Frontal Fibrosing Alopecia: Causes and Treatment
By Bonnke Arunga, MBBS – Updated on October 22, 2020 – Reviewed by dermatologist Dr. Suneil Gandhi, MD
Scarring alopecia is a term used for a group of disorders that destroy the hair follicle and replace it with scar tissue, causing hair loss. It is also known as cicatricial alopecia. They account for about 7% of all causes of hair loss.
There are several types of scarring alopecia. In this article, we will describe frontal fibrosing alopecia (FFA), a type of scarring alopecia with unique characteristics and features.
This is considered to be a rare variant of lichen planopilaris, a common cause of scarring alopecia. This article is aimed to better inform you of this poorly understood disease. It was previously thought that frontal fibrosing alopecia is irreversible.
However, a recent case report shows that people are achieving significant treatment outcomes from FFA. Read on and learn the causes, clinical features, and potential treatment options for FFA.
What is frontal fibrosing alopecia?
Frontal fibrosing alopecia is a type of hair loss occurring in a band-like distribution on the front part of your hair. It also appears on the eyebrows in a few cases. This type of alopecia primarily affects the hairline and presents in a manner reminiscent of a hairline receding backward.
Frontal fibrosing alopecia primarily affects women in their post-menopausal years. Though, the disorder can be experienced by men and women of various age groups. It is considered to be a variant of lichen planopilaris, a common cause of scarring alopecia.
Causes of frontal fibrosing alopecia
The scientific community is still not sure about the exact cause of frontal fibrosing alopecia. However, researchers have hypothesized that the reason is an interplay between inflammation, immune dysregulation, genetics, and hormonal influences.
There is strong evidence that FFA is an autoimmune condition where the immune system of the susceptible individual attacks the hair follicles. The hormonal component in the cause of FFA is what makes this condition prevalent among post-menopausal women. During menopause, the hormone (dehydroepiandrosterone) DHEA decreases significantly. The hormone essential for the production of PPAR-gamma, a ligand-receptor.
According to studies, the deficiency of PPAR-gamma due to decreased DHEA has significant implications in post-menopausal FFA. Androgens have been suggested to play a major role in the pathogenesis of FFA. DHEA levels decline during menopause. This leads to a decrease in the production of PPAR-γ, the deficiency of which leads to hair follicle inflammation and scarring.
The majority of cases of FFA are isolated. This observation means that the genetic component of FFA is not strong. However, genetic studies on this condition have been lacking.
The sequence of progression of FFA is as follows.
This step-by-step progression of FFA may be confusing at face value because it looks similar to other types of hair loss such as androgenic alopecia (AGA). However, subtle differences make FFA unique compared to other types of hair loss.
1. The location of the inflammation
In FFA, the inflammation is located in the stem cell bulge of the hair follicle. In contrast, the location of inflammation in androgenic alopecia is in the upper third of the follicle and is of a milder degree.
2. The nature of the inflammation
The nature of inflammation in FFA is relatively unknown. However, there are hypotheses relating it to autoimmunity. There is an increased activity of CD8+ T-lymphocytes in the hair bulge region. But in AGA, there is no scarring, and the problem is always at the androgenic receptors.
At face value, it may seem quite absurd to differentiate the types of hair loss based on the location and nature of inflammation. What difference does it make?
There’re a lot of differences because these differences are what predict the onset, extent, and progression of the scarring.
How to tell if you have frontal fibrosing alopecia
Unlike other conditions of scarring alopecia, FFA usually presents with few symptoms. It occurs in a very gradual manner, so it is usually noticed very late. The most common symptom is a receding frontotemporal hairline and sometimes, loss of eyebrows. Quite often, there is a symmetric and slow-progressing loss of hair at the sides of the scalp as well.
Can frontal fibrosing alopecia be reversed?
The mainstream information about frontal fibrosing alopecia in the scientific community is that it cannot be reversed. The explanation is always given as two major reasons.
Based on these two reasons, the general idea has always been that FFA cannot be reversed and that the treatment options can only slow its progression. However, this view is slowly changing as new evidence emerges, showing that FFA and several other types of scarring alopecia can be reversed – at least to some extent.
The belief that FFA can be reversed is based on the piece of evidence that FFA has been reversed before.
In one of the studies, a few individuals with FFA experienced slight hair regrowth. The halt of disease progression came after different therapies. These findings mean that either the stem cell bulges weren’t lost completely and could be recovered.
It is plausible that follicles regenerate even after stem cells are damaged – a concept that still needs to be studied. Thus, there is some evidence that FFA can be reversed in special circumstances.
Treatment options for frontal fibrosing alopecia
Currently, there are no specific treatments that are absolutely effective in treating FFA. There are limited clinical trials supporting the treatment of FFA. Thus, the efficacy of treatment should be established with caution. The treatment options can be categorized as either drug-based or non-drug-based treatments.
