What Is Lichen Planopilaris?
Lichen planopilaris (LPP) is a rare inflammatory disease causing permanent hair loss in affected individuals. This condition is a variation of lichen planus, a widespread skin condition resulting in characteristic reddish-purple body rashes. The predominant symptom of lichen planopilaris is patchy, gradual, irreversible scalp hair loss, which can also affect other hair-bearing areas like the pubic area and eyebrows. It causes inflammation, which harms the hair follicles and results in scarring and hair loss.
Because of inflammation, the hair follicles are frequently seen to be reddish and scaly. People can occasionally feel discomfort and irritation or show no symptoms at all. Three subtypes of lichen planopilaris are usually recognized: Graham-Little syndrome, frontal fibrosing alopecia, and classic lichen planopilaris. Treatment for lichen planopilaris consists of using antimalarial medications, topical tacrolimus, corticosteroids, and newly developed therapies, including JAK inhibitors. Furthermore, considering the major impact on look and lifestyle, long-term treatment techniques, interdisciplinary teamwork, and patient education are essential for enhancing functionality.
What Are the Clinical Variants of Lichen Planopilaris?
There are multiple clinical variations of Lichen planopilaris, each with unique clinical and histological characteristics. These variations include, among others,
- Frontal fibrosing alopecia.
- Classic lichen planopilaris.
- Graham-Little syndrome.
What Is Frontal Fibrosing Alopecia?
Frontal fibrosing alopecia primarily affects postmenopausal women. The main characteristic of frontal fibrosing alopecia is gradual frontotemporal hairline regression.
Symptoms - The typical presentation of frontal fibrosing alopecia is a homogeneous linear band of hair loss that recedes the frontal hair line along the front and sides of the scalp hair margin. Examples of atypical loss patterns are a diffuse zig-zag pattern, a pseudo-fringe sign, or continuous involvement throughout the entire hair edge in both the front and back. Common early symptoms include itching and soreness, which can appear before any noticeable loss of hair density. Rashes on the face are an additional possible early indicator. These can appear as diffuse erythema or red spots around hairs or as skin-colored or yellowish follicular papules on the forehead and temples.
Diagnosis -In order to rule out frontal fibrosing alopecia, a skin biopsy can be necessary. Lichen planopilaris and frontal fibrosing alopecia share the same histological characteristics. Histologically, frontal fibrosing alopecia causes irreversible hair loss by causing perifollicular lymphocytic inflammation and fibrosis.
Treatment -
- Finasteride or Dutasteride - According to studies, adding Finasteride or Dutasteride to the therapy regimen can stop more hair loss.
- Corticosteroids - The dermatologist may inject this drug into the scalp to lessen the inflammation brought on by FFA. Dermatologists may also recommend at-home corticosteroid therapy.
- Minoxidil - Applying Minoxidil on the scalp and other medicines, such as corticosteroids or Finasteride might help improve hair loss.
- Hydroxychloroquine - This drug may lessen discomfort and itching.
- Laser Therapy - This procedure may lessen the itchiness and puffiness on the scalp. Since it is a more recent therapy option, additional research is required.
What Is Classic Lichen Planopilaris?
Classic lichen planopilaris typically presents with perifollicular scale, erythematous follicular papules, and variable degrees of hair loss.
Symptoms - The characteristic symptom of classic lichen planopilaris is alopecia, or hair loss, which usually begins as a small, smooth, shining patch on the scalp. Many individuals report pain or discomfort in the impacted scalp regions. There may be noticeable skin scaling in the afflicted areas, which may appear red and swollen. As the illness worsens, permanent hair loss and scalp scarring may happen.
Causes - Although the exact cause of classic lichen planopilaris is unknown, the condition is considered autoimmune. When an autoimmune disease occurs, the immune system mistakenly targets and attacks the body's tissues; in this case, the hair follicles are attacked. Environmental variables and genetic predispositions may also cause the disease.
Diagnosis - During diagnosis, the scalp and afflicted areas are thoroughly examined. To find the distinctive characteristics of lichen planopilaris, a biopsy is performed, and a sample of the affected skin is obtained and studied under a microscope. To rule out other disorders or gauge the severity of the sickness, doctors may occasionally prescribe blood tests or additional imaging investigations.
Treatment -
- Corticosteroid - When administered topically to the affected scalp, corticosteroid creams or ointments might help lessen irritation and inflammation. In more severe situations, intralesional injections of corticosteroids into the scalp lesions may be employed.
- Immunosuppressive - In cases of broad or resistant disease, immunosuppressive drugs may be recommended.
- Light therapy - UV (ultraviolet) light phototherapy has been used, although not always helpful.
- Monitoring and Follow-up - Regular monitoring is crucial to evaluating the course of the disease, the effectiveness of the treatment, and any possible drug adverse effects.
What Is Graham-Little Syndrome?
Graham-Little syndrome is an extremely uncommon type of lichen planopilaris and it presents with a trio of scarring alopecia, noncicatricial alopecia (loss of hair without any scarring), and keratosis pilaris (patches of rough bumps on the skin due to blocking in the opening of hair follicles). The characteristic features of Graham-Little syndrome are follicular papules, patchy hair loss, and keratotic papules on the extremities. Women between the ages of 30 and 70 are most frequently affected by Graham-Little syndrome, with middle-aged postmenopausal women being the most affected.
Causes - It is unknown what causes Graham-Little syndrome. It could be an immunological condition. GLPLS is probably caused by mechanisms including IFN-γ dysregulation and altered integrin expression, which are both seen in lichen planopilaris. Uncertainty concerns the significance of a few reported rare familial cases and relationships.
Symptoms - The onset of symptoms is slow, with hyperkeratotic papules frequently manifesting prior to hair loss. Over time, these papules could develop into hyperkeratotic plaques. Three clinical characteristics associated with Graham-Little syndrome include increasing scalp hair loss with spotty scarring, non-scarring hair thinning in the groin and armpits, and hair follicle-based rough, spiky bumps.
Diagnosis - For Graham-Little syndrome, a comprehensive physical examination, and skin biopsy results are the main diagnostic techniques. Histological examination of hyperkeratotic papules and cicatricial areas revealed characteristics of lichen planopilaris, showing perifollicular inflammation that results in the destruction of pilosebaceous tissue.
Treatment - Managing GLPLS is still difficult as no well-accepted treatment plan exists.
- Corticosteroids: These are used intralesionally, systemically, and topically to lower inflammation and slow the course of the illness.
- Immunosuppressive Agents: Immune response suppression is possible with medications including Methotrexate, Cyclosporine, and Mycophenolate mofetil.
- Hydroxychloroquine: Using this has shown some degree of success.
- Retinoids: Actitretin can aid in symptom management in certain people.
Conclusion
Lichen planopilaris and its variations are a range of autoimmune diseases that mostly affect the hair follicles, resulting in variable degrees of scalp inflammation and hair loss. While these disorders range in their specific areas of involvement and clinical manifestations, they share common lichenoid inflammatory histological characteristics. Clinical assessment and biopsy frequently verify the diagnosis of LPP and its variations. These disorders are challenging to treat, and systemic and topical treatments are usually combined. Early diagnosis and management are essential to manage LPP and its variations, reduce the severity of hair loss, and enhance quality of life.
