Scarring Alopecia: Causes, Symptoms, Diagnosis, and Treatment

Scarring alopecia, also known as cicatricial alopecia, encompasses a group of hair loss disorders characterized by the permanent destruction of hair follicles and their replacement with scar tissue. This condition can lead to irreversible hair loss and can affect any area of the body with hair, though it most commonly affects the scalp.

Understanding Scarring Alopecia

Scarring alopecia is a rare condition affecting approximately 7% of individuals seeking professional help for hair loss. Unlike non-scarring alopecias, where hair follicles may be damaged but retain the potential for regeneration, scarring alopecia involves the complete and irreversible destruction of hair follicles. In nonscarring alopecia, hair may fall out or get thinner, but your hair follicle isn’t destroyed. So nonscarring alopecia may be temporary, and your hair can sometimes grow back. Androgenetic alopecia, also called male or female pattern baldness, is the most common type of nonscarring alopecia.

Primary vs. Secondary Scarring Alopecia

Cicatricial alopecias are classified as primary or secondary.

• Primary Scarring Alopecia: In primary cicatricial alopecias, the hair follicle is the direct target of a destructive inflammatory process. This form of alopecia is caused by an inflammatory or autoimmune disorder that directly targets and destroys the hair follicles. These disorders are often the result of underlying inflammation or an autoimmune response.

• Secondary Scarring Alopecia: In secondary cicatricial alopecias, destruction of the hair follicle is incidental to a non-follicle-directed process or external injury, such as severe infections, burns, radiation, or tumors. This type of alopecia is a side effect of injury or damage to your skin. Hair loss might result from burns, infections, radiation or tumors.

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This article primarily focuses on primary cicatricial alopecia.

Types of Primary Scarring Alopecia

Primary cicatricial alopecia can be further categorized based on the type of inflammatory cells that infiltrate the hair follicles: lymphocytic, neutrophilic, and mixed.

Lymphocytic Primary Cicatricial Alopecia:

• Lichen Planopilaris (LPP): The most common cause of cicatricial alopecia, accounting for at least 10% of cases. It is a clinical variant of lichen planus, and both conditions can occur simultaneously. Lichen planopilaris is characterized by multifocal or central patches with follicular hyperkeratosis and perifollicular erythema on the scalp. Skin atrophy is common in lichen planopilaris. Lichen planopilaris is generally treated with a combination of topical, intralesional, and oral therapies. Topical corticosteroid lotions will control itching and burning. Potent topical corticosteroid treatment for 12 weeks may halt disease progression but tends to produce substantial atrophy. Intralesional injection of corticosteroids (e.g., triamcinolone 10 mg/mL) into foci of active inflammation will stop hair loss at the site of injection.
• Frontal Fibrosing Alopecia (FFA): Considered a variant of lichen planopilaris, frontal fibrosing alopecia is characterized by eyebrow alopecia. The management of the frontal variant is similar to ordinary lichen planopilaris. Chiang and colleagues19 and Cho and colleagues20 reported benefit with the use of hydroxychloroquine and mycophenolate.
• Central Centrifugal Cicatricial Alopecia (CCCA): The next most common cause of primary cicatricial alopecia in North America. It is found almost exclusively in people of African descent. Previous hair-care practices such as the use of hot combs, relaxants, and excessive traction have been linked to the pathogenesis of central centrifugal cicatricial alopecia, although many affected women have never used these practices. Central centrifugal cicatricial alopecia and folliculitis decalvans are most common on the vertex and midfrontal scalp. In particular, central centrifugal alopecia may mimic female pattern baldness.
• Discoid Lupus Erythematosus (DLE): A chronic cutaneous condition caused by lupus erythematosus, an autoimmune disease. Dyspigmentation is a feature of chronic cutaneous lupus erythematosus. Chronic cutaneous lupus erythematosus is known to progress with exposure to ultraviolet light, and thus protection of the scalp with a hat, and face and body with broad-spectrum sun-screen should be used on a daily basis. Topical steroids may be effective in many cases, whereas intralesional triamcinolone is effective in most cases. For people with widespread or refractory disease, however, hydroxychloroquine should be considered. Patients usually require 400 mg daily during the summer months, but the dose can be reduced during the cooler seasons. For patients with chronic disease, a second antimalarial, mepacrine (quinacrine), can be added if the response to hydroxychloroquine is unsatisfactory after 6 months of therapy. In patients with resistance to antimalarials, oral retinoids could be used.
• Pseudopelade of Brocq: No treatment has been shown to influence Brocq pseudopelade.

