Lichen Planopilaris: Causes, Symptom, and Treatment RichFeel
Lichen Planopilaris is a chronic inflammatory condition that affects the scalp. It results in patches of hair loss on the scalp that may cause itching and burning. Lichen Planopilaris is a form of lichen planus that mostly affects women in young adulthood. Lichen Planopilaris may cause permanent hair loss and scarring due to primary Cicatricial Alopecia. The distinct feature of this disease is hair loss in patches that occur in many different patterns. This article explains the causes, symptoms and treatment options for Lichen Planopilaris.
What is Lichen Planopilaris?
Lichen Planopilaris is an inflammatory scalp disease that results in permanent hair loss. This disorder is said to be a variant of Lichen Planus, a common skin disorder causing distinct reddish purple rashes on the body. When Lichen Planus affects the scalp and causes bald patches, it is called Lichen Planopilaris. It damages the hair follicles by causing inflammation and leading to hair loss and scarring. The hair follicles are often observed to be reddish and scaly due to inflammation. Individuals sometimes experience pain and itching or are altogether asymptomatic. Lichen Planopilaris is generally said to have three subtypes: Classic Lichen Planopilaris, Frontal Fibrosing Alopecia, and Graham Little Syndrome. Classic Lichen planopilaris presents a patch on the scalp whereas Frontal Fibrosing Alopecia presents as a receding frontal hairline and scarring at the frontal scalp, at times eyebrows are affected too. Graham Little Syndrome represents Cicatricial Alopecia on the scalp causing permanent hair loss, and non-scarring alopecia on other regions of the body. The cause of the disease is not clearly understood but the primary mechanism remains the same, where the hair follicle gets inflamed causing hair loss due to scarring of follicle.
Who gets Lichen Planopilaris and why?
Lichen Planopilaris affects individuals across all ages; young men and women are likely to be affected as compared to children and elderly. However, women in the age group of 40 to 60 years of age are predominantly affected. This disorder often occurs in association with Lichen Planus, which affects the skin, nails and different mucosal membranes of the body. About 50% of individuals with Lichen Planopilaris show development of Lichen Planus. The exact cause of the disease is not known but there have been hypotheses that indicate autoimmunity as the probable cause. It is the most common reason of scarring alopecia. This disorder is not hereditary but there are certain genes that predispose an individual to the development of this disease.
Clinical features of Lichen Planopilaris
The key clinical features of Lichen Planopilaris are the presence of lesions with perifollicular erythema and perifollicular scale on the scalp. The lesions are reddish and show a scaly or crusty appearance mostly towards the periphery. The hair follicle is affected and shows inflammation. There are bald patches observed on the scalp that range from few to many anywhere on the scalp. These bald patches are often smooth and whitish in color and the pattern of hair loss is greatly varied. The small patches often fuse to form larger region of hair loss. Other hair bearing regions are seldom affected. Distinct absence of follicular ostia is noted. The variant of Lichen Planopilaris, Frontal Fibrosing Alopecia is often observed in postmenopausal women and shows recession and scarring of frontal hairline. In Graham Little Syndrome patchy loss of hair with scarring is observed on the scalp along with loss of hair on other body parts.
What are the symptoms of Lichen Planopilaris?
The symptoms of Lichen Planopilaris differ from individual to individual. Mainly the vertex and crown region is affected. A very evident symptom is hair loss and occurrence of bald patches. The lesions also show scaling towards the periphery. The primary symptoms exhibited by patients include pain and itching accompanied by redness and certain discomfort. Patients suffering from this disease often show presence of Lichen Planus, which affects the skin, and mucous membranes of the body.
Causes of Lichen Planopilaris
The precise cause of Lichen Planopilaris is not known and thus this makes the treatment a challenge. There are certain suspicions that point to autoimmunity as the cause of this disease. The immune system of the body reacts in an abnormal manner and mistakenly attacks the healthy tissues of the body. In this case the cells of the immune system called lymphocytes attack the hair follicles and destroy the stem cells. This causes the hair to fall off and a scar is formed. Various environment factors can trigger this abnormal immune response that results in hair follicle inflammation and hair loss. This type of hair loss is usually irreversible and permanent and there is not much scope for re-growing the hair. Presence of certain genes put a person at a risk of developing this disease.
How is Lichen Planopilaris diagnosed?
The diagnosis of Lichen Planopilaris requires a careful dermatologic examination of the scalp. All the clinical, histological and pathological observations together give a conclusive diagnosis. A biopsy is very crucial in this diagnosis. The biopsy specimen must be taken from an active lesion that displays perifollicular erythema and perifollicular scaling. Anagen Pull test is also done to confirm the diagnosis. In addition to this examination of nails, skin and various mucus membranes are done to look for underlying Lichen Planus. Trichoscopy, a recent non-invasive diagnostic method is also employed to investigate the scalp for the various clinical presentations of the disease.
Treatment of Lichen Planopilaris
There is no specific cure for Lichen Planopilaris, but there are treatments that help to relieve the symptoms to an extent and slow down the progression of the disease. An early diagnosis provides better scope of treatment. Any hair loss due to scarring alopecia cannot be revived and is permanent. The basic aim is to slow the pace of the disease. The usual course of treatment includes oral, topical and intralesional medications. Corticosteroids are often administered orally, topically through an intralesional injection. Hydrochloroquine is an antimalarial medication which helps reduce inflammation. Immunosuppressants like Methotrexate are also prescribed. Pioglitazone is an antidiabetic, which is also used in the treatment of this disease. The efficacy of these treatments is observed differently in different persons. When the disease has reached its burnt-out stage, cosmetic surgery and hair transplantation can be considered. Scope of recovery is very little and relapse is a common occurrence.
Complications of Lichen Planopilaris
Complications of Lichen Planopilaris include development of Cicatricial alopecia that causes scarring and irreversible hair loss. Many times Lichen Planopilaris is accompanied by Lichen Planus. Lichen Planus affects the skin, nails and various mucous membranes. It causes characteristic reddish purple flat rashes on the body. Skin pigmentation and disfiguration is also associated with Lichen Planopilaris.
What does Lichen Planopilaris look like?
Lichen Planopilaris shows presence of smooth bald patches that have a shine. These bald patches go on to fuse and form bigger patches of bald regions. The hair follicles are inflamed and show a red and scaly appearance. These patches appear anywhere on the scalp. Perifollicular erythema and perifollicular scales are commonly observed in Lichen Planopilaris. Perifollicular scale is a distinct feature of this disease. Development of Cicatricial alopecia resulting in hair loss and scarring is also observed. Other related variants include Follicular Lichen Planus, Frontal Fibrosing Alopecia, and Graham Little syndrome. Each has distinct patterns of hair loss and specific symptoms. Frontal Fibrosing alopecia shows scarring and hair loss in the frontal hair line where as Graham Little syndrome shows bald patches all over the scalp with itchy rash. These different patterns help diagnose the disorders.
Risk factors of Lichen Planopilaris
The risk factors of Lichen Planopilaris includes age, mostly middle-aged women are prone to developing this disease. Certain genes predispose a person to Lichen Planopilaris. Caucasian adults are reported to have a higher incidence of this disease as compared to other populations. Individuals with a Hepatitis C infection go on to develop this disease. Environmental factors like certain medicines and toxins can also trigger the development of this disease. It is also observed that a certain scalp trauma can cause this disease.
What is the outcome of Lichen Planopilaris?
Lichen Planopilaris spontaneously resolves, however the hair loss is permanent. Relapse is commonly observed and the disease can return even after years of being in remission. This disease has a slow course of progression and is erratic making it highly unpredictable.