What is Lichen Planopilaris?

Lichen planopilaris is a condition that causes inflammation and permanent hair loss, appearing in different patterns. Like lichen planus, the reason why lichen planopilaris happens is unknown, but it’s believed to be linked to the same cause as lichen planus. It results in patchy, ongoing hair loss, mainly on the scalp, but can also affect other areas where hair grows like eyebrows and pubic area. It’s crucial to understand how autoimmune diseases work and how inflammation plays a part, to come up with the right treatment.

The frequency of lichen planopilaris, a skin-related condition, is also unclear. Recent treatment methods based on scientific research suggest the use of corticosteroids, a type of anti-inflammatory medication; topical tacrolimus, a cream/ointment that helps reduce inflammation; antimalarial drugs, and new treatments such as JAK inhibitors which are medicines that block signals causing inflammation. What’s also important is patient education, cooperation between different medical teams, and planning for long-term management. These strategies will help improve the individual’s lifestyle and how they look, which is greatly affected by this condition.

What Causes Lichen Planopilaris?

Lichen planopilaris is a condition that causes hair loss and is linked to the immune system. It’s a specific kind of lichen planus, a skin disease, that affects the hair follicles (the tiny structures in the skin that hair grows from). This idea is well-supported by clinical findings as well as what we see when we look at tissue samples under a microscope.

Although we’re not completely sure what causes lichen planopilaris, it’s often considered an autoimmune disorder. In other words, it’s thought that the body’s immune system mistakenly targets specific parts of the hair follicles.

While we don’t know the exact cause, it seems like hair loss in this condition comes from a harmful immune response to something in the hair follicles that the body doesn’t recognize. Genetic factors or medications rarely trigger the disease. However, there might be a connection with certain types of antibodies (proteins your immune system uses to fight off harmful substances), such as pembrolizumab. The use of nilotinib, another type of drug, is also associated with this condition.

Risk Factors and Frequency for Lichen Planopilaris

The exact number of people who suffer from cicatricial alopecias, a group of conditions that result in hair loss, is unknown. Lichen planopilaris is the most common type of primary scarring alopecia which causes hair loss, and it accounts for 43% of such cases in a study involving 72 people. Other types of cicatricial alopecias include frontal fibrosing alopecia and Graham-Little syndrome, which are considered to be variants of lichen planopilaris. These conditions usually affect women between the ages of 40 and 60 more often than men. Additionally, about half of the patients may develop characteristic lesions of lichen planus which can affect the skin, mucous membranes, or nails.

  • Lichen planopilaris is the most common type of cicatricial alopecias, accounting for 43% of cases in a study of 72 patients.
  • Frontal fibrosing alopecia and Graham-Little syndrome are considered variants of lichen planopilaris.
  • These conditions often affect women aged between 40 and 60, more than men.
  • About half of the patients may develop symptoms of lichen planus that can affect the skin, mucous membranes, or nails.

Signs and Symptoms of Lichen Planopilaris

Lichen planopilaris is a condition that causes hair loss. The rate and pattern with which it happens can vary greatly. Some people may experience slow, gradual hair loss, while others might lose hair rapidly. The most common symptom is having several patches of hair loss scattered throughout the scalp. These areas are often red and flaky. Some people’s hair loss may be similar to central centrifugal alopecia or Brocq alopecia. The hairless areas can appear anywhere on the scalp, be either single or multiple and be concentrated or spread out. The areas of hair loss are usually surrounded by red and flaky skin.

A test in which hair is gently pulled to see if it comes out may indicate active disease in need of treatment if hairs are easily removed. Symptoms can include severe itching, burning, pain, and tenderness. Lichen planus, a related condition, can affect the nails, skin, and mucous membranes before, during, or after scalp involvement. Trichoscopy, a special kind of hair examination, of active lichen planopilaris usually shows perifollicular scaling, a unique feature of this condition. When the condition is not active, the trichoscopy will show small, irregular, white patches where there are no hair follicles, known as fibrotic white dots. There may also be large white areas showing fibrotic changes.

Testing for Lichen Planopilaris

The diagnosis of a certain disease is not only based on the signs and symptoms you are experiencing but also on the connection between those symptoms and the laboratory results. A highly specific type of test called a 4-mm-deep punch biopsy can be conducted. During this procedure, a small piece of your skin will be collected.

This small skin sample will then be prepared for examination under a microscope, a process known as horizontal sectioning, and will also be stained with a particular dye known as hematoxylin-eosin. This technique helps doctors to observe the microscopic details of the skin sample, aiding in diagnosis.

