The term “cicatricial alopecia” refers to a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. The clinical course is highly variable and unpredictable. Hair loss may be slowly progressive over many years, without symptoms, and unnoticed for long periods. Or the hair loss may be rapidly destructive within months and associated with severe itching, pain and burning. The inflammation that destroys the follicle is below the skin surface and there is usually no “scar” seen on the scalp. Affected areas of the scalp may show little signs of inflammation, or have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Cicatricial alopecia occurs in otherwise healthy men and women of all ages, is not contagious, not hereditary and is seen worldwide.

Yes, cicatricial alopecia is classified as primary or secondary. This discussion is confined to primary cicatricial alopecia in which the hair follicle is the target of the destructive inflammatory process. In secondary cicatricial alopecia, destruction of the hair follicle is incidental to a non-follicle-directed process or external injury, such as severe infections, burns, radiation, or tumors.

Primary cicatricial alopecia is currently classified by the type of inflammatory cells seen on scalp biopsy. The inflammatory cells may be primarily lymphocytes, neutrophils, or sometimes the inflammation has mixed cells. Cicatricial alopecias that involve predominantly lymphocytic inflammation include lichen planopilaris, frontal fibrosing alopecia, central centrifugal cicatricial alopecia, pseudopelade (Brocq), chronic cutaneous lupus erythematosus, and keratosis follicularis spinulosa decalvans. Cicatricial alopecia that involve predominantly neutrophilic inflammation include folliculitis decalvans and tufted folliculitis. Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process, or vice versa. Cicatricial alopecia with a mixed inflammatory infiltrate include dissecting cellulitis and folliculitis keloidalis, both of which are secondary to follicular rupture.

The cause of various cicatricial alopecia is poorly understood. However, cicatricial alopecia involves inflammation directed at the hair follicle, usually the upper part of the follicle where the stem cells and sebaceous glands (oil gland) are located. If the stem cells and the sebaceous glands are destroyed, there is no possibility for regeneration of the hair follicle and permanent hair loss results.

Research suggests that there is a loss of function of a “master regulator” called the peroxisome proliferator-activated receptor gamma, or PPAR gamma. PPAR gamma plays an important role in the preservation of hair follicle cells, including stem cells, and sebaceous glands. Decreased PPAR gamma leads to sebaceous gland dysfunction, which causes abnormal processing and buildup of “toxic” lipids. This abnormal buildup of lipids triggers inflammation that ultimately destroys the hair follicle.

Who is affected by cicatricial alopecia?
Cicatricial alopecia affects both men and women of all ages and are rare in children. Some disorders may manifest in the teenage years. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecia. In general, they are not common.

There have been a few reports of cicatricial alopecia occurring in a family. However, many patients with cicatricial alopecia have no family history of a similar condition.

Central centrifugal cicatricial alopecia affects women of African ancestry most commonly and may occur in several family members. Frontal fibrosing alopecia is seen most commonly in post-menopausal women but also occurs in young women and men. While it is possible to have more than one type of hair loss condition, non-scarring types of hair loss do not turn into scarring forms of hair loss.

Cicatricial alopecia affects both men and women of all ages and are rare in children. Some disorders may manifest in the teenage years. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecia. In general, they are not common.

There have been a few reports of cicatricial alopecia occurring in a family. However, many patients with cicatricial alopecia have no family history of a similar condition.

Central centrifugal cicatricial alopecia affects women of African ancestry most commonly and may occur in several family members. Frontal fibrosing alopecia is seen most commonly in post-menopausal women but also occurs in young women and men. While it is possible to have more than one type of hair loss condition, non-scarring types of hair loss do not turn into scarring forms of hair loss.

In general, cicatricial alopecia is not associated with other illnesses and usually occur in healthy men and women.

Patients with chronic cutaneous lupus erythematosus may have an increased personal and family history of autoimmune disorders.

A scalp biopsy is essential for the diagnosis of cicatricial alopecia and is the necessary first step. Findings of the scalp 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, to determine the degree of activity, and to select appropriate therapy.

