Lichen Planopilaris

Lichen planopilaris is a condition that causes inflammation in the scalp, leading to patches of permanent hair loss through scarring. While the exact cause remains unclear, early treatment is crucial to prevent further damage, as lost hair cannot regrow once the follicles are destroyed.

Table of contents

• What is Lichen Planopilaris?
• Types of Lichen Planopilaris
• Who Is Affected?
• Causes and Why It Happens
• Signs and Symptoms
• How It Is Diagnosed
• Treatment Options
• Outlook and Living with the Condition

What is Lichen Planopilaris?

Lichen planopilaris (LPP) is an inflammatory condition that targets the scalp and hair follicles. It belongs to a group of conditions called scarring alopecias, which means it causes permanent hair loss by destroying the hair follicles and replacing them with scar tissue^[1]. When hair follicles are scarred, they cannot produce new hair, making the hair loss irreversible.

The condition is considered a form of lichen planus, a broader inflammatory disease that can affect the skin, nails, and mucous membranes. However, in lichen planopilaris, the inflammation specifically targets areas where hair grows^[2]. Though relatively rare, LPP is recognized as one of the leading causes of scarring hair loss, accounting for about 43% of such cases in some studies^[2].

L66.1

Lichen planus follicularis, Follicular lichen planus, LPP

• Scalp
• Hair follicles
• Eyebrows
• Eyelashes
• Pubic area
• Armpits

Types of Lichen Planopilaris

There are three main types of lichen planopilaris, each with distinct patterns of hair loss and affected areas^[1]:

• Classic lichen planopilaris: This form causes patchy areas of hair loss on the scalp, typically affecting the top and sides. Scarring develops in these patches, leaving bald areas that may appear smooth and shiny.
• Frontal fibrosing alopecia (FFA): This type appears as a band of hair loss along the front hairline and sides of the scalp, typically 1 to 8 centimeters wide. It commonly affects eyebrow hair as well and occurs most often in women after menopause^[4].
• Graham-Little-Piccardi-Lassueur syndrome (GLPLS): This is the least common variant, combining patchy scalp hair loss with hair thinning in the armpits and pubic area. People with this type may also develop small, rough bumps on the skin around hair follicles and a bumpy rash on the body and limbs^[1].

Who Is Affected?

Lichen planopilaris most commonly affects women between the ages of 40 and 60, though it can occur in adults of any age and sex^[1]. Men can develop the condition as well, but it is between 2 and 5 times more common in women^[7]. In rare cases, the condition can affect young adults and children^[4].

The condition is more frequently observed in people with lighter skin tones compared to those with darker skin^[4]. There is also a noted association between LPP and hypothyroidism (underactive thyroid gland)^[4]. Nearly half of people with lichen planopilaris also develop symptoms of lichen planus on other parts of the body, such as the skin, mouth, or nails^[1].

Causes and Why It Happens

The exact cause of lichen planopilaris remains unclear, but researchers believe it is an autoimmune disorder. This means the body’s immune system mistakenly attacks its own tissues—in this case, the hair follicles^[1]. Specifically, immune cells called T-lymphocytes target and destroy a part of the hair follicle known as the “bulge,” which contains stem cells essential for hair growth. When these stem cells are damaged, permanent hair loss results^[4].

Some people with LPP have genetic differences in a protein called MHC class I, which helps the immune system distinguish between the body’s own cells and foreign invaders. In these individuals, the immune system fails to recognize hair follicles as part of the body and attacks them^[4]. Recent research has also found that people with LPP have lower levels of a protein called PPAR gamma, which is important for maintaining healthy hair follicles and oil-producing glands in the scalp. Low levels of this protein may trigger the immune system to attack the glands and nearby stem cells^[4].

Hormones may also play a role in the development of LPP, particularly since the condition often affects postmenopausal women and can respond to treatments that block certain hormones^[4]. In rare cases, the condition may be triggered by certain medications^[3].

It is important to note that lichen planopilaris is not contagious and cannot be spread from person to person^[1]. It is also not typically inherited, though there may be genetic factors that increase the risk of developing the condition^[7].

Signs and Symptoms

The symptoms of lichen planopilaris can develop gradually or appear more suddenly. In the early stages, people may experience discomfort on the scalp before visible hair loss occurs^[1].

Common symptoms include^[1][3]:

• Patches of hair loss on the scalp, which may be smooth and shiny where scarring has occurred
• Redness around the base of individual hairs at the edges of bald patches
• Scaling or flaking of the skin around hair follicles
• Itching, burning, pain, or tenderness on the scalp
• Small, rough bumps that feel spiny to the touch around hair follicles
• Hair that pulls out easily from affected areas

The most common areas affected are the top, sides, front, and lower back of the scalp. Over time, small affected areas may merge to form larger irregular patches of hair loss^[3]. In some cases, symptoms may be absent despite visible changes, while in others, discomfort can be significant^[3].

In frontal fibrosing alopecia, the hairline gradually recedes in a band-like pattern. Eyebrow and body hair may also be lost^[4]. In Graham-Little-Piccardi-Lassueur syndrome, hair loss occurs on the scalp along with thinning in the armpits and pubic area, and a bumpy rash may appear on the skin^[7].

How It Is Diagnosed

Diagnosing lichen planopilaris begins with a thorough examination of the scalp by a healthcare provider, usually a dermatologist. The doctor will look for characteristic signs such as patches of hair loss, redness, and scaling around hair follicles^[1]. They will also ask about your symptoms, health history, and any medications you are taking.

