Central Centrifugal Cicatricial Alopecia




Central centrifugal cicatricial alopecia is an inflammatory type of central scalp hair loss seen primarily in women of African descent. The prevalence is unknown, but may vary from 2.7% to 5.7% and increases with age. This review outlines the history and current beliefs and identifies clues for future research for this enigmatic condition. Despite that the cause of central centrifugal cicatricial alopecia is unknown, research is ongoing. The role of cytokeratins, androgens, genetics, and various possible sources of chronic inflammation in disease pathogenesis remain to be elucidated.


Key points







  • •

    Central centrifugal cicatricial alopecia is a common condition that mostly affects women of African descent and may occur in families.


  • •

    The cause of central centrifugal cicatricial alopecia is unclear and current evidence does not support a strong causal association with traction and/or the use of relaxers.


  • •

    Because genetic predisposition may be unmasked by grooming, patients are advised to avoid or limit the use of relaxers, heat, and occlusive moisturizers.


  • •

    Studies are needed to provide evidence-based therapeutic regimens for this condition.






Introduction


Hair loss is a frequent complaint in women of African descent. In 1983, Halder and colleagues described alopecia as the fifth most common dermatosis in African Americans, with chemical and traction alopecias being cited as the most common types. Since that time, central centrifugal cicatricial alopecia (CCCA), a lymphocyte mediated type of central scalp hair loss seen primarily in women of African descent, has been described with increasing frequency. Despite the prevalence of this condition, there has been a paucity of data on incidence, cause, and evidence-based therapeutic regimens. However, recent studies and literature are elucidating possible clues for future research for this seemingly enigmatic condition. Before discussing in depth the specific condition of CCCA, it is worthwhile to review the specifics of hair and hair care practices unique to women of African descent.




Introduction


Hair loss is a frequent complaint in women of African descent. In 1983, Halder and colleagues described alopecia as the fifth most common dermatosis in African Americans, with chemical and traction alopecias being cited as the most common types. Since that time, central centrifugal cicatricial alopecia (CCCA), a lymphocyte mediated type of central scalp hair loss seen primarily in women of African descent, has been described with increasing frequency. Despite the prevalence of this condition, there has been a paucity of data on incidence, cause, and evidence-based therapeutic regimens. However, recent studies and literature are elucidating possible clues for future research for this seemingly enigmatic condition. Before discussing in depth the specific condition of CCCA, it is worthwhile to review the specifics of hair and hair care practices unique to women of African descent.




Hair morphology


Although there are no biochemical differences between hair of African, European, and Asian descent, hair morphology does differ. Black hair appears elliptical in cross-section, with a curved follicle and is in contrast to the oval-appearing follicle of Caucasians and round follicle of Asians. African hair has a high degree of irregularity in the diameter along the hair shaft, with frequent twists and random reversals in direction. It is also curlier and exhibits more knots, with decreased ability of sebum to coat the hair, leading to less shiny and drier hair ; this may be one factor contributing to African hair having less tensile strength and breaking more easily than Caucasian hair. In addition, studies suggest that overall hair density in black people may be less than that in Caucasians. When comparing the biopsies of the scalp in 22 African Americans to 12 Caucasians without hair loss, Sperling documented a lower hair density, with an average of 36 follicles per 4-mm-diameter round punch found in the Caucasian specimens compared with 22 follicles in the African American specimens. African hair has also been found to have fewer elastic fibers anchoring the hair follicles to the dermis. These race-related differences in hair structure may play a role in the types of styling that black woman choose, and the subsequent development of CCCA.




Hair care practices


Black women can spend a great deal of time and money grooming their hair and may visit hair salons weekly or every 2 weeks for styling. Straightening tends to be the styling method of choice, as it believed to lead to increased manageability, although other methods are used to increase styling options and flexibility in appearance.