Clinical evaluation of the scalp is also important. Symptoms of itching, burning, pain or tenderness usually signal ongoing activity. Signs of scalp inflammation include redness, scaling, and pustules. However, in some cases there are few symptoms or signs and only the scalp biopsy demonstrates the active inflammation. The overall extent and pattern of hair loss is noted and sometimes photographed for future comparison. A hair “pull test” may be performed to identify areas of active disease in which hairs are easily pulled out. In addition, if pustules are present, cultures may be performed to identify which microbes, if any, may be contributing to the inflammation. A thorough evaluation that includes all of these parameters is important in diagnosing a cicatricial alopecia and in identifying features in individual patients that will help the selection of therapy.

Diagnosis and treatment of cicatricial alopecia is often challenging. For this reason, it is helpful to be evaluated by a dermatologist with a special interest or expertise in scalp and hair disorders, and who is familiar with current diagnostic methods and therapies.  Contact the CARF office for a list of physicians or investigate hair clinics at teaching hospitals in your area.

As mentioned above, primary cicatricial alopecia is classified by the predominant type of inflammatory cells that attack the hair follicles: i.e., lymphocytes, neutrophils, or mixed inflammatory cells. Treatment strategies are different for each of these three subtypes and detailed treatment options are beyond the scope of this discussion. However, certain general principles are reviewed below.

Treatment of the lymphocytic group of cicatricial alopecia (including lichen planopilaris, frontal fibrosing alopecia, central centrifugal cicatricial alopecia, pseudopelade (Brocq), chronic cutaneous lupus erythematosus, and keratosis follicularis spinulosa decalvans) involves use of anti-inflammatory medications. The goal of treatment is to decrease or eliminate the lymphocytic cells that are attacking and destroying the hair follicle. Oral medications may include hydroxychloroquine, doxycycline, mycophenolate mofetil, pioglitazone, cyclosporine, or corticosteroids. Topical medications may include corticosteroids, topical tacrolimus, Derma-Smoothe/FS scalp oil; triamcinolone acetonide (a corticosteroid) may be injected into inflamed, symptomatic areas of the scalp.

Treatment of the mixed group of cicatricial alopecia (dissecting cellulitis and folliculitis keloidalis) may include antimicrobials although culture often does not grow a pathogen. Isotretinoin, anti-inflammatory medications such as corticosteroids, and tumor necrosis factor inhibitors may be used. In dissecting cellulitis, incision and drainage of nodules may be needed. You should discuss any treatment with your dermatologist who will also explain potential side effects, as well as laboratory tests that are needed before starting medications and sometimes are monitored during treatment.

The course of cicatricial alopecia is usually prolonged and treatments are often given for 6 to 12 months or longer. Treatment is continued until the symptoms and signs of scalp inflammation are controlled. In other words, itching, burning, pain and tenderness have cleared and scalp redness, scaling, and/or pustules are no longer present.

Treatment may then be stopped. However, except for pioglitazone, current treatments do not usually influence the underlying process and may not arrest progression of hair loss even when symptoms and signs have cleared. Clinical activity often recurs, and treatment frequently needs to be repeated.

Because of the above last statement, surgical treatment is not an option in most cases except under certain circumstances. If the disease has been inactive for one or two years, then surgical removal of the scarred scalp and/or hair restoration surgery may be considered for cosmetic benefit. Folliculitis keloidalis is one exception in that excision of the affected scalp (scalp reduction) may provide relief for the patient.

Hair will not regrow once the follicle is destroyed. However, it may be possible to treat the inflammation in and around the surrounding follicles before they are destroyed and for this reason it is important to begin treatment as early as possible to halt the inflammatory process. Minoxidil solution (2% or 5%) applied once (5%) or twice (2%) daily to the scalp may help to stimulate any small, remaining, unscarred follicles. The progression of hair loss is unpredictable. In some cases, progression is slow and there is sufficient hair remaining to cover the affected scalp areas; in other cases, progression can be rapid and extensive.

Hair care products and shampoos can generally be used with any frequency desired, as long as the products are not irritating to the scalp. Dermatologists may recommend specific shampoos and products to decrease scalp symptoms, scaling, and inflammation. Hair pieces, wigs, hats, scarves may be used freely.

RELATED INFO

  Join Us          Donate        Volunteer

© 2019 Cicatricial Alopecia Research Foundation

Contact                Privacy Policy