Pharmacotherapy (drug-based treatment)
Corticosteroids
Corticosteroids for FFA can be administered topically, intralesional, or orally. Although corticosteroids are active against most cases of scarring alopecia, locally administered corticosteroids are insufficient at halting the progression of FFA in 93 percent of cases.
The cases of improvement and stabilization often occur when the corticosteroids are used alongside 2% or 5% minoxidil.
According to studies, triamcinolone acetonide in dosages of 10 mg/mL, exhibits a 60% improvement when administered intralesionally. Excessive usage of corticosteroids is also discouraged because they cause systemic effects and thinning of the skin.
Hydroxychloroquine
Hydroxychloroquine is a synthetic antimalarial and has been effective in treating FFA according to case studies. One study found out that this medication showed significant improvement of up to 73 percent after a 6-month usage.
The dosage of hydroxychloroquine for FFA is 7.5 mg/kg, though your doctor may make changes to the dosage to individualize the care you receive. Hydroxychloroquine can cause retinopathy, so periodic monitoring is required.
5-alpha-reductase inhibitors
The best results in FFA are achieved by the 5-alpha-reductase inhibitors. Finasteride and dutasteride are the most common drugs in this category. Studies have shown that a finasteride dosage of 2.5mg per day is appropriate for patients with FFA. On the other hand, the recommended dosage for dutasteride is slightly lower – 0.5 mg per day.
For better results, these medications can be used together with 2% minoxidil or topical calcineurin inhibitors for a period of 6 to 18 months, depending on the recommendations by your healthcare provider.
Other treatments to be mentioned
Pioglitazone is a synthetic PPAR-gamma (peroxisome proliferator-activated receptor). It is primarily used in a hospital setting to manage diabetes mellitus type 2. Moreover, it is one of the proposed treatments for frontal fibrosing alopecia and other cicatricial alopecia. It works the same way as PPAR-gamma in promoting hair growth.
Isotretinoin is not really a primary treatment for frontal fibrosing alopecia. Studies have found out that a section of patients with FFA tends to present with concomitant papules on the face. Thus, low-dose isotretinoin is often considered as the ideal treatment option for facial papules and also to alleviate scalp inflammation in FFA.
Naltrexone has potent anti-inflammatory benefits. A recent clinical trial showed that naltrexone given as an oral-low dose could be used to manage the inflammation associated with FFA.
Tofacitinib is a Janus Kinase (JAK) inhibitor and has been shown to be effective in treating non-cicatricial alopecia. In one study, patients received 5mg of Tofacitinib two to three times daily for a period between 2 to 19 months. In this study, only weight gain was the reported side effect.
Non-pharmacologic treatments for FFA
For a long time, FFA has been considered one of the indications for hair transplants. The indication for transplantation comes in handy when the alopecia is irreversible.
Besides, FFA evolves slowly making transplants an effective treatment option. However, hair transplant in scarring alopecia such as FFA is way different from that in non-scarring hair loss.
The grafts in non-scarring hair loss have a 90% survival rate, while those in FFA and other scarring hair loss disorders have a survival rate averaging 50%. Although this treatment can be used in FFA, the grafts are less likely to survive for long.
Can frontal fibrosing alopecia be prevented?
Yes and no. Let me explain.
As mentioned earlier, the cause of FFA is not known. Inflammation, autoimmunity, genetics, environmental factors, and hormones can contribute to its aetiology. However, these causes only start manifesting when the susceptible individual is exposed to triggers of FFA.
For instance, other autoimmune diseases, hairstyling, use of sunscreens on the forehead, post-menopausal state, bacterial infections, certain prescription drugs are among the things that can trigger causes of FFA.
So, yes, you can prevent FFA by avoiding products that trigger it, avoiding sunscreen on the forehead, or treating bacterial infections quickly. But, no, post-menopausal women who are susceptible to FFA cannot prevent it because menopause is a stage in the life of every woman.
Although you cannot prevent the onset of the disease per se, you can slow down the progression to scarring by early diagnosis and early treatment. As has been discussed already, 5-alpha-reductase-inhibitors such as finasteride and dutasteride are the most potent stabilizers of the disease.
Bottom line
Fortunately, frontal fibrosing alopecia is quite rare. As a group, scarring alopecia only constitutes 7% of hair loss disorders. But when they occur, the scarring is quite aggressive, destroying even the tissues surrounding the hair follicles.
This destruction of tissue and hair follicles is the reason why researchers believe that the disease cannot be reversed.
Even in the face of limited research on this disease, there are treatment options that can help. Combination treatments with anti-inflammatory drugs and 5-alpha-reductase inhibitors are among the most effective therapies.