Methods of Straightening


Thermal straightening, also known as hot combing or pressing, was the first method used by African American women to straighten their hair. It was popularized by Madame CJ Walker in the 1900s after she developed a wide-tooth comb, making it feasible to comb African American hair. Thermal hair straightening is accomplished by temporarily rearranging hydrogen bonds within the hair shafts. The process involves coating the hair with a lubricating oil or grease and combing the hair in sections with a very hot metal comb heated to 300 to 500°F until the hair has reached the desired level of straightness. Although less popular today, many black women still use this thermal technique as an alternative method to chemical relaxing. In the large series of 529 African American women ages 18 to 85 reported by Olsen and colleagues under the North American Hair Research Society (NAHRS) banner, 58% had ever used hot combs with 87% of those having used this by age 15 years old.


Flat irons are an alternative method of using heat as a method of hair straightening. Women place their hair between 2 smooth, often ceramic plates that are thought to heat more evenly and quickly than hot combs. Temperatures may still range from 180 to 450°F, but the even heating and better temperature control is thought to decrease potential damage. In addition, use of greases and oils is generally discouraged with this method, with many women using silicone-based heat protectant products to reduce damage to hair.


Alternate methods of hair straightening including the use of chemical relaxers were developed in the 1960s. The 2 major chemical agents used are sodium hydroxide in lye relaxers, and guanidine hydroxide found in no-lye relaxers. These hair relaxers produce permanent hair straightening by rearranging the disulfide bonds, accounting for the sulfurous odor sometimes noted during use. Relaxer use, in particularly when used improperly, has been associated with several adverse reactions, including irritant contact dermatitis, trichorrhexis nodosa, and brittle, more easily damaged hair. This brittle nature of the hair is likely associated with the reduced cysteine content found in relaxer-treated hair, which is crucial for hair strength because it is a component of the disulfide bonds broken by the chemicals. Chemical straightening via relaxers is currently much more popular than hot combing, with 90% of African American women having used this method at some point.


Styling


Hairstyling with braids and weaves is popular in people of African descent. There are several methods for braiding hair, including braiding into sections to produce “cornrows,” or adding human or synthetic hair for additional length and volume. Weaving uses similar methods whereby the hair is cornrowed, and additional synthetic or human hair is sewn or glued to the base of the hair, and the additional hair is worn loose. “Locs” or dreadlocks are a type of styling that traditionally avoids chemical or heat processing and allows the hair to knot into individual twistlike structures. These structures can be maintained via twisting/“palm-rolling” the root of the loc to allow for a uniform or “manicured” look ( Fig. 1 ) or may be free-formed, such that the locs/dreadlocks are allowed to form with little to no intervention. This styling may lead to tension at the root of locs if they are overtwisted, if each section is too small, or if locs are allowed to grow to very long lengths. These methods of styling have been associated with traction alopecia or marginal scalp hair loss, but because this type of styling is mostly unique to people of the African diaspora, there has been some speculation regarding their relationship to CCCA.




Fig. 1


Manicured locs in an elaborate style.


Hair Cleansing


African American women tend to shampoo less frequently than those of European descent, the most common frequency being once every 1 to 2 weeks. Some hairstyling methods are costly and time-consuming, which may contribute to the relative infrequency of hair washing needed to maintain these styles. In a study by Hall and colleagues, 85% of women surveyed spent 0 to 5 hours at the salon at each visit, and 46% of the women spent greater than $50 monthly on hair care. In addition, most African American women studied wore their hair in a relaxed or straightened style, which reverts to a more native or curlier texture when wet. The longevity of styles with weaves, braids, and locs may also be shortened by frequent shampooing. Last, shampooing too frequently may increase hair breakage secondary to decreasing relatively low levels of sebum on the hair shaft. How this infrequent removal of surface microorganisms may be related to CCCA remains to be evaluated.




