In continuation from our previous article, we will continue to look at other aspects of frontal fibrosing alopecia. Given that we have already looked at what the condition is, the history, the clinical features, the epidemiology as well as the pathogenesis, we will now continue to look at the differential diagnosis, how it presents itself and how it can be treated or contained.
Let’s first start with the differential diagnosis for frontal fibrosing alopecia:
In order to treat frontal fibrosing alopecia properly, it is important that it is diagnosed properly and there has to be complete assurance that it is FFA and not any other condition that presents in a similar manner.
It is important to ensure that the condition is frontal fibrosing alopecia and must not be confused with:
- A high frontal hairline or high forehead, which is quite common in women.
- Androgenetic alopecia, or more specifically, in the frontotemporal type of androgenetic alopecia, there is lesser inflammation at the edge of hair and there is also no clear band like pattern.It also occurs as bitemporal recession than frontal recession unlike FFA.Also,androgenetic alopecia in women rarely affects the front part of the hairline.
- Ophiasis, type of alopecia areata. Alopecia areata tends to affect the eyebrows too, but should not be confused with FFA. It is a smooth hairless patch without any scarring or inflammation unlike FFA.
- Traction alopecia can occur at the same site, but it does not mean that it should be confused with FFA. Proper history and presence of broken hair at varying length is more in favour of traction alopecia.
- Senile alopecia does not present the same kind of distribution, but there is loss of hair in the eyebrows.
- Other types of cicatricial alopecia, including folliculitis decalvans, pseudopelade and keloid acne. There is a progressive course of hair loss in FFA, but it might not be seen in others. There is also lesser inflammation, when it comes to FAA.
The doctor should be able to have a clear and proper understanding of whether the condition is FAA or not, before starting a course of treatment. Since there are so many overlapping features, there is always the chance of confusion, which could lead to wrong treatment as well. This is all the more reason why a differential diagnosis is crucial
Coming back to some of the basics, let’s take a look at what frontal fibrosing alopecia looks like:
- The loss of hair is similar to a head band occurring over the frontal area, and the continuation of the loss is in a similar pattern.
- The hair loss is not sudden, it will happen gradually.
- In certain cases, there is thinning in the eyebrows as well.
- In a majority of the cases, the condition starts in women who have crossed their menopause.
While there is no clear indication as to what causes frontal fibrosing alopecia, there are a few likely causes:
There are many studies that have shown that FFA could be a result of the immune system of the body attacking the hair follicles and this is what leads to the inflammation as well. And in turn, the inflammation damages the hair follicles further. Since most women who suffer the condition are post their menopause, there is also a consideration that there could hormones in play. There are little chances of hair growing back; however there are methods of disguising and treating the same to limit the loss.
Pre-treatment Diagnosis for frontal fibrosing alopecia:
Before starting on any course of treatment for frontal fibrosing alopecia, it is important to understand that the condition is not a reversible one, and the progression is a slow one. Since the condition still does not have a clear course of treatment that has been proven truly effective, the course of ‘treatment’ charted out by each doctor will vary.
The course of treatment is also decided by way of – scalp biopsy, the type of inflammation, the exact location of the inflammation and manner in which the scalp changes over a pre-decided period of time. All this needs to be taken into consideration, before deciding the suitable therapy and medications. Certain topical steroids have proven effective in arresting the speed at which the condition progresses.
Treatment of Frontal Fibrosing Alopecia
The treatment needs to be given in the manner of application of the above mentioned topical steroids or in the form of infiltrations.
- One of the preferred first lines of therapy is triamcinolone injections, which are given over the lesions
- A combination of Minoxidil and Finasteride has also proven to be effective in certain cases.
- Short course treatments of oral steroids or retinoids can also be tried.
- Studies have shown that hydroxychloroquine worked in courses that were spread over six and twelve months; and there was a visible improvement. As a matter of fact, improvements were seen within the first six months.
However, the studies did prove that hydroxychloroquine was the most effective of the spectrum of medications, even though there were certain people who had reactions, such as retinopathy and gastric issues.
Hair loss is of various types, and in order to treat any one of them, it is important to understand which type it is first. Frontal fibrosing alopecia is one of the types of scarring or cicatricial alopecia.A rare condition affecting mostly women in the menopausal or post-menopausal age group, it can lead to them losing a few inches of their hairline.
In most cases of frontal fibrosing alopecia or FFA, the scalp will seem normal, although there might be paleness. In many cases, there is also scarring on the scalp, which does not fade away with time. The condition presents itself in the form of a band, which is not wide, but does develop atrophy over time.
