopilaris; LPPAI, Lichen Planopilaris Activity Index.

4. Recognizing FFA as A Syndrome Would Open Up New Dialog Among Clinicians and Researchers

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.

5. Viewing FFA as a Syndrome Requires Us to Redefine What Constitutes a Treatment Success

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.

6. Recognizing FFA as A Syndrome Opens Up New Dialog Between Patients and Their Practitioners

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.

7. Conclusion

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.

Conflicts of Interest

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.