What is Frontal Fibrosing Alopecia?

Frontal Fibrosing Alopecia (FFA) is a form of hair loss which can be permanent or scarring in nature.  It is currently classified as a subtype of lichen planopilaris. The cause is unknown but is probably an inflammatory process induced by genetic or environmental factors.  It is classified as a primary inflammatory scarring type of hair loss. That is, it is not from infections, burns, surgical scars, caustic hair products, etc., but is a disease centered around the hair follicle unit itself.  Women with FFA develop mild to extensive hairline recession that can expand around the perimeter of the scalp.  Total eyebrow loss can occur as well.

Cause

The cause of frontal fibrosing alopecia is unknown.  Over the past few decades there has been an exponential increase in the number of new cases, especially in westernized countries.  This has lead researchers to believe that there may be an underlying environmental trigger.  Recent epidemiologic studies have suggested that leave on cosmetic products (sunscreen, makeup, etc.) may be linked to the cause, since their use has dramatically increased in the past few decades. However, more research is needed on this topic. Because this disease affects peri and post-menopausal women predominantly, it has been hypothesized that a relative lack of estrogen or hormonal shifting may trigger the onset of the disease.

Like its parent diagnosis, lichen planopilaris, the target of inflammation is the sebaceous or oil gland which is located at the same site as the stem cell region of the hair follicle.  Recent research has shown that there is a defect in a “master regulator” protein called the peroxisome proliferator-activated receptor gamma, or PPAR gamma.  PPAR gamma is responsible for in the preservation of hair follicle cells, including stem cells, and sebaceous glands.  Loss of PPAR gamma leads to sebaceous gland dysfunction, which causes abnormal processing and buildup of “toxic” lipids. This abnormal buildup of lipids triggers inflammation that destroys the oil gland inflammation and the nearby stem cells, which are innocent bystanders.  Once stem cells are destroyed, the follicles cannot regenerate, and permanent hair loss ensues.

Signs & Symptoms

The typical profile of a person with frontal fibrosing alopecia is a peri or post-menopausal adult female.  There are isolated cases of it affecting adult males and teenage girls.

Unlike lichen planopilaris, frontal fibrosing alopecia is largely without symptoms.  Because the disease is silent if often goes unnoticed for many years before it is diagnosed. There is a triad of findings in fibrosing alopecia which includes:

(1) Sudden eyebrow loss.  This can diffuse or patchy loss, and most of the time accompanied by redness and scaling around the hair follicles.  The existing eyebrow hairs themselves may change texture and become course and wiry.  Overtime complete permanent eyebrow loss can occur.

(2) Hairline recession.  As the name of the disease implicates, the frontal hairline is most often affected, followed by the sideburns, around the ears and the posterior hairline.  The hair loss is band-like and can recess further from the natural hairline if the disease is untreated.   The hair follicles surrounding the hair loss have characteristic redness and scaling around the hair follicles.

(3) Extremity hair loss. This is a more inconspicuous finding, because extremity hair loss can also happen naturally as we age. However, hair loss on your extremities, like arms and legs, in frontal fibrosing alopecia can be patchy and completely devoid of hair which is an unusual pattern for age related hair loss.

Other findings of frontal fibrosing alopecia include skin colored facial bumps, or papules, which represent involvement of the vellus (fine, non-pigmented hairs) of the facial region.  Sometimes frontal fibrosing alopecia can overlap with classic lichen planopilaris.

Diagnosis & Treatments

Detailed history, thorough physical examination of the scalp and interpretation of appropriate laboratory procedures, including scalp biopsies are necessary. Scalp biopsies are critical for the assessment and diagnosis of scarring hair loss and are often performed when considering a diagnosis of frontal fibrosing alopecia.

Early diagnosis and therapeutic intervention can often prevent permanent damage to existing hair follicles. Once a definitive diagnosis is made, we can determine the best treatment plan for everyone. Some regrowth is even possible with treatment.

Management of frontal fibrosing alopecia is complex. Treatment is case-dependent, based on severity of symptoms (itching and inflammation), findings on biopsy and extent of hair loss. Response to treatment varies from individual to individual.

In general, very mild disease may be managed by topical corticosteroid solutions or foams, topical calcineurin inhibitors (tacrolimus) and anti-inflammatory shampoos (such as tea tree oil). However, in most cases, topical treatments alone are not sufficient.  The next step could be the addition of corticosteroid injections to the involved areas of the scalp, if the disease is localized to small areas.  For more diffuse or severe cases, oral anti-inflammatory medications such as doxycycline, hydroxychloroquine (Plaquenil) are necessary to control the inflammation and destructive process.  Rarely, immune suppressant drugs such as azathioprine (Imuran), mycophenolate mofetil (Cellcept) and cyclosporine are needed to achieve control.  More recently, pioglitazone (Actos) an anti-diabetic drug which is a potent agonist for peroxisome proliferator-activated receptor-gamma (PPARγ), has shown some promising results.  Also, because the potential hormonal trigger, finasteride (a DHT blocker) has been shown to be effective alone or in combination with other treatments in treating this disease.

We encourage you to discuss these treatment options with your dermatologist.

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