Frontal Fibrosing Alopecia (FFA) is a patterned form of scarring hair loss that occurs along the frontal hair margin. It is characterized by band-like scarring hair loss with signs of follicular inflammation on the edges. The pathology lies in the replacement of hair follicles with fibrosis, which progressively causes hair loss and frontal scarring.
Frontal Fibrosing Alopecia is thought to be a variant of lichen planopilaris that usually affects post-menopausal women. The incidence of this condition is increasing worldwide, with a peak of presentation being around 60 years of age. A few of the cases of Frontal Fibrosing Alopecia were also reported in younger women and men, although, it remains a rare finding in this population. In African American females, the condition is often found to be associated with traction alopecia that worsens the prognosis of the disease.
What are the symptoms?
Frontal Fibrosing Alopecia usually presents with the following signs and symptoms:
A linear band like hair loss that occurs along the front and side margins of the scalp, causing a prematurely receding hairline. The pattern is almost symmetric and slowly progressive. The skin visible after the hair loss is usually shiny, pale with mild scarring. In the active phases of the disease, the affected skin may also show redness and scaling.
Similar to the scalp, loss of hair is apparent on other parts of the body as well, with almost complete loss of limb hair in most cases. A partial/complete loss of eyebrow hair is also a characteristic finding. Itching and pain are common and may occur in the early phase of the disease even before the hair loss starts. Facial rashes are another potential early signs and can be of red to yellowish papules in color, and can be found on the temples and forehead.
In a few cases, FFA may present as female pattern hair loss with diffuse thinning of the scalp hair with or without volume reduction.
What are the causes of Frontal Fibrosing Alopecia?
Although hormonal disbalances and inappropriate autoimmune responses are two of the most commonly proposed causes, the exact etiology of FFA remains unknown. It is believed that abnormal activation of the autoimmune system can attack the hair follicles that can result in early hair loss and scarring. Whereas, the high incidence of cases reported in post-menopausal women is more suggestive of a hormonal component in the pathophysiology.
Increasing familial cases point towards a genetic predisposition that can be triggered by environmental factors such as sun exposure, sunscreens, and viral infections. A study published in 2016 supported the role of both genetic and environmental factors in the development of FFA.
The condition is also frequently reported in patients with contact allergy, long-term sunscreen use, hypothyroidism, and autoimmune diseases, such as lupus and rheumatoid arthritis. Many cases were also observed after hair transplantation and facial lifting surgery, suggesting hair loss post-injury.
Diagnosis
Frontal Fibrosing Alopecia is to be suspected in presence of the previously mentioned signs and symptoms. The diagnosis can be further confirmed, with the help of a scalp biopsy, which can also effectively rule out other conditions having similar presentations. Dermoscopy, which is the examination of the skin under a microscope, can reveal a lack of hair follicles, redness, and scales on the affected scalp surface. An increase in the sebaceous glands may also be commented upon on this examination. Other recommended laboratory tests may include blood work, thyroid function tests, and autoimmune profile.
Treatment options for Frontal Fibrosing
Unfortunately, there is no single approved treatment regime to manage this condition as the etiology remains uncertain. A variety of drugs are commonly prescribed which may have varying levels of benefits in different individuals.
These include:
- Steroids: Short courses of oral and intralesional steroids are used to suppress the proposed autoimmune component of the disease. Studies show a 60% improvement when an intralesional application of corticosteroids is carried out.
- Anti-inflammatory: may be employed to provide relief from the itching, redness, and pain found in the early phases of the disease.
- Antibiotics: such as tetracyclines and anti-malarial are shown in some studies to provide benefits in patients with rapid onset of hair loss.
- Anti-androgens: such as 5-alpha reductase inhibitors (Finasteride and Dutasteride) are also reported to reduce the magnitude of hair loss. Minoxidil, another drug that is found effective, especially in patients with co-existing androgenetic alopecia.
- Immunosuppressants: Ciclosporin and Mycophenolate mofetil are two of the tested drugs that are currently in the trial to treat FFA. However, a high rate of recurrences is recorded with these drugs.
- Antigylcemic: an off label use of antidiabetic, Pioglitazone was found to reduce the symptoms and progression of FFA. However, the lack of further studies does not merit its use. The drug was also found to be associated with side effects such as weight gain and ankle swelling.
- Hair grafting: some practitioners suggest hair grafting and transplant once the disease activity is considered to be settled. It is preferred to perform a test graft to ensure the progression of the disease has halted.
Prognosis
The long-term prognosis of Frontal Fibrosing Alopecia varies from individual to individual, although, it is considered to be self-limiting in most cases. As a rough estimate, it is a slowly progressive disease that worsens over time. The progression of the disease is, however, faster without any treatment. In a few cases, the condition may stabilize over time, however, the final degree of hair loss is still difficult to predict.
References:
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Litaiem N, Idoudi S. Frontal Fibrosing Alopecia. StatPearls [Internet]. 2020 Aug 10.
Kang H, Alzolibani AA, Otberg N, Shapiro J. Lichen planopilaris. Dermatologic Therapy. 2008 Jul;21(4):249-56.
Tavakolpour S, Mahmoudi H, Abedini R, Hesari KK, Kiani A, Daneshpazhooh M. Frontal fibrosing alopecia: an update on the hypothesis of pathogenesis and treatment. International Journal of Women’s Dermatology. 2019 Jun 1;5(2):116-23.
Zhang M, Zhang L, Rosman IS, Mann CM. Frontal fibrosing alopecia demographics: a survey of 29 patients. Cutis. 2019 Feb 1;103(2):E16-22.
Tan KT, Messenger AG. Frontal fibrosing alopecia: clinical presentations and prognosis. British Journal of Dermatology. 2009 Jan;160(1):75-9.
NavarroBelmonte MR, Navarropez V, RamrezBosc A, MartnezAndrs MA, MolinaGil C, GonzlezNebreda M, AsnLlorca M. Case series of familial frontal fibrosing alopecia and a review of the literature. Journal of Cosmetic Dermatology. 2015 Mar;14(1):64-9.