The ideal place from where the skin sample should be taken is an area where you are experiencing symptoms. This could be a part of your skin with hair, that is red around the hair follicles (or perifollicular erythema) and has flaky skin around hair follicles (or perifollicular scale). It’s also important that this symptomatic area is at the edge of a hairless patch and when a gentle pull on the hair results in the hair coming out, which is what a positive anagen pull test means.

Treatment Options for Lichen Planopilaris

The main goals of treating hair loss caused by a disease like primary cicatricial alopecia are reducing further hair loss, controlling the symptoms and stopping the disease from causing more scarring. Unfortunately, once hair loss has happened because of this disease, it cannot regrow since it’s not possible to completely get rid of the inflammation caused by the disease.

As there’s no consistent way to measure how the disease is advancing, the treatment chosen is generally based on the severity of your symptoms and your comfort level with the treatment. It’s also worth mentioning that the length of time you will need to continue treatment depends on how well your body responds to it and how often your symptoms come back. The doctors usually suggest avoiding any process that could harm the hair, like coloring or curling it.

Many patients believe that how often they wash their hair may lead to more hair loss, but this isn’t true. The treatment often involves strong corticosteroids and a topical drug called tacrolimus. These are usually the first treatments suggested for all types of primary cicatricial alopecia.

In addition, antimalarial drugs are frequently used for a specific type of this disease, lichen planopilaris. Another drug called hydroxychloroquine might be used at a general dose. You might start to see improvements within 6 months of using it. Minoxidil is another drug commonly used, which can help make the most of hair growth from hair follicles that are still active. In recent times, JAK inhibitors drugs such as baricitinib and tofacitinib have also been used to treat lichen planopilaris.

To keep a track of how your body is responding to treatment and document how your disease is progressing, doctors may use a new system of scoring called the lichen planopilaris activity index. This system uses number values assigned to symptoms (itching, pain, burning), signs of the disease (redness, redness around hair follicles, scales around hair follicles) and how active the disease is (checked through anagen pull test). It also measures how much the disease is spreading comparing to the pre-treatment phase.

If you have been experiencing a long-term issue of a scaly scalp, it might originally be thought of as a condition called seborrheic dermatitis. However, there are other possible conditions that could be the cause. For example, if you suddenly notice patches of hair loss on your scalp, it might be a condition known as alopecia areata. But, if this hair loss is only partial, and you can see redness and scaly skin around the hair follicles in the affected area, then lichen planopilaris could be the actual condition.

What to expect with Lichen Planopilaris

The outlook for a condition called lichen planopilaris can be different for different people. This difference is based on several things, such as how severe the condition is, how much of the scalp is affected, what other symptoms are present, how well the treatment works, and individual characteristics of the patient.

Usually, hair loss from this condition is considered a long-lasting and worsening issue that can lead to significant health problems. This is particularly true when the disease is widespread and severe.

Possible Complications When Diagnosed with Lichen Planopilaris

Lichen planopilaris can lead to some potential complications:

  • Permanent hair loss: The condition can destroy the hair follicles, leading to scarring and irreversible hair loss in the areas of the scalp that are affected.
  • Scarring and fibrosis: This condition’s chronic inflammation can cause significant scarring and fibrosis within the scalp’s affected tissue. This process may result in a tight scalp, pain, discomfort, and may also impact the functionality of remaining hair follicles.
  • Follicular hyperkeratosis: Lichen planopilaris can lead to an accumulation of keratin around the hair follicles, leading to a condition known as hyperkeratosis. This results in rough, scaly patches on the scalp, leading to increased itching and irritation, and further inflammation.
  • Secondary infections: The persistent inflammation and compromised skin may expose the affected individuals to additional bacterial or fungal infections in the scalp area. These infections may increase existing symptoms like itching, pain, and inflammation and may require additional treatments such as antibiotics or antifungal medications.
  • Psychological impact: The evident changes caused by lichen planopilaris, such as hair loss, scarring, and scalp abnormalities, can severely impact a person’s self-esteem, leading to feelings of anxiety and depression. Consequently, addressing the psychological effects of lichen planopilaris is a crucial part of a comprehensive care plan for these patients.
  • Ocular involvement: In rare instances, lichen planopilaris can affect the eyebrows and eyelashes, leading to loss of these features and creating cosmetic concerns and potential eye irritation or discomfort.

In conclusion, early diagnosis, treatment adherence, and close monitoring by dermatologists and a multidisciplinary team are vital for optimal results in individuals struggling with lichen planopilaris.

