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

cicatricial_alopecia.jpgCicatricial (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 NAHRS 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 NAHRS 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 NAHRS, 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 NAHRS classification system for cicatricial alopecia was designed to help group patients who might respond to promising new treatments. The NAHRS 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: Minimize
  • 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: Minimize
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 NAHRS. No changes in treatment should be undertaken by a patient without discussion first with the patient's physician.
  

Cicatricial Alopecia Path Project Minimize

CPATHOLOGY EVALUATION FOR CICATRICIAL ALOPECIA
(Version 2)

Purpose:
This form was created during the North American Hair Research Society (NAHRS) – 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 NAHRS – 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

  

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