Because lichen planopilaris can resemble other conditions that cause hair loss, additional tests are often needed to confirm the diagnosis^[1]:

• Dermoscopy or trichoscopy: The doctor uses a magnifying device with a light to closely examine the scalp and hair follicles. In LPP, this examination typically reveals absent follicles, white dots, scaling around follicles, and redness^[3].
• Scalp biopsy: This is the most definitive test. During a biopsy, the doctor removes a small sample of skin from the affected area, usually under local anesthetic. The sample is sent to a laboratory where it is examined under a microscope. The biopsy can confirm the presence of inflammation and scarring typical of lichen planopilaris^[1][3]. The biopsy should include hairs with surrounding redness and scale from the edge of a hair loss area for the best chance of diagnosis.

The doctor will also examine your mouth, nails, and skin for signs of lichen planus elsewhere on the body, as this can support the diagnosis^[3]. However, it is not always possible to confirm the diagnosis through biopsy alone, especially if only patchy scarring and hair loss are present without active inflammation^[3].

Conditions that may look similar to lichen planopilaris include discoid lupus erythematosus, central centrifugal cicatricial alopecia, folliculitis decalvans, alopecia areata, and pseudopelade of Brocq^[3].

Treatment Options

Treatment for lichen planopilaris aims to control inflammation, slow or stop hair loss, and relieve symptoms such as itching and burning. It is important to understand that once hair follicles are destroyed and replaced with scar tissue, hair cannot regrow in those areas. This is why early treatment is essential to preserve remaining hair^[1][3].

Treatment success varies from person to person, and there is no single cure that works for everyone. Some people may not respond to any treatment^[7]. Your doctor will work with you to find the most appropriate approach based on the extent of your condition and your symptoms.

Topical treatments (applied to the skin):

• Corticosteroid creams, lotions, gels, or mousses: These are often used as first-line treatment. They help reduce inflammation and can improve itching and redness. Because scalp skin is thicker than facial skin, it can tolerate stronger steroid preparations. However, long-term use can cause skin thinning, so they must be used carefully^[1][7].
• Corticosteroid injections (intralesional steroids): For small, localized areas of active hair loss, steroid injections directly into the scalp can be effective. These injections can be uncomfortable and may carry a higher risk of side effects, such as skin thinning or changes in skin color^[7].
• Calcineurin inhibitors (tacrolimus or pimecrolimus): These are creams or ointments that reduce inflammation without the risk of skin thinning associated with steroids. They are often used as an alternative or in combination with other treatments^[9].

Oral medications (taken by mouth):

• Anti-malarial drugs (such as hydroxychloroquine): Originally used to treat malaria, these medications also help control inflammation in certain skin conditions, including LPP. They are commonly prescribed for moderate to severe cases^[1][14].
• Antibiotics (such as doxycycline): Some antibiotics have anti-inflammatory properties and can help reduce symptoms^[1][9].
• Corticosteroids (such as prednisone): Short courses of oral steroids may be used for rapidly progressing disease to quickly reduce inflammation. Long-term use is generally avoided due to potential side effects^[9].
• Immunosuppressive medications: In more severe or resistant cases, drugs such as cyclosporine, mycophenolate mofetil, or methotrexate may be prescribed to suppress the immune system and reduce inflammation^[9].
• Retinoids: These medications, related to vitamin A, help regulate skin cell growth and may be used in some cases^[1].
• Pioglitazone: This medication, typically used for diabetes, has shown promise in helping some patients achieve remission of LPP at doses of 15 to 45 mg daily. Patients should be informed about the labeled use, mixed evidence for effectiveness, and warnings about potential side effects^[9].
• JAK inhibitors (such as tofacitinib): These are newer medications that target specific pathways in the immune system. Early studies suggest they may be helpful for people whose condition has not responded to other treatments^[9].

Other treatments:

• Low-level laser therapy: This uses special light waves to treat scalp inflammation and may help reduce symptoms^[1].
• Platelet-rich plasma injections: Some studies have explored this treatment, where a concentrated form of the patient’s own blood is injected into the scalp, though results have been variable^[9].
• Minoxidil: While it does not control the underlying inflammation, minoxidil (a hair growth stimulant) may be used to maximize hair regrowth in patients who also have pattern hair loss, which affects nearly half the population^[9].

Your doctor may take photographs or measurements at clinic visits to help monitor whether the treatment is controlling the disease^[7]. It is important to take medications as prescribed and attend regular follow-up appointments. You do not have to pursue treatment if you choose not to, and you may want to discuss all options with your doctor, family, or friends^[7].

Outlook and Living with the Condition

Lichen planopilaris is usually a long-term condition, but in most cases, it eventually becomes inactive on its own. However, the timing of this is unpredictable, and the condition can follow a relapsing course with periods of worsening and improvement^[7][9]. Once hair follicles are destroyed and replaced by scar tissue, the hair loss is permanent, which is why early treatment to preserve remaining hair is so important.

Living with lichen planopilaris can affect emotional well-being and quality of life due to visible hair loss and ongoing symptoms. It is normal to experience feelings of frustration, sadness, or anxiety. Connecting with support groups or mental health professionals can be very helpful^[3].

Practical tips for managing the condition include:

• Follow your treatment plan carefully and attend regular dermatology appointments to monitor disease activity
• Use gentle hair care products and avoid harsh treatments or hairstyles that pull on the hair, as these can worsen hair loss
• Consider cosmetic options such as wigs, hairpieces, scarves, or hats if you wish to cover areas of hair loss. If you choose to use hair extensions, use caution to avoid traction that could damage remaining hair^[9]
• Seek information and support from patient organizations dedicated to scarring alopecia
• Remember that your worth is not defined by your hair, and that you are managing a challenging medical condition with courage

While there is no cure for lichen planopilaris, many people find that with appropriate treatment, they can control symptoms and prevent further hair loss. Ongoing research into the causes and treatment of the condition continues to offer hope for better management options in the future.