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Central centrifugal cicatricial alopecia: Past, present, and future
REVIEWS
Central centrifugal cicatricial alopecia: Past, present, and future Raechele Cochran Gathers, MD, and Henry W. Lim, MD Detroit, Michigan See related article on page 574 Clinical scarring alopecia in African American women has been recognized for years. The classification of this unique form of alopecia dates back to Lopresti, who first described the entity called ‘‘hot comb alopecia.’’ More recently, the term ‘‘central centrifugal cicatricial alopecia’’ has been adopted to describe a progressive vertex-centered alopecia most common in women of African descent. While this form of hair loss is widely recognized, and may even be on the rise, the causes of central centrifugal cicatricial alopecia are a constant source of debate and remain to be elucidated. This review outlines the descriptive evolution of central centrifugal cicatricial alopecia and the historical controversies ascribed to its pathoetiology; it also examines African hair structure and discusses how hair structure along with common physical and chemical implements utilized by individuals with African hair type may play a causal role in the development of central centrifugal cicatricial alopecia. ( J Am Acad Dermatol 2009;60:660-8.)
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air loss among African American women is exceedingly common. Although ethnic hair care is a billion-dollar industry,1,2,3 with innumerable products and services purportedly designed to meet the unique needs of ethnic hair, a disproportionate number of African American women still suffer from hair loss. Halder et al,4 in 1983, described alopecias as the fifth most common dermatosis in African Americans, with chemical and traction alopecias cited as the predominant types. While there are no recently published epidemiologic data describing the number of African American women presenting with a primary diagnosis of alopecia, in the present authors’ opinion, the incidence in this group may be even higher than that reported by Halder. Among African Americans, central centrifugal cicatricial alopecia (CCCA) has been purported to be responsible for more cases of scarring alopecia than all other forms combined.5 However, it should be noted that true epidemiologic and prevalence data do not exist to date. Clinically, CCCA develops
From the Multicultural Dermatology Center, Department of Dermatology, Henry Ford Hospital. Funding sources: None. Conflicts of interest: None declared. Reprint requests: Raechele Cochran Gathers, MD, Multicultural Dermatology Center, Henry Ford Hospital, 3031 W Grand Blvd, Suite 800, Detroit, MI 48202. E-mail: [email protected]. 0190-9622/$36.00 ª 2008 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2008.09.066
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Abbreviations used: CCCA: CCSA: FDS: IRS: ORS:
central centrifugal cicatricial alopecia central centrifugal scarring alopecia follicular degeneration syndrome inner root sheath outer root sheath
as a scarring, roughly circular area on the crown and/or vertex of the scalp.3 With time, this scarred centralized area progresses centrifugally. The affected scalp is in parts smooth and shiny and illustrates massive follicular dropout. Typically, a few short, brittle hairs remain within the scarred expanse (Fig 1). Often, patients complain of dysesthesia (tenderness, itching) in the affected area.3,6 Anecdotally, dysesthesias may be associated with progression of the alopecia and active inflammation. Scarring, or cicatricial alopecia, is characterized by visible loss of follicular ostia and follicular destruction.7,8 In contrast to nonscarring (non-cicatricial) alopecia, which is reversible since follicular epithelium remains intact, cicatricial alopecia is, at its end stages, irreversible, and follicular epithelium is replaced by connective tissues.5 Cicatricial alopecia can be subdivided into both primary and secondary types, depending on the pattern of follicular destruction. In primary cicatricial alopecia, the hair follicle itself is the target of inflammation, with relative sparing of the interfollicular reticular dermis.8 Primary cicatricial alopecias can be further subdivided into 4 categories: lymphocytic (CCCA), neutrophilic, mixed, and non-specific.9 In contrast to
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primary cicatricial alopecia, in secondary cicatricial alopecia, the hair follicle is an ‘‘innocent bystander,’’5 destroyed nonspecifically and by consequence (such as by neoplasm, sarcoidosis, or pemphigus vulgaris).7 Sellheyer and Bergfeld10 and Ross, Tan, and Shapiro7 have already provided in-depth reviews of both the histopathologic evaluation of alopecias and the classification of primary cicatricial alopecias, respectively. This article focuses on present knowledge regarding CCCA, unanswered questions and controversies, and possible future directions for both clinical and bench research.
CCCA: AN EVOLVING NOMENCLATURE AND DESCRIPTION The nomenclature and classification schemes describing clinical cicatricial alopecia in African American women has been a source of some debate, and has varied fairly widely over the years (Fig 2). LoPresti, Papa, and Kligman11 in 1968, first described an ‘‘irreversible alopecia of the scalp’’ occurring in 51 African American women who straightened their hair using a hot comb. They described an ‘‘absolutely characteristic’’ course and configuration of alopecia beginning on the crown, which, given time, spread peripherally to form a large circle of alopecia, which spared the lateral and posterior aspects of the scalp. They described the scalp surface as shiny, with an obvious decrease in the density of follicular orifices. Clinical inflammatory changes, such as scaling, were not described and the scalp was described as ‘‘soft and pliable, not hard and immovable as in discoid lupus erythematosus or morphea.’’11 LoPresti, Papa, and Kligman described the alopecia as incomplete, with some seemingly normal hairs always remaining within the area of involvement. This unique and eventually irreversible form of alopecia was termed ‘‘hot comb alopecia,’’ and it was hypothesized that petrolatum, heated during thermal hair straightening with a hot comb, caused a chronic inflammation around the upper segment of the hair follicle, leading to external root sheath degeneration, follicle destruction and, finally, follicular scarring. There was little mention of hot comb alopecia in the medical literature for more than two decades subsequent to the article by LoPresti, Papa, and Kligman.11 This peculiar form of scarring hair loss in African American women was occasionally mentioned in review articles on alopecia,6,12,13 but was not the primary issue of focus or study. In 1992, Sperling and Sau6 proposed the new term follicular degeneration syndrome (FDS) for what had previously been called ‘‘hot comb alopecia.’’ Sperling and Sau retrospectively studied 10 black women with the characteristic hair loss pattern of hot comb alopecia.
Fig 1. CCCA in patient with history of hair relaxer use.
However, because they noted poor correlation between the usage of a hot comb and the onset or progression of this particular form of scarring alopecia, they hypothesized that use of a hot comb was indeed not the cause of hair loss in this group of women. In fact, they described women with clinical disease who had never used a hot comb in their lives. Sperling and Sau hypothesized that two particular histological features: (1) premature inner root sheath (IRS) degeneration and (2) migration of the hair shaft through the outer root sheath (ORS), were distinctive in their cohort of patients, and they proposed the new terminology of FDS to describe what was thought to be a clinically and histologically distinctive form of scarring alopecia seen in African American women.6 ‘‘Follicular degeneration syndrome’’ is a descriptive term, though, and does not implicate a particular cause. While hot combs were refuted by Sperling and Sau as a cause of FDS, they conceded that hot combs, or other styling methods, might contribute to the observed follicular damage. They suggested that in FDS, abnormal follicles might be predisposed to external injury, but remain intact as long as the scalp was not irritated. It was hypothesized that the use of hair relaxers, traction, or other chemicals might create irritation that was sufficient to act as a catalyst for FDS.6 In 1992, Nicholson et al14 described a pattern of scarring alopecia on the scalp vertex in 8 young females of Afro-Caribbean ancestry in the south London area of Great Britain. The histologic patterns described by Nicholson et al were consistent with those detailed by Sperling and Sau6 and similarly differed from that described by LoPresti, Papa, and Kligman11 in the lack of epidermal atrophy noted. Nicholson et al14 coined the term chemically induced cosmetic alopecia and hypothesized that the observed alopecia was related to the combination of chemical and physical insults to the hair and scalp. In a 1996 review of cicatricial alopecia, Headington15 refuted the hypothesis that the
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Fig 2. Nomenclature evolution of CCCA.
so-called FDS was a clinicopathologic entity. Headington maintained that selective premature degeneration of the follicular inner hair sheath could be seen in a variety of different scarring alopecias and was therefore neither specific nor diagnostic; it was probably a result of altered outer hair sheath biology by cell mediated injury or trauma. Headington, referring to the stereotypic hair loss seen in African American women as ‘‘scarring alopecia in African Americans,’’ concluded that in scarring hair loss of the type predominant in African American women, follicular stem cells had been repeatedly injured by chemical or physical trauma (ie, relaxers, traction) so that the entire follicular structure was ultimately lost. In a 2000 editorial, Sperling, Solomon, and Whiting16 described the term central centrifugal scarring alopecia (CCSA) to encompass a group of closely related clinical patterns of hair loss. Hair loss centered on the crown of the scalp, chronic and progressive disease with eventual ‘‘burnout’’, symmetrical expansion with the most active disease at the periphery, and clinical and histologic evidence of inflammation in the active peripheral zone were described as common features of CCSA.16 The term FDS was included under the subset of CCSA, as was pseudopelade (not to be confused with classic pseudopelade of Brocq) and folliculitis decalvans. While FDS was described to be a form of CCSA, it was noted that FDS demonstrated the distinctive histologic finding of premature desquamation of the IRS in both inflamed follicles and non-inflamed ‘‘normal’’ scalp.16 Most recently, in the 2001 Summary of the North American Hair Research Societyesponsored Workshop on Cicatricial Alopecia, published in 2003, the currently popular term CCCA was proposed.9 CCCA describes a form of lymphocytic
primary cicatricial alopecia characterized by hair loss starting in the central scalp and progressing centrifugally. CCCA was proposed as a more descriptive term that was thought to encompass what had previously been termed FDS, hot comb alopecia, pseudopelade in African Americans and central elliptical pseudopelade in Caucasians.
