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:
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:
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.
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.