An in-depth introduction to frontal fibrosing alopecia:
This is a type of primary cicatricial alopecia, in which the hair loss occurs in a pattern along the front part of the hairline. In many cases, the progression is so rapid and extensive that there is a loss of eyebrows too. The condition is often considered to be a variation oflichen planus of the scalp and there can be associated lichen planus lesions on the skin and mucous membrane in some of these patients. While the exact cause of the condition is still unknown, it has been observed that it affects women who are above fifty five years of age.
Studies have shown that one possible reason for FFA could be a disturbance in the response of the immune system of the body to the hair follicles. Yet another study has shown the possibility that the hair loss could be caused due to fluctuations in the hormonal system of the body.
Lets now move to the history and epidemiology of frontal fibrosing alopecia:
While the condition might have existed for several years, as a clinical entity, the condition received recognition only in the early 1990s. As a matter of fact, it was in 1994 that the condition was first described by Dr. Steven Kossards, in great detail. His initial studies described the condition in six women, who were in the later part of their lives. Several studies followed and many cases have been further reported.
If one were to look at this condition histologically, then, the condition is often characterised by a dense infiltration of lymphocytic bodies. These are most prominent around specific regions of the hair follicles, namely the isthmus, the infundibulum and the bulge. There is inflammation that leads to the irreparable destruction of the hair follicles,with loss of the sebaceous glands and what remains are scar tissues due to fibrosis.
Studies showed that most of the women who were affected were in the post menopausal age group or approaching their menopause.There have been rare cases of the condition being reported in women who are below 40 years of age and in men.
Moving onto the pathogenesis of frontal fibrosing alopecia
The histopathology of FFA is similar to lichen planopilaris and the manner in which it presents is also similar. While in most cases, the FFA develops in women who already are presenting characteristics of androgenetic alopecia, it can appear otherwise as well. The onset of this condition can also happen, well past the ages that have already been discussed. Given that both conditions tend to respond to a course of Minoxidil, there is still doubt whether the hormones involved in both conditions are similar. However, there is a general consensus that regional factors might be involved. Environmental factors like certain toxins can also act as a trigger for this condition.
Now that we have looked at the pathogenesis of frontal fibrosing alopecia, lets move onto the actual pathology and investigations:
The characteristic pattern of alopecia and the age group can help the doctor to clinch the diagnosis. However to confirm the same, and also in rare presentations, a scalp biopsy is advised.
Histopathology is similar to lichen planopilaris characterised by the presence of lymphocytic infiltratearound the isthmus as well as the infundibular regions. There is follicular hyperkeratosis and in the later stages, hair follicles may be replaced by fibrous tracts.
Finally, lets take a look at the clinical features of frontal fibrosing alopecia:
There is a band like alopecia, which is most obviously visible in the front part of the scalp. The band can spread and affect even the eyebrows.
In certain cases, there is a slight itch, but in most cases, there are no such indications.
The scalp portions that have been affected will seem shiny and pale due to atrophy and scarring of the underlying tissues.
There could be thinning of the eyebrows due to loss of eyebrow hair. There could be thinning of hair on other parts of the body like as well.
In the next article, we will continue to look at other aspects of frontal fibrosing alopecia, including differential diagnosis and treatments.
In this series, we will take you through real life cases (using assumed names), to give you an in depth understanding of the various conditions that can affect the scalp.
This particular case study is about Swati, 55yr old female who was diagnosed with frontal fibrosing alopecia. Given here are the features, causes, diagnosis and treatment that was administered to her.
- Features: When Swati first walked into our clinic, she came with history of massive hair loss from the frontal side. We observed that hair loss was present over the front part of her scalp almost in a straight line backwards and there was some amount of associated
scarring. She was put through a basic hair and scalp analysis. We also noted that there was an almost symmetric band like loss of hair, on the front part of her scalp, as well as towards the side. The skin where the hair loss had taken place, looked more or less normal, with a slight amount of scarring. On closer inspection, we were able to see the redness and the scaling. This helped us to diagnose the condition as frontal fibrosing alopecia, where the frontal part of the scalp was affected. The condition normally affects women over the age of 50.
- Causes: We informed Swati that the cause is usually unknown; however, it is thought to be a variant of the disease called lichen planus where the body’s immune system is at fault. The immune response to certain components of the hair follicles becomes fault ridden, and that might be the cause for the condition. Since the condition appears post menopause, we feel that there might be certain hormones in play and relayed the same to Swati as well.
- Diagnosis: Our first step was to inform Swati that this condition tends to progress slowly, but does stabilise after a while. We then moved onto confirming our diagnosis, for which we conducted a scalp biopsy. We harvested the hair follicles that had been affected recently and these were encircled by inflammatory cells in lichenoid pattern.
- Treatment:We put Swati on a course of steroid pills and anti-inflammatory antibiotics and were able to contain the condition. After the disease has become stable for over a year, we were able to conduct a transplant procedure on her, allowing her to style her hair in a normal manner.
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