EPIDEMIOLOGY OF CCCA While there are sparse epidemiologic data on the racial and sex distribution of CCCA, the vast majority of reports describe women of African ancestry. LoPresti, Papa, and Kligman11 described 51 African American women in their description of hot comb alopecia, but also reported one Caucasian woman with an ‘‘identical clinical state.’’ In their 1992 description of FDS, Sperling and Sau6 described 10 black women. In their review of chemically induced cosmetic alopecia from Europe, Nicholson et al14 described female patients of Afro-Caribbean descent. A central scarring alopecia in Caucasians, central elliptical pseudopelade in Caucasians, is mentioned rarely in the literature,7,9 and descriptions of centrally patterned scarring alopecia in Caucasians may very well represent pseudopelade of Brocq, which may have similar histopathologic features to CCCA, but clinically is very different, typically presenting with numerous irregularly outlined alopecic patches, classically defined as ‘‘footprints in the snow.’’10 Pseudopelade of Brocq typically affects white adults, and the disease is often characterized by spurts of disease activity followed by dormant periods, unlike CCCA, which typically has a slow and steady progression.16,17 Like racial predilection, there are few data regarding sex distribution of CCCA. Sperling and Cowper5 have described a female/male ratio of 3:1 among black patients and, in a 1994 observation, reported 8
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men with purported FDS.18 Overall, this group of men had more evidence of active inflammation than did the previously described women with FDS, often illustrating follicular and perifollicular papules in a pattern less commonly described in women with a similar diagnosis. Whether the male patients reported by Sperling18 are true cases of what is now termed CCCA is debatable, and in the present authors’ opinion, the female/male ratio in CCCA is much higher than 3:1. While it is clear that women of African descent appear to be disproportionately affected by CCCA, true epidemiologic data with regard to race and sex distribution still need to be elucidated.
HISTOLOGY OF CCCA In their landmark 1968 description of ‘‘hot comb’’ alopecia, LoPresti, Papa, and Kligman11 described ORS degeneration in association with superficial perifollicular lymphocytic infiltrate and epidermal atrophy with elongated rete ridges and hypogranulosis, with ultimate replacement of the follicular unit, save the arrector pili muscle, with dense bands of eosinophilic collagen.7,11 Later, Sperling and Sau6 described two histologic features as being unique in what they had termed FDS: (1) premature degeneration of the IRS and (2) migration of the hair shaft through the ORS. Sperling and Sau also described a marked diminution in hair follicle density with some follicles being totally replaced by fibrosis, as noted by LoPresti, Papa, and Kligman. The epidermal atrophy and thinned and elongated rete ridges described by LoPresti, Papa, and Kligman were not prominent features noted by Sperling and Sau. Other histologic findings included a mononuclear cell infiltrate and lamellar fibroplasias at the level of the isthmus; disintegration of the follicular epithelium; disintegration of the ‘‘naked’’ hair shafts with surrounding foreign body giant cell reaction; and replacement of the entire follicle by thick fibrous tracts. However, these findings were noted by Sperling and Sau6 to be found in other forms of scarring alopecia as well, and were not described as unique to FDS. In contrast to Sperling and Sau,6 Headington15 in his discussion of vertex scarring in African Americans described the histologic findings of selective premature degeneration of the IRS as being nonspecific and present in a variety of different scarring alopecias, including chronic cutaneous lupus. However, Headington described other findings similar to those noted by Sperling and Sau: primary follicular scarring with decreased follicular density, loss of sebaceous epithelium, follicular fusion, premature fragmentation of the IRS, and slight perivascular lymphocytic inflammation. Late-stage lesions were characterized
by destruction of pilosebaceous units, dermal scarring, and dermal lymphocytic and plasma cell infiltrate. In the so-called ‘‘chemically induced cosmetic alopecia’’ described by Nicholson et al,14 histologic findings were largely similar to those previously described in hot comb alopecia, FDS, vertex scarring in African Americans and CCCA, with perifollicular chronic inflammatory cells, perifollicular fibrosis in a lamellar pattern, naked hair shafts within the dermis, and eventual total replacement of hair follicles by fibrosis. Whether CCCA is a distinct clinicopathologic entity or whether histopathologic changes are nonspecific and seen in other forms of scarring alopecia remains a controversy. However, common histopathologic themes do exist and are commonly accepted: a lymphocytic folliculitis, perifollicular granulomatous inflammation with hair shaft foreign body giant cells, prominent destruction of the folliculosebaceous units with scar tissue fibrosis at the sites of former hair follicles, and common retention of arrector pili muscles.7
AFRICAN HAIR STRUCTURE As it appears clear that CCCA disproportionately affects those of African descent, the unique structural properties of African hair must be considered. In general, hair in people of African descent is elliptical in cross section, with the physical shape resembling a twisted oval rod.19,20 By contrast, the hair of Asians demonstrates a round hair shaft with a very large diameter. Caucasian hair generally appears structurally between the two. While Asian hair has a circular geometry, African hair presents a high degree of irregularity in the diameter along the hair shaft, with frequent twists and random reversals in direction with pronounced flattening.19,21 The curly spiraled nature of African hair is believed to be caused by the curvature of the hair follicle. It is largely held that, because of its frequent twists and spiraling, African hair generally has less tensile strength and breaks more easily than Caucasian hair.19 Additionally, in its natural state, its curly configuration makes it less amenable to traditional combing methods. The tensile strength of a fiber is highly dependent on its section size. African hair differs from both Caucasian and Asian hair by its earlier breaking time under force and by its lower stress requirements at breaking.19 It is hypothesized that the lower tensile strength and relative brittleness of African hair may be explained by its morphology and geometry, with natural constrictions along the twisted hair shaft leading to microcracks and fractures in the hair fiber, exacerbated by everyday combing and grooming.
