Cicatricial Alopecia - Dirk M. Elston, MD

Cicatricial (scarring) alopecia 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. Sometimesa 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. 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, special stains for bacteria, fungi and elastic tissue. In some infectious disorders, a biopsy must be sent for tissue culture.

At the 2001 Workshop on Cicatricial Alopecia held at Duke University Medical Center, a useful classification system was developed, emphasizing the microscopic findings in early disease. This classification divides cicatricial alopecia into hair loss caused by inflammatory cells called lymphocytes versus hair loss caused by a different type of inflammatory cells called neutrophils The new classification will help group patients for studies of new treatments. Even with the new classification, some cases of hair loss remain unclassifiable.


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 skin disease, and do not have systemic lupus erythematosus (SLE). It is important to determine which patients do have SLE, because they may need special treatment for internal organ involvement, especially kidney disease. Treatments you should discuss with your doctor include corticosteroids, 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. LPP 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.

Central Centrifugal cicatricial alopecia (CCCA)
This disorders 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 most commonly in African American women, and has also been referred to as follicular degeneration syndrome and hot comb alopecia. When seen in Caucasian women, it is called central elliptical pseudopelade. Some authors group all these condtions under the broader heading of "idiopathic pseudopelade". It is unclear, but suspected, that this condition may be related to chemical processing, heat, or chronic tension on the hair.


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 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 antibiotics, particularly Rifampin and Clindamycin combination therapy, topical corticosteroids, fusidic acid, and zinc sulfate: there are side effects of each of these which must be discussed with your 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 establishing the correct diagnosis and suggesting the most appropriate treatment for your hair loss. Many patients do not respond to the first treatment they receive and often the condition relapses when treatment is stopped. The new classification system for cicatricial alopecia was designed to help group patients who might respond to promising new treatments. Your dermatologist can help guide you through the array of off-label and experimental treatments that are available.


References:

• Elston DM, Bergfeld WF: Cicatricial Alopecia (and other causes of permanent alopecia). In Olsen EA (ed).: Disorders of Hair Growth: Diagnosis and Treatment, McGraw-Hill, New York, pp 285-313, 1994.
  
• Whiting DA: Cicatricial Alopecia: Clinico-pathological findings and treatment. Clinics in Dermatol 19:211-225, 2001.
  
• Sperling LC, Solomon AR: A new look at scarring alopecia. Arch Dermatol 136:235-242, 2000
  

Frequently asked questions:

• Why is this called scarring or cicatricial alopecia? Does a scar develop in the areas involved?
• 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?
• I have been told the areas of hair loss in my scalp are secondary to lupus. Does this mean I have internal lupus? Do I need to take medicines by mouth for this?
• 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?
• 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?

1. 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 a hairy area is replaced by hairless skin that is "slick", without the usual visible pores, and may be depressed. It is "like a scar" but does not necessarily have the definition between normal and scarred skin as do traumatic scars. 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.
   
   
2. 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. Topical corticosteroids and oral antibiotics are sometimes used to treat this disorder. 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, and neither chemical treatments nor tension on the hairs has been proved to be the cause. Once lost, the hair rarely regrows.
   
   
3. I have been told the areas of hair loss in my scalp are secondary to lupus. Does this mean I have internal 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
   
   
4. 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, including vitamin A derivatives called retinoids.
   
   
5. 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?
   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 doxycycline and topical steroids. Long term oral antibiotics, oral corticosteroids (cortisone-type drugs), retinoids, anti-malaria pills and thalidomide may be useful in some cases. 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.