Information About Cicatricial Alopecia or Scarring Hair Loss

Frontal Fibrosing Alopecia

Folliculitis Decalvans

Lichen Planopilaris

Discoid Lupus Erythematosus

Cicatricial Alopecia - Dirk M. Elston, MD & Elise Olsen, MD

See FAQs at the bottom of the page.

Cicatricial (scarring) alopecia (hair loss) is the term used for a group of disorders that cause permanent hair loss. During the active, evolving stage of hair loss, patches of alopecia commonly appear red and inflamed at the base of the hair shaft. Sometimes crops of pustules are noted. Some types of cicatricial alopecia destroy the hairs deep within the scalp, without inflammation visible on the skin surface. While some types of cicatricial alopecia result in rapid hair loss, slow progression of hair loss is more common.

A skin biopsy is generally required to establish the diagnosis, and to guide treatment. The biopsy punch is an instrument that removes a plug of skin about the size and shape of a pencil eraser. The biopsy is performed after an injection of local anesthetic to numb the skin. After the skin biopsy is removed, the biopsy site is closed with stitches or filled with a plug of special material that stops the bleeding. Sometimes a single biopsy specimen can establish the diagnosis, but usually more than one specimen is required. Your doctor will try to limit the number of biopsy specimens, but it is generally best to have more than one performed early on in your evaluation, so that an accurate diagnosis can be established and appropriate treatment started.

Some types of hair loss are best diagnosed under the microscope based on slices of the specimen cut vertically from the skin surface down to the deep fat (vertical sections). Other types of hair loss are best diagnosed by horizontal sections cut sideways through the specimen (horizontal sections). Each of these types of examination requires a separate biopsy specimen. Biopsies are best done of active, inflamed sites on the scalp which still have remaining hair. A biopsy of an older scarred area may be helpful to predict the likelihood of regrowth of hair, and to help establish the diagnosis by evaluating the pattern of scar formation. If certain types of cicatricial alopecia are suspected, your doctor may send a biopsy specimen for additional special tests including direct immunofluorescence, and special stains for bacteria, fungi and elastic tissue. In some infectious disorders that can cause cicatricial alopecia, a biopsy must be sent for tissue culture.

At the 2001 AHRS Workshop on Cicatricial Alopecia held at Duke University Medical Center, a useful classification system for cicatricial alopecia was developed, emphasizing the microscopic (histological) findings on a representative biopsy of an area of active loss. This classification divides cicatricial alopecia into hair loss caused by inflammatory cells called lymphocytes versus hair loss caused by inflammatory cells called neutrophils. The new classification will help identify patients who may be appropriate candidates for studies of new treatments since it is likely that medications will not work on both lymphocytic and neutrophilic predominant cases of cicatricial alopecia. Even with the new histological classification, some cases of hair loss remain unclassifiable.

Treatment for cicatricial alopecia remains poor. Once the hair is destroyed, the hair loss becomes permanent. It is primarily the hair at the periphery of the hair loss and/or the islands of remaining hair that are at risk of being destroyed that is the main focus of treatment. The main goals of treatment for cicatricial alopecia are to prevent further hair loss and to eradicate or at least lessen the redness, scale and itching associated with the process. There are no current FDA approved treatments for cicatricial alopecia although the AHRS has been working to initiate interest in this area among industry sponsors. All treatment now for cicatricial alopecia is strictly based on the experience of the prescribing physician or anecdotal reports as there has never been any multicenter clinical trial in this area.

MAJOR TYPES OF CICATRICIAL ALOPECIA CAUSED BY LYMPHOCYTES:

Chronic cutaneous lupus erythematosus (CCLE)

Chronic lupus erythematosus occurs more frequently in females than males and more commonly in adults than children. Most patients with CCLE only have evidence of lupus in their skin, and do not have systemic lupus erythematosus (SLE). Blood tests may be necessary to rule out SLE. It is important to determine which patients with cicatricial alopecia secondary to lupus do have SLE, because they may need special treatment for internal organ involvement, especially kidney disease.

Treatments utilized for cicatricial alopecia caused by CCLE include corticosteroids (topical, intralesional or internal), antimalarial pills such as Plaquenil, vitamin A derivatives, Dapsone and even Thalidomide. Each drug has its own potential side effects for which you would need to be monitored. Surgery can also be helpful to remove areas of scar but surgical removal of bald areas should be approached cautiously, as it can sometimes result in a flare of the skin disease in surrounding skin.