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Table I. Popular styling methods among African Americans (1) (2) (3) (4) (5) (6)
Braiding/cornrowing—Interlocking of 3 or more pieces of hair to create a loose or bound-down 3-dimensional hairstyle Twisting—Interlocking of 2 pieces of hair to create a loose or bound-down 3-dimensional hairstyle Thermal straightening (pressing)—Metal comb heated to approximately 3508F to achieve temporary straightening Chemical straightening—Application of alkaline chemicals to the hair, which causes permanent hair straightening Hair weaving—Addition of exogenous human or synthetic hair by sewing or bonding glue application Locking—Twists of hair allowed to remain, unmanipulated, such that the hair eventuates in permanently interlocking strands
While the proteins and amino acids constituting hair keratin are similar among races, there are other known differences between hair in individuals of Caucasian and African descent: African hair has fewer elastic fibers anchoring the hair follicles to the dermis22,23; melanosomes have been observed to be in both the ORS and the bulb of vellus hairs in African hair; African hair has more pigment and on microscopy has larger melanin granules in comparison with other hair types23; and African hair has less moisture content than Caucasian hair.19
AFRICAN AMERICAN HAIR CARE Just as its tight curl pattern may make African textured hair more likely to break and fracture, and may arguably make it more susceptible to disorders not largely seen in individuals with differing hair types, the culturally unique grooming patterns and practices among those with African textured hair may also make them uniquely susceptible to a myriad of dermatologic quandaries (Table I). Thermal hair straightening relaxes the tight coils of African textured hair by temporarily rearranging hydrogen bonds within the hair shafts.3 The application of heat, used to straighten the typically tightly coiled hair, has long been hypothesized to play a role in the etiology of hair and scalp disease among individuals with African textured hair. As previously discussed, LoPresti, Papa, and Kligman11 implicated thermal straightening by a hot comb as an etiologic factor for what is now called CCCA. While modernday hot combs and flat irons often feature protective ceramic surfaces and self-dialed heat controls (Fig 3), many women still straighten their hair with traditional metal combs, heated to 3008-5008 F on a stovetop or in an electric heating oven. Thermal burns are a common sequela to this method of hair straightening, as is hair breakage resulting from excessive heat causing drying and brittleness of the hair shaft. Whether CCCA is linked to thermal straightening and other heat sources (ie, blow dryer, heated curling irons) remains controversial. Chemical straightening is another hair care practice widely practiced by women with African textured hair. It has been estimated that 70% to 80% of
African American women use chemical applications to achieve a straighter hair style.24 Chemical straighteners have been in existence since at least the early 1900’s, when early chemicals such as lye, hog lard, and boiled eggs were used to achieve a straighter hair texture.20 In the mid 1900’s, chemical hair straighteners (termed relaxers since they relax the normally tight coils of African textured hair) became more widely used. These alkaline relaxers typically contain sodium, potassium, lithium, and guanine hydroxides, or, more rarely, sulfites and thioglycates, which work to permanently break the hydrogen/ disulfide bonds of curly hair, thus leaving it in a straighter formation.20 Unfortunately, chemical relaxers have been long documented to cause cuticular disruption, hair shaft damage, scalp irritation and chemical burns. In addition, chemical relaxers have also been implicated in the development of severe toxic irritant contact dermatitis and subsequent Staphylococcus aureus abscess formation.25 Most cases of damage from relaxers are typically caused by inappropriate application and usage.3 However, chemical hair relaxers have been hypothesized as being, if not a causative agent for scarring hair loss, at least an exacerbating factor. Headington15 has described prolonged cosmetic hair care practices in African Americans, such as chemical straightening with sodium, potassium, and lithium hydroxide, as being ‘‘suspect’’ in the pathogenesis of CCCA. In an African study of 39 female patients, Nnoruka26 found that women with scarring alopecia complained of local chemical burns and contact scalp irritation after too frequent and prolonged use of chemical relaxers. It was hypothesized that misuse of lye containing relaxers may have triggered a primary irritation, subsequent inflammation, and fibrosis, and ultimately a scarring alopecia. Chemically induced injury has also been suggested by Nicholson et al. as a causative factor in the development of scarring alopecia in their cohort of women of Afro-Caribbean descent. Nicholson et al14 hypothesized that the observed alopecia was related not to alkali relaxer misuse alone, but to a sum effect of physical and chemical insults that caused a natural progression from mild fibrosis and inflammation to
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end-stage scarring. More recently, Swee, Klontz, and Lambert27 published a report of a national outbreak of alopecia associated with a nontraditional acidic hair relaxer that included metal salts in its formulation. The FDA received (to date of this writing) the largest number of complaints the agency had ever received for a cosmetic product for this hair-straightening system, which caused hair breakage and loss in 95% of survey respondents, with some 9% still reporting no hair regrowth some 2 years after using the product. While chemical relaxers have not been definitively implicated as causative of CCCA, their misuse may be considered a pathogenic factor, perhaps one of many, in its evolution. At our center, we recommend that patients diagnosed with CCCA avoid chemical relaxers, as we believe that the associations proposed by Nnoruka26 and Nicholson et al14 are not unlikely. Clearly, however, without well-designed and well-conducted studies, this is still conjecture. Additional hair care practices that are largely unique to those with African textured hair include the widespread use of hair braiding or cornrowing, often with substantial amounts of added human or synthetic hair for additional length and volume. Braiding and extensions have been associated with excessive traction with subsequent folliculitis and alopecia, as well as with allergic contact dermatitis resulting from synthetic hair.3,28 Hair weaving typically involves the addition of color- and texture-matched synthetic or human hair extensions. Hair extensions may be added by sewing in the exogenous hair or by using a synthetic bonding glue.3 Hair weaving has been implicated in traction alopecia, folliculitis, and allergic reaction to the bonding glue.3,29,30 It is documented that African textured hair has fewer elastic fibers anchoring hair follicles to the dermis compared with that of Caucasians.22 Excessive weight from hair extensions, ponytails, or weaves may uniquely impact those with African textured hair. In a recently conducted study at our center, presented at the 2007 L’Oreal Institute for Ethnic Hair and Skin Research International Symposium, we found that certain hair grooming practices do appear to be more common in women in whom CCCA develops; these include the use of both sewn and glued hair weave as well as the use of cornrow or braided hairstyles with added extension hair. Furthermore, CCCA patients wearing these 3 implicated hairstyles were more likely to report a history of hair damage, reflected as a history of tender scalp or uncomfortable pulling. This seems to implicate, at least in part, either traction or perhaps an irritant dermatitis from synthetic hair and glue in the development of CCCA.
Fig 3. Thermal straightening implement: Hot comb.
A final aspect of grooming practices among persons with African textured hair which merits mention is the use of thick emollients as a hair lubricant, and styling gels and spritzes used to aid in hair styling. Historically, many African Americans have utilized thick hair lubricants (‘‘hair grease’’) in an effort to moisturize and style the hair, as well as to aid in ease of grooming. These emollients often contain mineral oils, petrolatum or lanolin and vegetable oils, which have been reported to worsen the inflammation of seborrheic dermatitis.31 Styling gels and spritzes containing a copolymer of polyvinyl pyrrolidone and vinyl acetates coat the hair. As they are often left on the hair for extended periods (weeks) between washings, they may exacerbate already dry and brittle chemically relaxed hair, and lanolin additives may exacerbate seborrhea.32 While our center’s recently presented study on grooming practices associated with CCCA found no clear correlation between the use of hair emollients or styling preparations with CCCA, it is still possible that this additional inflammatory insult may exacerbate the concurrent inflammation noted in the development of CCCA.
ETIOLOGY: A QUANDARY The etiology of CCCA remains controversial (Table II). Sperling, in his description of FDS, has hypothesized that the condition results from early degeneration of the IRS which allows the hair shaft to impinge upon the exposed soft ORS and subsequently precipitate a chain of histopathologic events which result in scarring.6,10 Ackerman et al10,33 have described what is now termed CCCA as a form of
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Table II. Factors implicated in the development of CCCA (1) (2) (3) (4)
Thermal straightening (hot comb, flat iron) Chemical straightening (relaxer) Traction/physical trauma (braiding, cornrows, weaves) Irritant dermatitis (glues, hair dressings)
traction alopecia, while Headington postulates that follicular stem cells are repeatedly injured by chemical and/or physical trauma so that the entire follicular structure is ultimately destroyed. In a recently completed study by the present author, we have found that both sewn and glued hair weave as well as the use of cornrow or braided hairstyles with added extension hair seem to be positively associated with CCCA. This finding appears to support, at least in part, the theories of both Ackerman et al33 and Headington.15 Nnoruka26 implicates duration of hair care practices, such as relaxers, in the development of CCCA. Other authors31,33 have reported a scarring alopecia that has been temporally or historically related to chemical relaxers. In an unpublished study, McMichael found that most African American patients without scarring had discontinued relaxing compared with those with scarring alopecia.20 Our recently presented study found no clear association between chemical relaxers and CCCA. However, because studies are limited to date, we still support the discontinuation of chemical relaxers in those with CCCA. Clearly, further population-based study is needed to delineate causative agents involved in the pathogenesis of CCCA.
TREATMENT OF CCCA To date, there are no published case-controlled prospective studies documenting effective treatment of CCCA. In fact, as CCCA is an ultimately scarring process, at its very late stages there is likely to be no treatment at all, save the possibility of hair transplants for some patients.3 However, it is important to note that in its early stages, CCCA can be treated, perhaps halted, and a significant amount of hair saved. As such, early identification and treatment of these patients is crucial. Many clinicians advocate a symptomatic treatment approach for patients diagnosed with CCCA (Table III). At the Henry Ford Hospital Multicultural Dermatology Center, our treatment approach is based not only on objective and histopathologic evidence of inflammation, but also on subjective symptoms of inflammation such as paresthesias, itching, and tenderness. First-line therapies at our center include mid- or high-potency topical steroids and intralesional corticosteroids.