Neutrophilic Primary Cicatricial Alopecia:

• Folliculitis Decalvans: Typically seen in adults, though it can affect adolescent males. Folliculitis decalvans is characterized by pustules and honey-coloured crusting at the periphery of a patch of hair loss. It is most often treated with antibiotics and antiseptics. All patients with folliculitis decalvans should use an antiseptic shampoo. Topical benzoyl peroxide or topical clindamycin will control mild cases. For severe or refractory cases, oral antibiotics will be required. The most common bacteria identified is Staphylococcus aureus.
• Dissecting Cellulitis of the Scalp: Purulent discharge, bogginess, and sinus tract formation can occur in dissecting cellulitis of the scalp. In contrast to folliculitis decalvans, dissecting cellulitis responds well to oral isotretinoin (0.5-1 mg/kg daily).

Mixed Primary Cicatricial Alopecia:

• Acne Keloidalis Nuchae
• Acne Necrotica
• Erosive Pustular Dermatosis of the Scalp

Causes and Risk Factors

The exact cause of primary cicatricial alopecias remains elusive. However, all types involve inflammation directed at the upper part of the hair follicle where the stem cells and sebaceous gland (oil gland) are located. If the stem cells and sebaceous gland are destroyed, there is no possibility for regeneration of the hair follicle, leading to permanent hair loss. Cicatricial alopecias are not contagious.

While the causes of primary scarring alopecia are not well understood, secondary scarring alopecia can arise from:

• Excessive heat or chemicals applied to the scalp for hair styling over months or years.
• Bacterial or fungal infections (less commonly).
• Trauma such as burns.
• Radiation treatments for cancer.
• Tumors.

Certain populations are more susceptible to specific types of scarring alopecia. For instance, central centrifugal alopecia is more prevalent in women of African descent, and dissecting cellulitis primarily affects dark-skinned males.

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Symptoms of Scarring Alopecia

Scarring alopecia generally starts with the appearance of small patches of hair loss that may grow larger over time. The inflammation that destroys the follicle is below the skin surface and there is usually no “scar” seen on the scalp, although the affected scalp is usually left bare and smooth without hair and without the usual pore markings. Affected areas of the scalp may have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Other cases may show little signs of inflammation.

Other symptoms may include:

• Itching or pain in the scalp associated with hair loss.
• A ragged edge around the patch of hair loss.
• Smooth, shiny bald patches.
• Small blisters on the scalp.
• Tenderness.
• Burning sensations.

Diagnosis of Scarring Alopecia

Early diagnosis is essential to prevent further hair loss. Because scalp biopsy is often nondiagnostic, a careful history and examination are needed to diagnose primary cicatricial alopecia. Diagnosis typically involves:

• Physical Examination: A dermatologist will examine the scalp, noting the pattern and extent of hair loss, and looking for signs of inflammation such as redness, scaling, and pustules. On examination, the first thing to establish is that the hair loss is due to cicatricial alopecia. Central centrifugal cicatricial alopecia and folliculitis decalvans are most common on the vertex and midfrontal scalp. In particular, central centrifugal alopecia may mimic female pattern baldness. Eyebrow alopecia is characteristic in frontal fibrosing alopecia. Skin atrophy is common in lichen planopilaris and central centrifugal cicatricial alopecia. Dyspigmentation is a feature of chronic cutaneous lupus erythematosus and central centrifugal cicatricial alopecia. Pustules and honey-coloured crusting at the periphery of a patch of cicatricial alopecia along with tufting of the hair suggest folliculitis decalvans. Purulent discharge, bogginess, and sinus tract formation can occur in dissecting cellulitis of the scalp.
• Scalp Biopsy: A scalp biopsy for the diagnosis of cicatricial alopecia is the necessary first step. Findings of the biopsy, including the type of inflammation present, location and amount of inflammation and other changes in the scalp are necessary to diagnose the type of cicatricial alopecia, determine the degree of activity and to select appropriate therapy. The biopsy specimen is taken with a biopsy punch, which is an instrument that removes a sample of skin about the size and shape of a small pencil eraser, after anesthetizing the local area. One or two biopsy specimens are taken, and ideally are examined after sectioning the skin samples both horizontally and vertically. Scalp biopsy from the centre of the lesion provides confirmation of permanent hair loss, whereas a biopsy from the edge or an area of active inflammation may shed light on the underlying pathology. Paired 4-mm punch biopsies for horizontal and vertical sectioning are normally recommended by dermatologists.
• Hair Pull Test: A hair pull test, done by gripping about 20 hairs and gently pulling upward and away from the skin allows for the assessment of clinical activity on the scalp. It is normal for about 3 hairs to be extracted with each pull. If more than 10 hairs are removed, the test is considered positive and the hairs should be examined under a microscope to determine hair follicle damage. If crusting, pustules, bogginess or scales are present, hairs should be extracted from the edge of the bald area for microscopy, culture and sensitivity testing (bacterial and fungal), and any pustule should be swabbed and the fluid cultured.
• Microscopy, Culture, and Sensitivity Testing: If crusting, pustules, bogginess or scales are present, hairs should be extracted from the edge of the bald area for microscopy, culture and sensitivity testing (bacterial and fungal), and any pustule should be swabbed and the fluid cultured.
• Autoantibody Testing: Autoantibodies should be obtained if chronic cutaneous lupus erythematosus is suspected.