Lichen planopilaris

Lichen planopilaris (LPP) is a chronic inflammatory skin disease of the scalp that causes cicatricial alopecia. It is the most common type of cicatricial alopecia seen in the Caucasian population and is more common in women. Some patients have overlapping features of CCLE and LPP.

LPP is treated with many of the same drugs as chronic cutaneous lupus erythematosus although there have been recent trials using other immunosuppressive medications as well.

Central Centrifugal Cicatricial alopecia (CCCA)

This disorder is slowly progressive, usually begins in the crown and advances to the surrounding areas. It may be confused with female pattern hair loss, especially in its early presentation. This condition is seen almost exclusively in African Americans, most commonly in African American women. It has also been referred to as “follicular degeneration syndrome” and “hot comb alopecia”. It is unclear, but suspected, that this condition may be related to chemical processing, heat, occlusive ointments or greases or chronic tension on the hair.

The AHRS, in conjunction with Procter and Gamble, is currently spearheading a national project to help discover the incidence and the causes of this often very extensive hair loss. Treatment suggestions usually include (1) stopping relaxing or chemical processing of any kind and any heat based straightening, (2) culturing for and treating any bacterial or viral infection that may be present and (3) keeping the scalp hair in a natural, twists or light braids. Some doctors prescribe antibiotics (for their anti-inflammatory effect as well as antibacterial properties), topical steroids and/or topical minoxidil. 

MAJOR TYPES OF CICATRICIAL ALOPECIA CAUSED BY NEUTROPHILS:

Folliculitis Decalvans

Follculitis decalvans presents as crops of pustules that affect the hair follicle and result in permanent hair loss. The surrounding scalp can be soft and boggy or firm. Bacteria, especially Staphylococcus aureus, are often noted with special stains of biopsies and/or cultures of pustules. Unlike ordinary Staph infections, short courses of antibiotic therapy will not cure the condition. Treatments that have been reported as potentially useful include prolonged use of oral antibiotics, particularly Rifampin and Clindamycin combination therapy, topical corticosteroids, fusidic acid, and zinc sulfate: there are side effects of each of these which the patient should discuss with his/her physician before use.

Dissecting Cellulitis

Dissecting cellulitis of the scalp looks like deep cystic acne involving the scalp. It occurs primarily in African American men. Antibiotics, retinoids and corticosteroids may be helpful. Effective treatment often requires combination therapy, with drainage and injection of individual cysts.

SUMMARY:

There are many other less common types of cicatricial alopecia. A careful physical examination, scalp biopsies and blood tests can be helpful in order to establish the correct diagnosis and to suggest the most appropriate treatment for the hair loss. Many patients do not respond to the first treatment they receive and often the condition relapses when treatment is stopped. The new AHRS classification system for cicatricial alopecia was designed to help group patients who might respond to promising new treatments. The AHRS multicenter study of CCCA in African American women may hopefully lead to an explanation and effective treatment for this condition. If you have cicatricial alopecia, your dermatologist can help guide you through the array of off-label and experimental treatments that are available.

References:

  • Bergfeld WF and Elston DM. Cicatricial Alopecia in Olsen EA (editor). Disorders of Hair Growth: Diagnosis and Treatment. McGraw-Hill, New York, 2003.
  • Whiting DA: Cicatricial Alopecia: Clinico-pathological findings and treatment. Clinics in Dermatol 19:211-225, 2001.
  • Olsen EA. Female pattern hair loss and its relationship to permanent/cicatricial alopecia: a new perspective. J Investig Dermatol Symp Proc 10: 217-221, 2005

Cicatricial Alopecia Frequently Asked Questions:

The following are frequently asked questions on cicatricial alopecia. The information provided is not meant to be a substitute for the information obtained at an evaluation and by discussion with a physician, but merely to encourage understanding of this condition. No questions regarding individual scenarios will be answered by the AHRS. No changes in treatment should be undertaken by a patient without discussion first with the patient's physician.

Why is this called scarring or cicatricial alopecia? Does a scar develop in the areas involved?

In all forms of cicatricial alopecia, fibrous tissue replaces the hair follicles. In most conditions, the inflammatory cells destroy all the appendages (hair, oil and sweat glands) in an area of the scalp and an area with hair is replaced by hairless skin that appears "slick", without the usual visible pores, and may be slightly depressed on palpation. It is "like a scar" but does not necessarily have the appearance of a scar that occurs after trauma ie it may not look very different from normal skin to most people. It is not the result of a break in the skin being closed by scar tissue. In cicatricial aloplecia, the scar is mostly underneath the surface where there is gradual thickening of the fibrous tissue.