Preventing Lichen Planopilaris

It’s important for patients with lichen planopilaris (a condition that causes inflammation and hair loss on the scalp) to understand their condition and how to manage it. Educating patients about their condition can help them stick to their treatment plans and avoid further complications. Being aware of the symptoms like itchiness, burning, and hair loss can help indicate when the condition may be worsening and when to seek medical help.

It’s critical that patients reach out to their doctors if they notice anything unusual with their scalp or hair. Once they have started treatment, it’s important that they follow their doctor’s instructions carefully. Lastly, it’s crucial not to overlook the emotional impact of the disease. Providing guidance and emotional support can help patients cope better with their condition.

Frequently asked questions

Lichen planopilaris is a condition that causes inflammation and permanent hair loss, mainly on the scalp but can also affect other areas where hair grows like eyebrows and pubic area.

Lichen planopilaris is the most common type of cicatricial alopecias, accounting for 43% of cases in a study of 72 patients.

Signs and symptoms of Lichen Planopilaris include: - Hair loss that can occur slowly or rapidly - Multiple patches of hair loss scattered throughout the scalp - Red and flaky areas where hair loss has occurred - Similar hair loss patterns to central centrifugal alopecia or Brocq alopecia - Hairless areas that can be single or multiple and concentrated or spread out - Surrounding red and flaky skin around the areas of hair loss - Severe itching, burning, pain, and tenderness - Possible involvement of nails, skin, and mucous membranes in lichen planus - Perifollicular scaling seen in trichoscopy of active lichen planopilaris - Fibrotic white dots and large white areas showing fibrotic changes in trichoscopy when the condition is not active.

We don't know the exact cause of Lichen Planopilaris, but it is often considered an autoimmune disorder where the body's immune system mistakenly targets specific parts of the hair follicles.

A doctor needs to rule out the following conditions when diagnosing Lichen Planopilaris: 1. Seborrheic dermatitis 2. Alopecia areata

The types of tests needed for Lichen Planopilaris include: - 4-mm-deep punch biopsy: A small piece of skin is collected and examined under a microscope to observe microscopic details of the skin sample. - Horizontal sectioning: The skin sample is prepared for examination under a microscope. - Staining with hematoxylin-eosin dye: This technique helps doctors observe the microscopic details of the skin sample. - Anagen pull test: A gentle pull on the hair to see if it comes out, which is a positive indication of Lichen Planopilaris. - Lichen planopilaris activity index: A scoring system that measures symptoms, signs of the disease, and disease activity to track progress and response to treatment.

Lichen Planopilaris is treated using various methods. The main goals of treatment are to reduce further hair loss, control symptoms, and prevent more scarring. The treatment chosen depends on the severity of symptoms and the patient's comfort level. Strong corticosteroids and a topical drug called tacrolimus are commonly used as the first treatments for all types of primary cicatricial alopecia. Antimalarial drugs, such as hydroxychloroquine, may be used specifically for lichen planopilaris. Minoxidil can help promote hair growth from active hair follicles. JAK inhibitors drugs, like baricitinib and tofacitinib, have also been used to treat lichen planopilaris. Doctors may use a scoring system called the lichen planopilaris activity index to track the response to treatment and the progression of the disease.

When treating Lichen Planopilaris, there can be potential side effects and complications, including: - Permanent hair loss: The condition can destroy hair follicles, leading to scarring and irreversible hair loss in affected areas of the scalp. - Scarring and fibrosis: Chronic inflammation from the disease can cause significant scarring and fibrosis in the affected scalp tissue, resulting in a tight scalp, pain, discomfort, and potential impact on remaining hair follicles. - Follicular hyperkeratosis: Lichen Planopilaris can lead to an accumulation of keratin around hair follicles, resulting in rough, scaly patches on the scalp, increased itching, irritation, and further inflammation. - Secondary infections: Persistent inflammation and compromised skin may expose individuals to additional bacterial or fungal infections in the scalp area, which can worsen symptoms and require additional treatments. - Psychological impact: The visible changes caused by Lichen Planopilaris, such as hair loss and scarring, can have a significant impact on self-esteem, leading to feelings of anxiety and depression. - Ocular involvement: In rare cases, Lichen Planopilaris can affect the eyebrows and eyelashes, leading to their loss and potential cosmetic concerns and eye irritation or discomfort.

The prognosis for Lichen Planopilaris can vary depending on several factors, including the severity of the condition, the extent of scalp involvement, the presence of other symptoms, the effectiveness of treatment, and individual patient characteristics. In general, hair loss from Lichen Planopilaris is considered a long-lasting and worsening problem that can lead to significant health issues, especially when the disease is widespread and severe.

Dermatologist.

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