Table III. Treatment of CCCA (1) (2) (3) (4) (5)
High-potency topical corticosteroids Intralesional corticosteroids Oral antibiotics (eg, doxycycline, tetracycline) Hydroxychloroquine Immunosuppressive agents (mycophenolate mofetil, cyclosporine) (6) Avoidance or limitation of hair grooming practices implicated in CCCA
Topical steroids are initially utilized daily, until stabilization, and then 3 days weekly for maintenance. Intralesional corticosteroids, ranging in potency up to 10 mg/mL, are administered monthly for a period of at least 6 months, and then symptomatically thereafter. For particularly aggressive cases, oral anti-inflammatory agents, such as tetracycline or minocycline, are added for a duration of typically no less than 6 months. Often, a seborrheic dermatitis treatment regimen is helpful in decreasing pruritus and scaling.3 This includes increasing hair-washing frequency to at least once weekly. Anecdotally, these aggressive anti-inflammatory treatments have been effective for some patients, particularly those presenting with early disease. Reversing existing scarring alopecia is not possible and should not be the goal of therapy. Target areas of therapy should be directed at the periphery of the alopecic region, including the surrounding normal-appearing scalp, in an effort to thwart progression of the inflammatory process. In addition to potent topical corticosteroids, and oral tetracyclines,18 some authors have found antimalarials to be useful.31,34 Other proposed treatments have included minoxidil, thalidomide, cyclosporine, mycophenolate mofetil, vitamins, and various herbal treatments.20,35,36 A significant response to an anti-inflammatory regimen is often noted within a few months, but many take up to 6 months. Once response is achieved, treatment frequency may be gradually tapered and then discontinued when remission is sustained for a full year.7 Avoidance of potentially damaging hair care practices such as chemical relaxers, excessive heat, traction, and hardening gels and sprays should be advised. Many authors have suggested a ‘‘chemicalfree’’ natural hair style.3 At our center, patients diagnosed with CCCA are strongly advised to discontinue all chemical relaxers, as well as all hair styles which are likely to be associated with excessive traction (cornrowing, braiding, weaving, or other extensions). For patients in whom ceasing use of chemical relaxers is not a viable option, it should be recommended that chemical services be
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professionally done (salon), that a base (usually petrolatum derivative) be applied to the entire scalp prior to relaxer applications, and that relaxers be applied no more frequently than every 8 to 10 weeks.3 In addition, patients should be advised that relaxers be left on for a maximum of 20 minutes, that they be applied within no more than 1 cm of the scalp surface, and that they never be applied in conjunction with other straightening or coloring agents.14 Such strict regulations are necessary due to the caustic nature of the agents, especially with inappropriate technique. For those individuals with end-stage scarring, camouflage techniques such as custom wigs, hair weaving, color sticks or crayons, and micropigmentation (permanent tattooing) can be recommended. Hair transplantation may be an option for some patients, but it should be noted that the presence of recipient site scarring presents a significant challenge and may decrease graft survival rates.3
CCCA: CONTROVERSIES AND UNANSWERED QUESTIONS Whether or not the unique hair care practices of those with African textured hair influences the pathogenesis of CCCA remains a source of ongoing debate, and to date there are few published retrospective studies regarding CCCA and grooming practices. Could chemical relaxers, traction, glues, or heat, or their chronicity of usage, predispose users to CCCA? Are those with CCCA exposed to the deleterious insult early in life, when scalp and follicular morphology is nascent? As Headington15 proposed, are follicular stem cells—repeatedly injured by grooming practices—lost, eventually leading to loss of the entire follicular structure? A further source of debate is the true race and sex epidemiology of CCCA. While vertex-centered alopecias in Caucasians has been described, whether or not this represents the same clinicopathologic entity as CCCA remains to be seen. If CCCA truly favors those individuals with African textured hair, as it clearly seems to, it should be questioned whether the unique shape of the hair shaft and follicles or the decrease in anchoring elastic fibers make these individuals uniquely susceptible to damage from certain styling or grooming methods.22 Also, while the female/male ratio of CCCA has been reported to be 3:1, the true sex ratio remains a source of ongoing debate. In addition, some physicians have anecdotally reported a possible increase in the incidence of CCCA among their patient populations. Whether this anecdotal increase portends a true increase in incidence, or perhaps indicates only a greater public savvy regarding dermatologic disease and greater
access to dermatologists, remains to be delineated. Clearly, further population-based studies are needed for epidemiologic clarification.
THE FUTURE Hair loss has a significant impact upon life quality and is commonly associated with loss of self-confidence and self-esteem.20 The patient with CCCA must be approached from a standpoint of cultural understanding and competence, which are imperative if therapeutic regimens and counseling are to be successful. There is a wealth of anecdotal knowledge where the pathogenesis and therapy of CCCA is concerned. Now, it is incumbent upon the dermatologic research community—clinical, epidemiological, basic science, and genetic—to further our knowledge about this devastating and epidemic form of alopecia that affects so much of our population. REFERENCES 1. Quinn CR, Quinn TM, Kelly AP. Hair care practices in African American women. Cutis 2003;72:280-9. 2. Callender VD. African-American scalp disorders and treatment considerations. Skin Aging 2002;10(S):12-4. 3. Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther 2004; 17:164-76. 4. Halder RM, Grimes PE, McLaurin CI, Kress MA, Kenney JA Jr. Incidence of common dermatoses in a predominantly black dermatologic practice. Cutis 1983;32:378-80. 5. Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg 2006;25:41-50. 6. Sperling LC, Sau P. The follicular degeneration syndrome in black patients. Arch Dermatol 1992;128:68-74. 7. Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol 2005;53:1-37. 8. Templeton SF, Solomon AR. Scarring alopecia: a classification based on microscopic criteria. J Cutan Pathol 1994;21:97-109. 9. Olsen EA, Bergfeld WF, Cotsarelis G, Price VH, Shapiro J, Sinclair R, et al. Summary of North American Hair Research Society (NAHRS)esponsored workshop on cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol 2003;48:103-10. 10. Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol 2006;28:236-59. 11. LoPresti P, Papa CM, Kligman AM. Hot comb alopecia. Arch Dermatol 1968;98:234-8. 12. Price VH. Hair loss in cutaneous disease. In: Baden HP, editor. Symposium on Alopecia. New York: HP Publishing Co; 1987. pp. 3-11. 13. Whiting DA. Current concepts: the diagnosis of alopecia. Kalamazoo (MI): The Upjohn Co; 1990. p. 24. 14. Nicholson AG, Harland CC, Bull RH, Mortimer PS, Cook MG. Chemically induced cosmetic alopecia. Br J Dermatol 1993; 128:537-41. 15. Headington JT. Cicatricial alopecia. Dermatol Clin 1996;14: 773-82. 16. Sperling LC, Solomon AR, Whiting DA. A new look at scarring alopecia. Arch Dermatol 2000;136:235-42. 17. Sperling LC. Scarring alopecia and the dermatopathologist. J Cutan Pathol 2001;28:333-42.
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18. Sperling LC, Skelton HG III, Smith KJ, Sau P, Friedman K. Follicular degeneration syndrome in men. Arch Dermatol 1994;130:763-9. 19. Franbourg A, Hallegot P, Baltenneck F, Toutain C, Leroy F. Current research on ethnic hair. J Am Acad Dermatol 2003;48: S115-9. 20. McMichael AJ. Ethnic hair updates: past and present. J Am Acad Dermatol 2003;48:S127-33. 21. Kamath YK, Hornby SB, Weigmann HD. Mechanical and fractographic behavior of negroid hair. J Soc Cosmet Chem 1984;35:21-43. 22. Taylor SC. Skin of color: biology, structure, function and implications for dermatologic disease. J Am Acad Dermatol 2002;46:S41-62. 23. Richards GM, Oresajo CO, Halder RM. Structure and function of ethnic skin and hair. Dermatol Clin 2003;21:595-600. 24. Burrall BA. Ethnic skin: a spectrum of issues. CME Medscape. com/viewartile/529349 ª2006. 25. Kaur BJ, Singh H, Lin-Greenberg A. Irritant contact dermatitis complicated by deep-seated staphylococcal infection caused by a hair relaxer. J Natl Med Assoc 2003;94:121-3. 26. Nnoruka NE. Hair loss: is there a relationship with hair care practices in Nigeria? Int J Dermatol 2005;44(S1):13-7.
27. Swee W, Klontz KC, Lambert LA. A nationwide outbreak of alopecia associated with the use of a hair-relaxing formulation. Arch Dermatol 2000;136:1104-8. 28. Fox GN, Stausmire JM, Mehregan DR. Traction folliculitis: an underreported entity. Cutis 2007;79:26-30. 29. Wakelin SH. Contact anaphylaxis from natural latex used as an adhesive for hair extensions. Br J Dermatol 2002;146:340-1. 30. Cogen FC, Beezhold DH. Hair glue anaphylaxis: a hidden latex allergy. Ann Allergy Asthma Immunol 2002;88:61-3. 31. McMichael AJ. Hair and scalp disorders in ethnic populations. Dermatol Clin 2003;21:629-44. 32. Wilborn WS. Disorders in hair growth in African Americans. In: Olsen EA, editor. Disorders of hair growth. New York: McGrawHill; 1994. pp. 389-407. 33. Ackerman AB, Walton NW, Jones RE, et al. ‘‘Hot comb alopecia:/’’ Follicular degeneration syndrome’’ in African-American women: is traction alopecia! Dermatopathol Pract Concept 2000;6-21. 34. Bulengo-Ransby SM. Chemical and traumatic alopecia from thioglycolate in a black woman. Cutis 1992;49:99-103. 35. Scott DA. Disorders of the hair and scalp in blacks. Dermatol Clin 1988;6:387-95. 36. Price VH. The medical treatment of cicatricial alopecia. Semin Cutan Med Surg 2006;25:56-9.
