Lichen Planopilaris / Frontal Fibrosing Alopecia

Overview

Lichen planopilaris is a form of scarring hairloss (alopecia) that is most frequently seen in women aged 40-70. Lichen planopilaris is a broad umbrella term that refers to redness, inflammation, and scaling involving hairs anywhere on the scalp. A specific subtype of lichen planopilaris is frontal fibrosing alopecia where the redness, inflammation, and scarring is focused at the hairline. This condition is often progressive and treatment is aimed at slowing progression to prevent further loss. In this article we will discuss causes of lichen planopilaris, symptoms, diagnosis, treatment, and prognosis. 

Cause

The cause of lichen planopilaris / frontal fibrosing alopecia is unknown. However, it is thought to be an autoimmune disorder where the body’s immune system attacks the hair follicle. This results in inflammation and scarring of the follicle which leads to hair loss. Unlike alopecia areata whether the bulb ("root") of the hair is attacked by the immune system, in LPP the infundibulum (where the hair exits the scalp) is attacked. This difference in location is significant as when there is inflammation near where the hair exists the scalp (follicular ostia) permanent scarring can develop. It is unclear if this condition has a genetic basis and runs in families. Certain medications (such as checkpoint inhibitors like pembrolizumab) can induce lichen planopilaris. Some new data suggest certain types of sunscreens may contribute to LPP/FFA.

Symptoms

Symptoms of LPP typically include redness, itching, and scaling of the scalp followed by hair loss. The hair loss associated with scarring alopecia is gradual and progressive. In frontal fibrosing alopecia the hair loss is predominately at the frontal scalp / hair line. There may also be thinning of the eyebrows. Another feature is prominent veins on the temples and the presence of "lonely hairs" at the hairline. Lichen planopilaris in the most general form can effect anywhere on the scalp.

Source: https://commons.wikimedia.org/wiki/File:Frontal_fibrosing_alopecia.webp

License: Tziotzios, C., Petridis, C., Dand, N. et al., CC BY 4.0 <https://creativecommons.org/licenses/by/4.0>, via Wikimedia Commons

Diagnosis

Diagnosis of LPP is typically made by a dermatologist based on clinical examination and review of symptoms. Dermoscopy is often sufficient by identifying peri-follicular erythema and scale in a characteristic distribution. Particully when other features (prominent vascular, lonely hair sign, thinning of the eyebrows) are also present. Sometimes a biopsy may be performed in cases of diagnostic uncertainty.

Treatment

Treatment for LPP is aimed at slowing progression and preventing further hair loss. In the authors practice, systemic therapy with dutasteride is first line therapy. A recent review article showed single agent dutasteride to be the most effective systemic therapy achieving remission in nearly 2/3 of patients. Additional treatments include topical or intralesional steroids. Topical means the steroid is applied directly to the scalp where intralesional steroids are injected superficially into the skin. Non steroid topical therapy with calcineurin inhibitors (tacrolimus) or JAK inhibitors such as ruxolitnib (opzelura) can be helpful. Historically other systemic therapy with antimalarials (plaquenil) were utilized but this has fallen out of practice given the improved efficacy of dutasteride over plaquenil. In the authors practice, oral minoxidil is often used in attempt to salvage partially damaged hairs and thicken remaining hair. When eyebrows are involved, camouflage in the form of microblading is often employed.

Prognosis / outlook

Patients that seek treatment early have an improved prognosis compared with those that delay in seeking treatment. With medical therapy the majority of patients can stabilize their hair loss. Areas that have scarred typically do not regrow. The longer treatment is delayed, the worse the prognosis will be.

If you are experiencing any of the symptoms associated with scarring alopecia, it is important to see a dermatologist for proper diagnosis and treatment. Treatment is often successful in slowing progression and preventing further hair loss. However, scarring alopecia can be a progressive condition so early diagnosis and treatment is key to the best possible outcome.

 FAQs

  • While several small observational studies have suggested a link, 2020 review in the Journal of the American Academy of Dermatology found insufficient evidence to establish a direct causal relationship at this time:

    "The incidence of FFA appears to be increasing with time, leading to suspicion of a possible environmental trigger. Observational studies have reported a positive correlation between facial sunscreen use and FFA. This finding raises the question of whether sunscreen use plays a role in disease development. In this article, we review the available literature on the association of sunscreen with FFA. There is insufficient evidence to establish a direct causal relationship between sunscreen and FFA. Further studies are required to better characterize the role of sunscreen and the environment in the pathogenesis of this unique disease."

    https://pubmed.ncbi.nlm.nih.gov/31654665/

  • Hair transplants can be performed in patients with LPP/FFA, however, disease activity should be quiescent for at least one year prior to proceeding with a transplant. Ideally the transplant should be performed by a board certified dermatologist trained to assess activity of LPP/FFA. In the event the transplant surgeon is not a dermatologist, treatment should be coordinated with a dermatologist who can monitor activity over time.

  • Lichen planopilaris is a broad umbrella term that refers to redness, inflammation, and scaling involving hairs anywhere on the scalp. A specific subtype of lichen planopilaris is frontal fibrosing alopecia where the redness, inflammation, and scarring is focused at the hairline. Frontal fibrosing alopecia often involves the eyebrows. All frontal fibrosing alopecia is considered lichen planopilaris. Not all causes of lichen planopilaris are frontal fibrosing alopecia.