Treatment Options

The goal of treatment is to stop further hair loss and to camouflage the residual bald areas with cosmetics. Early effective treatment is key to the management of these disorders, with the goal of reducing symptoms and slowing disease progression. Unfortunately, treatment response is variable and often incomplete. The variable natural history of these disorders makes it difficult to determine the required duration of treatment. The first order of business in treating scarring alopecia is to treat the primary inflammatory disease medically. Once the underlying cause of the hair loss patches has been addressed, you may begin to treat and remedy the affected areas.

Medical Treatments

Current treatment options to stop hair loss in primary cicatricial alopecia are derived from small case series and consensus guidelines. Treatments have evolved empirically based on the nature of the inflammation. Physicians need to take into account the nature and extent of the disease, rate of progression, potential for adverse effects and patient flexibility (i.e., cost, compliance) in choice of treatment.

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• Corticosteroids: Topical corticosteroid lotions will control itching and burning. Potent topical corticosteroid treatment for 12 weeks may halt disease progression but tends to produce substantial atrophy. Intralesional injection of corticosteroids (e.g., triamcinolone 10 mg/mL) into foci of active inflammation will stop hair loss at the site of injection. Oral corticosteroids (e.g., 25-40 mg/d of prednisone for 2-4 mo) are reserved for rapidly progressing and severely symptomatic disease.
• Antimalarials: Antimalarials such as hydroxychloroquine are usually used as first-line systemic therapy, with onset of action between 3 and 6 months.
• Oral Retinoids: Oral retinoids are effective in some people but are poorly tolerated because of their tendency to induce hair shedding. The response to oral retinoids is often rapid, but the risk of teratogenicity should be considered. Telogen effluvium, drug-induced hair loss that causes the hair follicle to enter the resting phase (telogen) and fall out prematurely, may occur within 6 months of treatment with oral retinoids.
• Antibiotics and Antiseptics: Folliculitis decalvans is most often treated with antibiotics and antiseptics. All patients with folliculitis decalvans should use an antiseptic shampoo. Topical benzoyl peroxide or topical clindamycin will control mild cases. For severe or refractory cases, oral antibiotics will be required. The ability of an antibiotic to achieve adequate bactericidal concentration within hair follicles is an important consideration. The most common bacteria identified is Staphylococcus aureus. Minocycline is commonly used and will control many cases. The combination of rifampicin (300 mg twice daily), clindamycin (300 mg twice daily) and fusidic acid together with topical corticosteroid lotion for 3 months has been used with success.
• Isotretinoin: In contrast to folliculitis decalvans, dissecting cellulitis responds well to oral isotretinoin (0.5-1 mg/kg daily).

Surgical Options

In general, surgery is useful only for patients with lesions that are stable in size and for whom the inflammation driving the hair loss has been burned out for at least 1 year. Typically, your scarring alopecias can be corrected by adding a large number of follicular groupings into the affected scar tissue. The growth of transplanted hair in scarring alopecias is more unpredictable than for typical male or female pattern hair loss and often requires one to three procedures to achieve your cosmetic goal.

• Hair Transplantation: For patients with visible hair loss not reversible with medical therapy, hair transplantation is an excellent reconstructive procedure.
• Scalp Reduction: Scalp reduction may be considered in these instances.

Other Therapies

• Platelet-Rich Plasma (PRP) Therapy:
• Low-Level Light Laser Therapy:
• Minoxidil: Minoxidil solution or foam (2% or 5%) applied twice daily to the scalp may be helpful to stimulate any small, remaining, unscarred follicles.

Living with Scarring Alopecia

Living with scarring alopecia can be challenging, both physically and emotionally. Visible hair loss due to scarring alopecia can also have emotional and psychological effects.

• Hair Care Products: Hair care products and shampoos are generally safe as long as they are non-irritating to the scalp. A dermatologist can recommend specific shampoos and products to decrease scalp symptoms, scaling and inflammation and will recommend frequency of their use.
• Protective Measures: Protection of the scalp with a hat, and face and body with broad-spectrum sun-screen should be used on a daily basis.
• Cosmetic Camouflage: Hair pieces, wigs, hats and scarves are all safe, will not aggravate the condition, and may be used freely.

Prevention

There’s currently no known preventive measures for primary scarring alopecia. In the case of secondary subtypes, it may be possible to prevent some underlying causes, such as burns. Infections, cancer treatments, and other external factors may be more difficult to avoid.

tags: #scarring #alopecia #causes

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