Central centrifugal cicatricial alopecia: Past, present, and future Raechele Cochran Gathers, MD, and Henry W. Lim, MD Detroit, Michigan See related article on page 574 Clinical scarring alopecia in African American women has been recognized for years. The classification of this unique form of alopecia dates back to Lopresti, who first described the entity called ‘‘hot comb alopecia.’’ More recently, the term ‘‘central centrifugal cicatricial alopecia’’ has been adopted to describe a progressive vertex-centered alopecia most common in women of African descent. While this form of hair loss is widely recognized, and may even be on the rise, the causes of central centrifugal cicatricial alopecia are a constant source of debate and remain to be elucidated. This review outlines the descriptive evolution of central centrifugal cicatricial alopecia and the historical controversies ascribed to its pathoetiology; it also examines African hair structure and discusses how hair structure along with common physical and chemical implements utilized by individuals with African hair type may play a causal role in the development of central centrifugal cicatricial alopecia. ( J Am Acad Dermatol 2009;60:660-8.)
H
air loss among African American women is exceedingly common. Although ethnic hair care is a billion-dollar industry,1,2,3 with innumerable products and services purportedly designed to meet the unique needs of ethnic hair, a disproportionate number of African American women still suffer from hair loss. Halder et al,4 in 1983, described alopecias as the fifth most common dermatosis in African Americans, with chemical and traction alopecias cited as the predominant types. While there are no recently published epidemiologic data describing the number of African American women presenting with a primary diagnosis of alopecia, in the present authors’ opinion, the incidence in this group may be even higher than that reported by Halder. Among African Americans, central centrifugal cicatricial alopecia (CCCA) has been purported to be responsible for more cases of scarring alopecia than all other forms combined.5 However, it should be noted that true epidemiologic and prevalence data do not exist to date. Clinically, CCCA develops
From the Multicultural Dermatology Center, Department of Dermatology, Henry Ford Hospital. Funding sources: None. Conflicts of interest: None declared. Reprint requests: Raechele Cochran Gathers, MD, Multicultural Dermatology Center, Henry Ford Hospital, 3031 W Grand Blvd, Suite 800, Detroit, MI 48202. E-mail: [email protected]. 0190-9622/$36.00 ª 2008 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2008.09.066
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Abbreviations used: CCCA: CCSA: FDS: IRS: ORS:
central centrifugal cicatricial alopecia central centrifugal scarring alopecia follicular degeneration syndrome inner root sheath outer root sheath
as a scarring, roughly circular area on the crown and/or vertex of the scalp.3 With time, this scarred centralized area progresses centrifugally. The affected scalp is in parts smooth and shiny and illustrates massive follicular dropout. Typically, a few short, brittle hairs remain within the scarred expanse (Fig 1). Often, patients complain of dysesthesia (tenderness, itching) in the affected area.3,6 Anecdotally, dysesthesias may be associated with progression of the alopecia and active inflammation. Scarring, or cicatricial alopecia, is characterized by visible loss of follicular ostia and follicular destruction.7,8 In contrast to nonscarring (non-cicatricial) alopecia, which is reversible since follicular epithelium remains intact, cicatricial alopecia is, at its end stages, irreversible, and follicular epithelium is replaced by connective tissues.5 Cicatricial alopecia can be subdivided into both primary and secondary types, depending on the pattern of follicular destruction. In primary cicatricial alopecia, the hair follicle itself is the target of inflammation, with relative sparing of the interfollicular reticular dermis.8 Primary cicatricial alopecias can be further subdivided into 4 categories: lymphocytic (CCCA), neutrophilic, mixed, and non-specific.9 In contrast to
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primary cicatricial alopecia, in secondary cicatricial alopecia, the hair follicle is an ‘‘innocent bystander,’’5 destroyed nonspecifically and by consequence (such as by neoplasm, sarcoidosis, or pemphigus vulgaris).7 Sellheyer and Bergfeld10 and Ross, Tan, and Shapiro7 have already provided in-depth reviews of both the histopathologic evaluation of alopecias and the classification of primary cicatricial alopecias, respectively. This article focuses on present knowledge regarding CCCA, unanswered questions and controversies, and possible future directions for both clinical and bench research.
CCCA: AN EVOLVING NOMENCLATURE AND DESCRIPTION The nomenclature and classification schemes describing clinical cicatricial alopecia in African American women has been a source of some debate, and has varied fairly widely over the years (Fig 2). LoPresti, Papa, and Kligman11 in 1968, first described an ‘‘irreversible alopecia of the scalp’’ occurring in 51 African American women who straightened their hair using a hot comb. They described an ‘‘absolutely characteristic’’ course and configuration of alopecia beginning on the crown, which, given time, spread peripherally to form a large circle of alopecia, which spared the lateral and posterior aspects of the scalp. They described the scalp surface as shiny, with an obvious decrease in the density of follicular orifices. Clinical inflammatory changes, such as scaling, were not described and the scalp was described as ‘‘soft and pliable, not hard and immovable as in discoid lupus erythematosus or morphea.’’11 LoPresti, Papa, and Kligman described the alopecia as incomplete, with some seemingly normal hairs always remaining within the area of involvement. This unique and eventually irreversible form of alopecia was termed ‘‘hot comb alopecia,’’ and it was hypothesized that petrolatum, heated during thermal hair straightening with a hot comb, caused a chronic inflammation around the upper segment of the hair follicle, leading to external root sheath degeneration, follicle destruction and, finally, follicular scarring. There was little mention of hot comb alopecia in the medical literature for more than two decades subsequent to the article by LoPresti, Papa, and Kligman.11 This peculiar form of scarring hair loss in African American women was occasionally mentioned in review articles on alopecia,6,12,13 but was not the primary issue of focus or study. In 1992, Sperling and Sau6 proposed the new term follicular degeneration syndrome (FDS) for what had previously been called ‘‘hot comb alopecia.’’ Sperling and Sau retrospectively studied 10 black women with the characteristic hair loss pattern of hot comb alopecia.
Fig 1. CCCA in patient with history of hair relaxer use.
However, because they noted poor correlation between the usage of a hot comb and the onset or progression of this particular form of scarring alopecia, they hypothesized that use of a hot comb was indeed not the cause of hair loss in this group of women. In fact, they described women with clinical disease who had never used a hot comb in their lives. Sperling and Sau hypothesized that two particular histological features: (1) premature inner root sheath (IRS) degeneration and (2) migration of the hair shaft through the outer root sheath (ORS), were distinctive in their cohort of patients, and they proposed the new terminology of FDS to describe what was thought to be a clinically and histologically distinctive form of scarring alopecia seen in African American women.6 ‘‘Follicular degeneration syndrome’’ is a descriptive term, though, and does not implicate a particular cause. While hot combs were refuted by Sperling and Sau as a cause of FDS, they conceded that hot combs, or other styling methods, might contribute to the observed follicular damage. They suggested that in FDS, abnormal follicles might be predisposed to external injury, but remain intact as long as the scalp was not irritated. It was hypothesized that the use of hair relaxers, traction, or other chemicals might create irritation that was sufficient to act as a catalyst for FDS.6 In 1992, Nicholson et al14 described a pattern of scarring alopecia on the scalp vertex in 8 young females of Afro-Caribbean ancestry in the south London area of Great Britain. The histologic patterns described by Nicholson et al were consistent with those detailed by Sperling and Sau6 and similarly differed from that described by LoPresti, Papa, and Kligman11 in the lack of epidermal atrophy noted. Nicholson et al14 coined the term chemically induced cosmetic alopecia and hypothesized that the observed alopecia was related to the combination of chemical and physical insults to the hair and scalp. In a 1996 review of cicatricial alopecia, Headington15 refuted the hypothesis that the
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Fig 2. Nomenclature evolution of CCCA.
so-called FDS was a clinicopathologic entity. Headington maintained that selective premature degeneration of the follicular inner hair sheath could be seen in a variety of different scarring alopecias and was therefore neither specific nor diagnostic; it was probably a result of altered outer hair sheath biology by cell mediated injury or trauma. Headington, referring to the stereotypic hair loss seen in African American women as ‘‘scarring alopecia in African Americans,’’ concluded that in scarring hair loss of the type predominant in African American women, follicular stem cells had been repeatedly injured by chemical or physical trauma (ie, relaxers, traction) so that the entire follicular structure was ultimately lost. In a 2000 editorial, Sperling, Solomon, and Whiting16 described the term central centrifugal scarring alopecia (CCSA) to encompass a group of closely related clinical patterns of hair loss. Hair loss centered on the crown of the scalp, chronic and progressive disease with eventual ‘‘burnout’’, symmetrical expansion with the most active disease at the periphery, and clinical and histologic evidence of inflammation in the active peripheral zone were described as common features of CCSA.16 The term FDS was included under the subset of CCSA, as was pseudopelade (not to be confused with classic pseudopelade of Brocq) and folliculitis decalvans. While FDS was described to be a form of CCSA, it was noted that FDS demonstrated the distinctive histologic finding of premature desquamation of the IRS in both inflamed follicles and non-inflamed ‘‘normal’’ scalp.16 Most recently, in the 2001 Summary of the North American Hair Research Societyesponsored Workshop on Cicatricial Alopecia, published in 2003, the currently popular term CCCA was proposed.9 CCCA describes a form of lymphocytic
primary cicatricial alopecia characterized by hair loss starting in the central scalp and progressing centrifugally. CCCA was proposed as a more descriptive term that was thought to encompass what had previously been termed FDS, hot comb alopecia, pseudopelade in African Americans and central elliptical pseudopelade in Caucasians.
