frontal fibrosing alopecia

Frontal fibrosing alopecia

Frontal fibrosing alopecia describes hair loss (alopecia) and scarring in the frontal region of the scalp. In some cases, the eyebrows, eye lashes and/or other parts of the body may be involved, as well 1). Frontal fibrosing alopecia was first described in a group of Australian post-menopausal women in 1994 2). Frontal fibrosing alopecia is a localized form of lichen planopilaris. Frontal fibrosing alopecia usually affects post-menopausal women over the age of 50. Frontal fibrosing alopecia can infrequently appear in premenopausal women and more rarely in men. Hair loss can also affect the body. Frontal fibrosing alopecia incidence is reported to be increasing in white-skinned women (possibly because of greater awareness of the condition), and it is uncommon in women with dark skin.

Frontal fibrosing alopecia may be due to hormonal changes or an autoimmune response, the exact cause of this condition is not yet known.

Frontal fibrosing alopecia presents as a progressive symmetric band-like alopecia (hair loss), affecting the frontal hair line, the preauricular scalp and, less commonly and distinctively, the retroauricular areas. ”Orphaned” hairs ie isolated hairs, may remain in areas of hair loss. The eyebrows are often affected and this may occur before the frontal scalp. Clinical inflammation is not observed in the eyebrows. The other vellus or terminal hair of the face can be involved, including the eyelashes. Additional clinical features – atrophy of affected sites with prominent forehead veins and facial papules. Progression, for the majority, is relatively slow

Dermoscopic features – clearly perifollicular, with keratin scale surrounding and extending along the proximal part of the hair shafts. Erythema often mild or absent.

Figure 1. Frontal fibrosing alopecia

frontal fibrosing alopecia
frontal fibrosing alopecia

Frontal fibrosing alopecia causes

The exact underlying cause of frontal fibrosing alopecia is unknown. Frontal fibrosing alopecia is thought to be an autoimmune condition in which an affected person’s immune system mistakenly attacks the hair follicles (structures in the skin that make hair). There is a disturbed immune response to some component of the intermediate-sized and vellus scalp hair follicles. Genetic, hormonal and environmental factors may be involved. A 2016 study suggests that there are both genetic and environmental components involved in frontal fibrosing alopecia. Contact allergy or photocontact allergy to cosmetics, hair dye, and sunscreens have been suggested as possible but unconfirmed causative factors 3).

While most frontal fibrosing alopecia cases appear to be isolated and the only one in a particular family, there have been familial cases reported in the literature. Genetic studies, however, have been lacking.

Frontal fibrosing alopecia is considered a variant of lichen planopilaris.

What is lichen planopilaris?

Lichen planopilaris is a rare inflammatory condition that results in patchy progressive permanent hair loss mainly on the scalp. Three forms are recognized 4):

  • Classic lichen planopilaris, also known as follicular lichen planus
  • Frontal fibrosing alopecia
  • Graham Little syndrome

Lichen planopilaris usually affects young adult women, although the age range is wide and it also affects men. It commonly develops in association with lichen planus affecting the skin, mucosa and nails.

The cause of lichen planopilaris is unknown.

Although lichen planopilaris is rare, it is one of the common causes of scarring hair loss of the scalp.

Frontal fibrosing alopecia symptoms

Frontal fibrosing alopecia is characterized primarily by a usually symmetrical band of hair loss (alopecia) and scarring on the front and sides of the scalp. The edge may appear moth-eaten, and single ‘lonely’ hairs may persist in the bald areas. The band of hair loss on the front and sides of the scalp is usually symmetrical and slowly progressive (worsening over time). Approximately half of all affected people experience loss of eyebrows, as well. Less commonly, the eyelashes may also be involved. Some people with frontal fibrosing alopecia develop hair loss in areas other than the scalp and face. Some women with frontal fibrosing alopecia also have female pattern hair loss, which is associated with thinning of hair on the scalp due to increased hair shedding and/or a reduction in hair volume.

The skin in the affected area usually looks normal but may be pale, shiny or mildly scarred, without visible follicular openings. At the margins of the bald areas, close inspection or dermatoscopy shows redness and scaling around hair follicles.

In some cases, there are skin coloured or yellowish follicular papules located on the forehead and temples 5).

Trichoscopy (dermoscope for the scalp and hair) reveals absent follicles, white dots, tubular perifollicular scale and perifollicular erythema. In skin that tans easily, perifollicular pigmentation may be observed.

Frontal fibrosing alopecia diagnosis

The clinical features of frontal fibrosing alopecia are characteristic. A skin biopsy examination in the laboratory may help to make or confirm the diagnosis. The newly affected hair follicles are surrounded by a lichenoid pattern of inflammation associated with scarring. The histopathological features are identical to those of lichen planopilaris.

Perifollicular erythema and scarring white patches are seen on dermoscopy. On scalp biopsy, lymphocytic and granulomatous perifolliculitis with eccentric atrophy of follicular epithelia and perifollicular fibrosis are visualized.

Biopsy of skin papules may show a lichenoid pattern of inflammation, fibrosing alopecia, or sebaceous gland hyperplasia.

Frontal fibrosing alopecia differential diagnosis

Important diagnoses to consider include female pattern hair loss, chronic telogen effluvium and alopecia areata.

  • Female pattern hair loss is a non-scarring progressive miniaturization of the hair follicle with one of three different characteristic patterns.
  • Chronic telogen effluvium is an idiopathic disease causing increased hair shedding and bi-temporal recession, usually in middle aged women.
  • Alopecia areata is an autoimmune attack of hair follicles that usually causes hair to fall out in small round patches.

Frontal fibrosing alopecia treatment

There is no very effective treatment available for frontal fibrosing alopecia to date. A short course of oral steroids, intralesional steroid injections, anti-inflammatory antibiotics such as tetracyclines, or antimalarial tablets may benefit patients who have a rapid onset of hair loss. The five alpha-reductase inhibitors finasteride and dutasteride have been reported to stop further hair loss in some women but has not been confirmed by controlled studies 6).

The use of the antidiabetic agent pioglitazone (off-label) for the treatment of frontal fibrosing alopecia was reported to reduce symptoms, inflammation, and progression of frontal fibrosing alopecia 7) but its use has not been supported by further investigations 8). Side effects include ankle swelling and weight gain.

Adalimumab, a tumor necrosis factor-alpha (TNFα) inhibitor, has also been reported to be effective in a few patients 9).

Frontal fibrosing alopecia cure

Unfortunately, there is currently no cure for frontal fibrosing alopecia.

Frontal fibrosing alopecia prognosis

The long-term outlook (prognosis) for people with frontal fibrosing alopecia varies. Usually, frontal fibrosing alopecia is slowly progressive (worsening over time); however, the condition does stabilize after a few years in some patients. Hair regrowth has been reported in some patients.

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