History of CCCA


This entity was first published in the literature by LoPresti and coworkers, in 1968, whereby they described an “irreversible alopecia of the scalp” in black women in their 20s and 30s, with a characteristic course of beginning on the crown and spreading peripherally, sparing the lateral and posterior aspects of the scalp. This hair loss differed from that seen in other scarring conditions such as morphea or discoid lupus erythematosus, in that the skin remained soft and pliable, with a “glistening, shiny surface,” although there was a “striking…decrease in the density of the follicular orifices.” They found that the 51 black women studied clinically with these findings all straightened their hair with hot combs, “in order to conform with cosmetic standards of the fashion setting white group,” although they describe one case with similar clinical findings in a Caucasian woman who had used a hot curling iron for years. It was postulated that the hot petrolatum/oil used while hot combing ran down the hair shafts of the central scalp, causing a chronic lymphocytic inflammation around the upper segment of the hair follicle, leading to external root sheath degeneration, follicle destruction, and finally, follicular scarring. This entity was coined “hot comb alopecia.”


Following this description, there was little mention of this entity in the literature for over 2 decades except for occasional mentions in review articles on alopecia. However, by 1987, Price recognized that hot comb usage was not essential for development of the condition and recommended abandoning the term “hot comb alopecia.” In 1992, Sperling and Sau “revisited and revised” this condition. In their study 10 black women who were seeking evaluation of their central scalp hair loss were found to have histologic findings of premature desquamation of the inner root sheath, lamellar fibroplasia, and mononuclear inflammation, consistent with this condition. They found that not all patients had a history of hot comb use and that this method of styling was not temporally related to the onset or progression of this condition. In addition, the use of hot combs had fallen in popularity by the early 1990s and had been somewhat replaced by the use of relaxers, yet cases of this condition persisted. Furthermore, patients in their control group of 10 women with no history of hair loss or hair problems practiced similar hair care methods (including use of hot combs and/or relaxers) and had not developed the entity. They proposed that “a predisposed population…exists that begins to express premature desquamation of the inner root sheath some time during adulthood.” Several factors, including mechanical factors specific to the follicle, heredity, and methods of hair grooming, contributed to this predisposition, and any combination of the above could lead to the expression of the syndrome. They also noted that this condition does not seem to begin in childhood, so that the defect must emerge later in life, or be expressed in relation to certain factors such as traumatic hair care techniques. Secondary to the seemingly specific histologic findings of premature inner root sheath degeneration and migration of the hair shaft through the outer root sheath, Sperling and Sau proposed the term “follicular degeneration syndrome (FDS).”


In a 1996 review, Headington argued that the follicular degeneration syndrome was not a distinct clinicopathologic entity but that the findings of selective premature degeneration of the follicular inner hair sheath may be seen in a variety of different scarring alopecias. He postulated that premature fragmentation of the inner sheath is probably a result of altered outer hair sheath biology by cell-mediated injury or trauma. He used the term “scarring alopecia in African Americans” and proposed that a combination of relaxer use in addition to traction may contribute to the development of this condition.


In 2000, Sperling and coworkes coined the term central centrifugal scarring alopecia, to encompass clinical patterns of hair loss that have the following findings:



  • 1.

    Hair loss centered on the crown or vertex of the scalp


  • 2.

    Chronic and progressive disease with eventual burnout


  • 3.

    Symmetric expansion with most active disease at the periphery


  • 4.

    Both clinical and histologic evidence of inflammation in the active peripheral zone.



By this definition, central centrifugal scarring alopecia included not only follicular degeneration syndrome, but other conditions, such as traumatic alopecia, folliculitis decalvans, lichen planopilaris, or discoid lupus erythematosus.


In 2001, the term “central centrifugal cicatricial alopecia (CCCA)” was adopted by the NAHRS to refer specifically to the central scarring hair loss seen predominately in African Americans. This term encompassed hot comb alopecia and follicular degeneration syndrome.




Clinical features


The clinical features of CCCA that have been described have remained constant over the decades. Patients present with a chronic and progressive central scalp hair loss that expands centrifugally in a somewhat symmetric fashion. Advanced cases show a smooth and shiny scalp with impressive follicular dropout. Occasionally, a few strands of hair remain in the affected bald area, some demonstrating polytrichia. There is typically no overt evidence of inflammation, although there may be erythema or follicular pustules early in the course. However, follicular erythema or papules as seen with lichen planopilaris are lacking ( Fig. 2 A–C ).


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Feb 12, 2018 | Posted by in Dermatology | Comments Off on Central Centrifugal Cicatricial Alopecia

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