EPIDEMIOLOGY OF CCCA While there are sparse epidemiologic data on the racial and sex distribution of CCCA, the vast majority of reports describe women of African ancestry. LoPresti, Papa, and Kligman11 described 51 African American women in their description of hot comb alopecia, but also reported one Caucasian woman with an ‘‘identical clinical state.’’ In their 1992 description of FDS, Sperling and Sau6 described 10 black women. In their review of chemically induced cosmetic alopecia from Europe, Nicholson et al14 described female patients of Afro-Caribbean descent. A central scarring alopecia in Caucasians, central elliptical pseudopelade in Caucasians, is mentioned rarely in the literature,7,9 and descriptions of centrally patterned scarring alopecia in Caucasians may very well represent pseudopelade of Brocq, which may have similar histopathologic features to CCCA, but clinically is very different, typically presenting with numerous irregularly outlined alopecic patches, classically defined as ‘‘footprints in the snow.’’10 Pseudopelade of Brocq typically affects white adults, and the disease is often characterized by spurts of disease activity followed by dormant periods, unlike CCCA, which typically has a slow and steady progression.16,17 Like racial predilection, there are few data regarding sex distribution of CCCA. Sperling and Cowper5 have described a female/male ratio of 3:1 among black patients and, in a 1994 observation, reported 8
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men with purported FDS.18 Overall, this group of men had more evidence of active inflammation than did the previously described women with FDS, often illustrating follicular and perifollicular papules in a pattern less commonly described in women with a similar diagnosis. Whether the male patients reported by Sperling18 are true cases of what is now termed CCCA is debatable, and in the present authors’ opinion, the female/male ratio in CCCA is much higher than 3:1. While it is clear that women of African descent appear to be disproportionately affected by CCCA, true epidemiologic data with regard to race and sex distribution still need to be elucidated.
HISTOLOGY OF CCCA In their landmark 1968 description of ‘‘hot comb’’ alopecia, LoPresti, Papa, and Kligman11 described ORS degeneration in association with superficial perifollicular lymphocytic infiltrate and epidermal atrophy with elongated rete ridges and hypogranulosis, with ultimate replacement of the follicular unit, save the arrector pili muscle, with dense bands of eosinophilic collagen.7,11 Later, Sperling and Sau6 described two histologic features as being unique in what they had termed FDS: (1) premature degeneration of the IRS and (2) migration of the hair shaft through the ORS. Sperling and Sau also described a marked diminution in hair follicle density with some follicles being totally replaced by fibrosis, as noted by LoPresti, Papa, and Kligman. The epidermal atrophy and thinned and elongated rete ridges described by LoPresti, Papa, and Kligman were not prominent features noted by Sperling and Sau. Other histologic findings included a mononuclear cell infiltrate and lamellar fibroplasias at the level of the isthmus; disintegration of the follicular epithelium; disintegration of the ‘‘naked’’ hair shafts with surrounding foreign body giant cell reaction; and replacement of the entire follicle by thick fibrous tracts. However, these findings were noted by Sperling and Sau6 to be found in other forms of scarring alopecia as well, and were not described as unique to FDS. In contrast to Sperling and Sau,6 Headington15 in his discussion of vertex scarring in African Americans described the histologic findings of selective premature degeneration of the IRS as being nonspecific and present in a variety of different scarring alopecias, including chronic cutaneous lupus. However, Headington described other findings similar to those noted by Sperling and Sau: primary follicular scarring with decreased follicular density, loss of sebaceous epithelium, follicular fusion, premature fragmentation of the IRS, and slight perivascular lymphocytic inflammation. Late-stage lesions were characterized
by destruction of pilosebaceous units, dermal scarring, and dermal lymphocytic and plasma cell infiltrate. In the so-called ‘‘chemically induced cosmetic alopecia’’ described by Nicholson et al,14 histologic findings were largely similar to those previously described in hot comb alopecia, FDS, vertex scarring in African Americans and CCCA, with perifollicular chronic inflammatory cells, perifollicular fibrosis in a lamellar pattern, naked hair shafts within the dermis, and eventual total replacement of hair follicles by fibrosis. Whether CCCA is a distinct clinicopathologic entity or whether histopathologic changes are nonspecific and seen in other forms of scarring alopecia remains a controversy. However, common histopathologic themes do exist and are commonly accepted: a lymphocytic folliculitis, perifollicular granulomatous inflammation with hair shaft foreign body giant cells, prominent destruction of the folliculosebaceous units with scar tissue fibrosis at the sites of former hair follicles, and common retention of arrector pili muscles.7
AFRICAN HAIR STRUCTURE As it appears clear that CCCA disproportionately affects those of African descent, the unique structural properties of African hair must be considered. In general, hair in people of African descent is elliptical in cross section, with the physical shape resembling a twisted oval rod.19,20 By contrast, the hair of Asians demonstrates a round hair shaft with a very large diameter. Caucasian hair generally appears structurally between the two. While Asian hair has a circular geometry, African hair presents a high degree of irregularity in the diameter along the hair shaft, with frequent twists and random reversals in direction with pronounced flattening.19,21 The curly spiraled nature of African hair is believed to be caused by the curvature of the hair follicle. It is largely held that, because of its frequent twists and spiraling, African hair generally has less tensile strength and breaks more easily than Caucasian hair.19 Additionally, in its natural state, its curly configuration makes it less amenable to traditional combing methods. The tensile strength of a fiber is highly dependent on its section size. African hair differs from both Caucasian and Asian hair by its earlier breaking time under force and by its lower stress requirements at breaking.19 It is hypothesized that the lower tensile strength and relative brittleness of African hair may be explained by its morphology and geometry, with natural constrictions along the twisted hair shaft leading to microcracks and fractures in the hair fiber, exacerbated by everyday combing and grooming.