I am a 32-year-old African American female who has been losing my hair in the middle of my scalp for approximately 2 years. My dermatologist has told me the hair won't grow back, that I should stop using relaxers but didn't offer any specific treatment. Surely this hair loss has a cause and a therapy that will help?

The hair loss you describe fits the description of central centrifugal cicatricial alopecia (CCCA). As a group, patients with this disorder have similar clinical and biopsy findings, however the cause of the disorder remains unknown. No treatment has been proved to be effective although topical corticosteroids and oral antibiotics are sometimes sometimes help stabilize the condition and prevent it from getting worse. Because it has been suggested that the disorder may be related to hair grooming practices, many physicians recommend avoiding chemical treatments and excessive hair tension. Research into this condition continues, including work currently being done by the NAHRS. Once lost, the hair rarely regrows so the emphasis must be on preventing further loss.

I have been told the areas of hair loss in my scalp are secondary to lupus. Does this mean I have systemic lupus? Do I need to take medicines by mouth for this?

Most patients with chronic cutaneous lupus erythematosus of the scalp do not have systemic lupus erythematosus, and will never develop internal problems related to lupus. A physical examination together with blood and urine tests can be used to determine who is likely to have internal problems with their lupus. Depending on the severity of the skin lupus, you may require courses of oral treatment in addition to topical treatments. Cicatricial alopecia caused by lupus may actually show some hair regrowth with treatment which is different from other types of cicatricial alopecia where prevention of further loss and not regrowth is the hoped for result of effective therapy.

I am a 30-year-old African American male with a 3-year history of swelling and hair loss primarily in the back of my scalp. I have been told this is "cellulitis" but it doesn't seem to clear with antibiotics. What do I have?

The findings you describe suggest a diagnosis of Dissecting Cellulitis of the scalp. Although bacteria play a role in this disorder, it is not simply an infection of the skin. The disorder resembles severe cystic acne of the scalp. Long term use of antibiotics, together with drainage and injection of individual lesions may be effective. Many patients need a combination of treatments. Vitamin A derivatives, also called retinoids, have shown some success with this problem.

I am a 55-year-old white female with a two-year history of a sore scalp and hair loss in spots over my scalp. There are small bumps around the remaining hair. My doctor thought this was "lichen planopilaris" but the hair loss has progressed despite doxycycline and topical steroids. Do you have any suggestions for further work-up or treatment?

First, it is important to establish the correct diagnosis. If you have not had scalp biopsies, or if they were inconclusive, additional biopsies are the best means of establishing the diagnosis. Tests such as direct immunofluorescence, tissue culture and special stains may be helpful in establishing the diagnosis. Some conditions that can mimic LPP include lupus erythematosus and folliculitis decalvans (FD). Biopsy will often distinguish these entities. If you experience periodic crops of pustules on the scalp, the diagnosis is more likely to be FD. FD requires long-term treatment with antibiotics. The antibiotics used to treat FD are often different from those used to treat LPP.

Having said this, the hair loss in the pattern you describe is often caused by lichen planopilaris (LPP). LPP can be difficult to treat, and may not respond to antibiotics and topical steroids. Other treatments such as oral or intralesional corticosteroids (cortisone-type drugs), retinoids, antimalaria pills such as plaquenil or thalidomide may be useful in some cases.

Cicatricial Alopecia Path Project

CPATHOLOGY EVALUATION FOR CICATRICIAL ALOPECIA (Version 2)

Purpose: This form was created during the North American Hair Research Society (AHRS) – sponsored workshop on cicatricial alopecia (Feb 10 and 11, 2001). The intent was to create a standardized template for recording pathologic findings in patients with cicatricial alopecia. The original form (version 1) has already been published (J Amer Acad Dermatology 2003; 48: 103 – 110).

An electronic version of the form (version 2) was created as part of a AHRS – sponsored study of the utility of the form in actual use by dermatopathologists experienced in the pathologic evaluation of alopecia. This version is now available to physicians who wish to utilize or test the form while recording findings from their own pathologic specimens.

DOWNLOAD ELECTRONIC VERSION HERE

(The electronic version is MS Excel format and contains macros. Security settings within Excel will need to be set to medium or lower to open the file.) PDF VERSION HERE (text only)

Questions or comments regarding the form can be directed to: lsperling@usuhs.mil