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Table I. Popular styling methods among African Americans (1) (2) (3) (4) (5) (6)
Braiding/cornrowing—Interlocking of 3 or more pieces of hair to create a loose or bound-down 3-dimensional hairstyle Twisting—Interlocking of 2 pieces of hair to create a loose or bound-down 3-dimensional hairstyle Thermal straightening (pressing)—Metal comb heated to approximately 3508F to achieve temporary straightening Chemical straightening—Application of alkaline chemicals to the hair, which causes permanent hair straightening Hair weaving—Addition of exogenous human or synthetic hair by sewing or bonding glue application Locking—Twists of hair allowed to remain, unmanipulated, such that the hair eventuates in permanently interlocking strands
While the proteins and amino acids constituting hair keratin are similar among races, there are other known differences between hair in individuals of Caucasian and African descent: African hair has fewer elastic fibers anchoring the hair follicles to the dermis22,23; melanosomes have been observed to be in both the ORS and the bulb of vellus hairs in African hair; African hair has more pigment and on microscopy has larger melanin granules in comparison with other hair types23; and African hair has less moisture content than Caucasian hair.19
AFRICAN AMERICAN HAIR CARE Just as its tight curl pattern may make African textured hair more likely to break and fracture, and may arguably make it more susceptible to disorders not largely seen in individuals with differing hair types, the culturally unique grooming patterns and practices among those with African textured hair may also make them uniquely susceptible to a myriad of dermatologic quandaries (Table I). Thermal hair straightening relaxes the tight coils of African textured hair by temporarily rearranging hydrogen bonds within the hair shafts.3 The application of heat, used to straighten the typically tightly coiled hair, has long been hypothesized to play a role in the etiology of hair and scalp disease among individuals with African textured hair. As previously discussed, LoPresti, Papa, and Kligman11 implicated thermal straightening by a hot comb as an etiologic factor for what is now called CCCA. While modernday hot combs and flat irons often feature protective ceramic surfaces and self-dialed heat controls (Fig 3), many women still straighten their hair with traditional metal combs, heated to 3008-5008 F on a stovetop or in an electric heating oven. Thermal burns are a common sequela to this method of hair straightening, as is hair breakage resulting from excessive heat causing drying and brittleness of the hair shaft. Whether CCCA is linked to thermal straightening and other heat sources (ie, blow dryer, heated curling irons) remains controversial. Chemical straightening is another hair care practice widely practiced by women with African textured hair. It has been estimated that 70% to 80% of
African American women use chemical applications to achieve a straighter hair style.24 Chemical straighteners have been in existence since at least the early 1900’s, when early chemicals such as lye, hog lard, and boiled eggs were used to achieve a straighter hair texture.20 In the mid 1900’s, chemical hair straighteners (termed relaxers since they relax the normally tight coils of African textured hair) became more widely used. These alkaline relaxers typically contain sodium, potassium, lithium, and guanine hydroxides, or, more rarely, sulfites and thioglycates, which work to permanently break the hydrogen/ disulfide bonds of curly hair, thus leaving it in a straighter formation.20 Unfortunately, chemical relaxers have been long documented to cause cuticular disruption, hair shaft damage, scalp irritation and chemical burns. In addition, chemical relaxers have also been implicated in the development of severe toxic irritant contact dermatitis and subsequent Staphylococcus aureus abscess formation.25 Most cases of damage from relaxers are typically caused by inappropriate application and usage.3 However, chemical hair relaxers have been hypothesized as being, if not a causative agent for scarring hair loss, at least an exacerbating factor. Headington15 has described prolonged cosmetic hair care practices in African Americans, such as chemical straightening with sodium, potassium, and lithium hydroxide, as being ‘‘suspect’’ in the pathogenesis of CCCA. In an African study of 39 female patients, Nnoruka26 found that women with scarring alopecia complained of local chemical burns and contact scalp irritation after too frequent and prolonged use of chemical relaxers. It was hypothesized that misuse of lye containing relaxers may have triggered a primary irritation, subsequent inflammation, and fibrosis, and ultimately a scarring alopecia. Chemically induced injury has also been suggested by Nicholson et al. as a causative factor in the development of scarring alopecia in their cohort of women of Afro-Caribbean descent. Nicholson et al14 hypothesized that the observed alopecia was related not to alkali relaxer misuse alone, but to a sum effect of physical and chemical insults that caused a natural progression from mild fibrosis and inflammation to
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end-stage scarring. More recently, Swee, Klontz, and Lambert27 published a report of a national outbreak of alopecia associated with a nontraditional acidic hair relaxer that included metal salts in its formulation. The FDA received (to date of this writing) the largest number of complaints the agency had ever received for a cosmetic product for this hair-straightening system, which caused hair breakage and loss in 95% of survey respondents, with some 9% still reporting no hair regrowth some 2 years after using the product. While chemical relaxers have not been definitively implicated as causative of CCCA, their misuse may be considered a pathogenic factor, perhaps one of many, in its evolution. At our center, we recommend that patients diagnosed with CCCA avoid chemical relaxers, as we believe that the associations proposed by Nnoruka26 and Nicholson et al14 are not unlikely. Clearly, however, without well-designed and well-conducted studies, this is still conjecture. Additional hair care practices that are largely unique to those with African textured hair include the widespread use of hair braiding or cornrowing, often with substantial amounts of added human or synthetic hair for additional length and volume. Braiding and extensions have been associated with excessive traction with subsequent folliculitis and alopecia, as well as with allergic contact dermatitis resulting from synthetic hair.3,28 Hair weaving typically involves the addition of color- and texture-matched synthetic or human hair extensions. Hair extensions may be added by sewing in the exogenous hair or by using a synthetic bonding glue.3 Hair weaving has been implicated in traction alopecia, folliculitis, and allergic reaction to the bonding glue.3,29,30 It is documented that African textured hair has fewer elastic fibers anchoring hair follicles to the dermis compared with that of Caucasians.22 Excessive weight from hair extensions, ponytails, or weaves may uniquely impact those with African textured hair. In a recently conducted study at our center, presented at the 2007 L’Oreal Institute for Ethnic Hair and Skin Research International Symposium, we found that certain hair grooming practices do appear to be more common in women in whom CCCA develops; these include the use of both sewn and glued hair weave as well as the use of cornrow or braided hairstyles with added extension hair. Furthermore, CCCA patients wearing these 3 implicated hairstyles were more likely to report a history of hair damage, reflected as a history of tender scalp or uncomfortable pulling. This seems to implicate, at least in part, either traction or perhaps an irritant dermatitis from synthetic hair and glue in the development of CCCA.
Fig 3. Thermal straightening implement: Hot comb.
A final aspect of grooming practices among persons with African textured hair which merits mention is the use of thick emollients as a hair lubricant, and styling gels and spritzes used to aid in hair styling. Historically, many African Americans have utilized thick hair lubricants (‘‘hair grease’’) in an effort to moisturize and style the hair, as well as to aid in ease of grooming. These emollients often contain mineral oils, petrolatum or lanolin and vegetable oils, which have been reported to worsen the inflammation of seborrheic dermatitis.31 Styling gels and spritzes containing a copolymer of polyvinyl pyrrolidone and vinyl acetates coat the hair. As they are often left on the hair for extended periods (weeks) between washings, they may exacerbate already dry and brittle chemically relaxed hair, and lanolin additives may exacerbate seborrhea.32 While our center’s recently presented study on grooming practices associated with CCCA found no clear correlation between the use of hair emollients or styling preparations with CCCA, it is still possible that this additional inflammatory insult may exacerbate the concurrent inflammation noted in the development of CCCA.
ETIOLOGY: A QUANDARY The etiology of CCCA remains controversial (Table II). Sperling, in his description of FDS, has hypothesized that the condition results from early degeneration of the IRS which allows the hair shaft to impinge upon the exposed soft ORS and subsequently precipitate a chain of histopathologic events which result in scarring.6,10 Ackerman et al10,33 have described what is now termed CCCA as a form of
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Table II. Factors implicated in the development of CCCA (1) (2) (3) (4)
Thermal straightening (hot comb, flat iron) Chemical straightening (relaxer) Traction/physical trauma (braiding, cornrows, weaves) Irritant dermatitis (glues, hair dressings)
traction alopecia, while Headington postulates that follicular stem cells are repeatedly injured by chemical and/or physical trauma so that the entire follicular structure is ultimately destroyed. In a recently completed study by the present author, we have found that both sewn and glued hair weave as well as the use of cornrow or braided hairstyles with added extension hair seem to be positively associated with CCCA. This finding appears to support, at least in part, the theories of both Ackerman et al33 and Headington.15 Nnoruka26 implicates duration of hair care practices, such as relaxers, in the development of CCCA. Other authors31,33 have reported a scarring alopecia that has been temporally or historically related to chemical relaxers. In an unpublished study, McMichael found that most African American patients without scarring had discontinued relaxing compared with those with scarring alopecia.20 Our recently presented study found no clear association between chemical relaxers and CCCA. However, because studies are limited to date, we still support the discontinuation of chemical relaxers in those with CCCA. Clearly, further population-based study is needed to delineate causative agents involved in the pathogenesis of CCCA.
TREATMENT OF CCCA To date, there are no published case-controlled prospective studies documenting effective treatment of CCCA. In fact, as CCCA is an ultimately scarring process, at its very late stages there is likely to be no treatment at all, save the possibility of hair transplants for some patients.3 However, it is important to note that in its early stages, CCCA can be treated, perhaps halted, and a significant amount of hair saved. As such, early identification and treatment of these patients is crucial. Many clinicians advocate a symptomatic treatment approach for patients diagnosed with CCCA (Table III). At the Henry Ford Hospital Multicultural Dermatology Center, our treatment approach is based not only on objective and histopathologic evidence of inflammation, but also on subjective symptoms of inflammation such as paresthesias, itching, and tenderness. First-line therapies at our center include mid- or high-potency topical steroids and intralesional corticosteroids.
Table III. Treatment of CCCA (1) (2) (3) (4) (5)
High-potency topical corticosteroids Intralesional corticosteroids Oral antibiotics (eg, doxycycline, tetracycline) Hydroxychloroquine Immunosuppressive agents (mycophenolate mofetil, cyclosporine) (6) Avoidance or limitation of hair grooming practices implicated in CCCA
Topical steroids are initially utilized daily, until stabilization, and then 3 days weekly for maintenance. Intralesional corticosteroids, ranging in potency up to 10 mg/mL, are administered monthly for a period of at least 6 months, and then symptomatically thereafter. For particularly aggressive cases, oral anti-inflammatory agents, such as tetracycline or minocycline, are added for a duration of typically no less than 6 months. Often, a seborrheic dermatitis treatment regimen is helpful in decreasing pruritus and scaling.3 This includes increasing hair-washing frequency to at least once weekly. Anecdotally, these aggressive anti-inflammatory treatments have been effective for some patients, particularly those presenting with early disease. Reversing existing scarring alopecia is not possible and should not be the goal of therapy. Target areas of therapy should be directed at the periphery of the alopecic region, including the surrounding normal-appearing scalp, in an effort to thwart progression of the inflammatory process. In addition to potent topical corticosteroids, and oral tetracyclines,18 some authors have found antimalarials to be useful.31,34 Other proposed treatments have included minoxidil, thalidomide, cyclosporine, mycophenolate mofetil, vitamins, and various herbal treatments.20,35,36 A significant response to an anti-inflammatory regimen is often noted within a few months, but many take up to 6 months. Once response is achieved, treatment frequency may be gradually tapered and then discontinued when remission is sustained for a full year.7 Avoidance of potentially damaging hair care practices such as chemical relaxers, excessive heat, traction, and hardening gels and sprays should be advised. Many authors have suggested a ‘‘chemicalfree’’ natural hair style.3 At our center, patients diagnosed with CCCA are strongly advised to discontinue all chemical relaxers, as well as all hair styles which are likely to be associated with excessive traction (cornrowing, braiding, weaving, or other extensions). For patients in whom ceasing use of chemical relaxers is not a viable option, it should be recommended that chemical services be
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professionally done (salon), that a base (usually petrolatum derivative) be applied to the entire scalp prior to relaxer applications, and that relaxers be applied no more frequently than every 8 to 10 weeks.3 In addition, patients should be advised that relaxers be left on for a maximum of 20 minutes, that they be applied within no more than 1 cm of the scalp surface, and that they never be applied in conjunction with other straightening or coloring agents.14 Such strict regulations are necessary due to the caustic nature of the agents, especially with inappropriate technique. For those individuals with end-stage scarring, camouflage techniques such as custom wigs, hair weaving, color sticks or crayons, and micropigmentation (permanent tattooing) can be recommended. Hair transplantation may be an option for some patients, but it should be noted that the presence of recipient site scarring presents a significant challenge and may decrease graft survival rates.3
CCCA: CONTROVERSIES AND UNANSWERED QUESTIONS Whether or not the unique hair care practices of those with African textured hair influences the pathogenesis of CCCA remains a source of ongoing debate, and to date there are few published retrospective studies regarding CCCA and grooming practices. Could chemical relaxers, traction, glues, or heat, or their chronicity of usage, predispose users to CCCA? Are those with CCCA exposed to the deleterious insult early in life, when scalp and follicular morphology is nascent? As Headington15 proposed, are follicular stem cells—repeatedly injured by grooming practices—lost, eventually leading to loss of the entire follicular structure? A further source of debate is the true race and sex epidemiology of CCCA. While vertex-centered alopecias in Caucasians has been described, whether or not this represents the same clinicopathologic entity as CCCA remains to be seen. If CCCA truly favors those individuals with African textured hair, as it clearly seems to, it should be questioned whether the unique shape of the hair shaft and follicles or the decrease in anchoring elastic fibers make these individuals uniquely susceptible to damage from certain styling or grooming methods.22 Also, while the female/male ratio of CCCA has been reported to be 3:1, the true sex ratio remains a source of ongoing debate. In addition, some physicians have anecdotally reported a possible increase in the incidence of CCCA among their patient populations. Whether this anecdotal increase portends a true increase in incidence, or perhaps indicates only a greater public savvy regarding dermatologic disease and greater
access to dermatologists, remains to be delineated. Clearly, further population-based studies are needed for epidemiologic clarification.
THE FUTURE Hair loss has a significant impact upon life quality and is commonly associated with loss of self-confidence and self-esteem.20 The patient with CCCA must be approached from a standpoint of cultural understanding and competence, which are imperative if therapeutic regimens and counseling are to be successful. There is a wealth of anecdotal knowledge where the pathogenesis and therapy of CCCA is concerned. Now, it is incumbent upon the dermatologic research community—clinical, epidemiological, basic science, and genetic—to further our knowledge about this devastating and epidemic form of alopecia that affects so much of our population. REFERENCES 1. Quinn CR, Quinn TM, Kelly AP. Hair care practices in African American women. Cutis 2003;72:280-9. 2. Callender VD. African-American scalp disorders and treatment considerations. Skin Aging 2002;10(S):12-4. 3. Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther 2004; 17:164-76. 4. Halder RM, Grimes PE, McLaurin CI, Kress MA, Kenney JA Jr. Incidence of common dermatoses in a predominantly black dermatologic practice. Cutis 1983;32:378-80. 5. Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg 2006;25:41-50. 6. Sperling LC, Sau P. The follicular degeneration syndrome in black patients. Arch Dermatol 1992;128:68-74. 7. Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol 2005;53:1-37. 8. Templeton SF, Solomon AR. Scarring alopecia: a classification based on microscopic criteria. J Cutan Pathol 1994;21:97-109. 9. Olsen EA, Bergfeld WF, Cotsarelis G, Price VH, Shapiro J, Sinclair R, et al. Summary of North American Hair Research Society (NAHRS)esponsored workshop on cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol 2003;48:103-10. 10. Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol 2006;28:236-59. 11. LoPresti P, Papa CM, Kligman AM. Hot comb alopecia. Arch Dermatol 1968;98:234-8. 12. Price VH. Hair loss in cutaneous disease. In: Baden HP, editor. Symposium on Alopecia. New York: HP Publishing Co; 1987. pp. 3-11. 13. Whiting DA. Current concepts: the diagnosis of alopecia. Kalamazoo (MI): The Upjohn Co; 1990. p. 24. 14. Nicholson AG, Harland CC, Bull RH, Mortimer PS, Cook MG. Chemically induced cosmetic alopecia. Br J Dermatol 1993; 128:537-41. 15. Headington JT. Cicatricial alopecia. Dermatol Clin 1996;14: 773-82. 16. Sperling LC, Solomon AR, Whiting DA. A new look at scarring alopecia. Arch Dermatol 2000;136:235-42. 17. Sperling LC. Scarring alopecia and the dermatopathologist. J Cutan Pathol 2001;28:333-42.
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18. Sperling LC, Skelton HG III, Smith KJ, Sau P, Friedman K. Follicular degeneration syndrome in men. Arch Dermatol 1994;130:763-9. 19. Franbourg A, Hallegot P, Baltenneck F, Toutain C, Leroy F. Current research on ethnic hair. J Am Acad Dermatol 2003;48: S115-9. 20. McMichael AJ. Ethnic hair updates: past and present. J Am Acad Dermatol 2003;48:S127-33. 21. Kamath YK, Hornby SB, Weigmann HD. Mechanical and fractographic behavior of negroid hair. J Soc Cosmet Chem 1984;35:21-43. 22. Taylor SC. Skin of color: biology, structure, function and implications for dermatologic disease. J Am Acad Dermatol 2002;46:S41-62. 23. Richards GM, Oresajo CO, Halder RM. Structure and function of ethnic skin and hair. Dermatol Clin 2003;21:595-600. 24. Burrall BA. Ethnic skin: a spectrum of issues. CME Medscape. com/viewartile/529349 ª2006. 25. Kaur BJ, Singh H, Lin-Greenberg A. Irritant contact dermatitis complicated by deep-seated staphylococcal infection caused by a hair relaxer. J Natl Med Assoc 2003;94:121-3. 26. Nnoruka NE. Hair loss: is there a relationship with hair care practices in Nigeria? Int J Dermatol 2005;44(S1):13-7.
27. Swee W, Klontz KC, Lambert LA. A nationwide outbreak of alopecia associated with the use of a hair-relaxing formulation. Arch Dermatol 2000;136:1104-8. 28. Fox GN, Stausmire JM, Mehregan DR. Traction folliculitis: an underreported entity. Cutis 2007;79:26-30. 29. Wakelin SH. Contact anaphylaxis from natural latex used as an adhesive for hair extensions. Br J Dermatol 2002;146:340-1. 30. Cogen FC, Beezhold DH. Hair glue anaphylaxis: a hidden latex allergy. Ann Allergy Asthma Immunol 2002;88:61-3. 31. McMichael AJ. Hair and scalp disorders in ethnic populations. Dermatol Clin 2003;21:629-44. 32. Wilborn WS. Disorders in hair growth in African Americans. In: Olsen EA, editor. Disorders of hair growth. New York: McGrawHill; 1994. pp. 389-407. 33. Ackerman AB, Walton NW, Jones RE, et al. ‘‘Hot comb alopecia:/’’ Follicular degeneration syndrome’’ in African-American women: is traction alopecia! Dermatopathol Pract Concept 2000;6-21. 34. Bulengo-Ransby SM. Chemical and traumatic alopecia from thioglycolate in a black woman. Cutis 1992;49:99-103. 35. Scott DA. Disorders of the hair and scalp in blacks. Dermatol Clin 1988;6:387-95. 36. Price VH. The medical treatment of cicatricial alopecia. Semin Cutan Med Surg 2006;25:56-9.