Keywords

Introduction

“People of color” refers to a large group of people with pigmented skin from several different racial and ethnic origins, including but not limited to Africans, African Americans, African Caribbeans, Chinese, Japanese, Navajo Indians, Indians, Pakistanis, and Arabs. Already comprising a sizeable section of the American population, this group is predicted to grow dramatically over the next several decades, with patients of color comprising near 50% of the US population by the year 5050. These demographic changes emphasize the importance of understanding the cultural practices and dermatologic needs of this population [1]. One important area in which dermatologist expertise will be invaluable is hair loss. Unlike Caucasians and Asians, epidemiological data in African American women reveals alopecia among the group’s top ten dermatologic conditions [2].

Hair is central to identity and appearance and may play a role in thermoregulation and photoprotection. Thus, it is no surprise that hair loss can have a detrimental psychological impact. The effect of alopecia extends beyond outward appearance; a majority of women in one study reported a change in their daily activities and loss of self-confidence due to their hair loss [3]. Studies in women with androgenetic alopecia demonstrate a reduced quality of life and reduced self-esteem at both the time of hair loss and for extended periods thereafter. Regardless of ethnicity or the underlying pathophysiology of hair loss, alopecia is likely to negatively impact a patient and encourage them to seek dermatologic help.

With a higher rate of alopecia in women of color (WOC) and the negative psychosocial impact of hair loss, it is imperative that all dermatologists are up to date on how to prevent, counsel, and treat hair-related concerns effectively in this population. This chapter will explain the structural qualities of hair in WOC and how it differs from the hair of Caucasians, including the follicle and shaft shape, the density of hair, and the intra-shaft interactions of the hair, all of which bear importance on the overall quality and strength of the hair. We will also discuss hair practices in WOC and how these practices may increase risk of certain types of hair loss. Finally, we will focus on the presentation, pathophysiology, and management of the types of alopecia that present uniquely in WOC.

Structural Differences of Hair Between Patients of Color and Caucasian Patients

Hair of different ethnicities can be separated into three groups based on differences in shaft and follicular architecture: African, Caucasian, and Asian [4]. The hair shaft is composed of a cortex surrounded by several layers of cuticular cells [5]. The chemical structure of the shaft itself, specifically the keratin and the amino acid configurations, is similar across ethnicities [2, 4]. However, Black hair reserves some distinctions. It is more densely pigmented than Caucasian hair [1], and unlike Caucasian hair, it contains melanosomes in the outer root sheath and in the bulb of vellus hairs [1]. The size of the melanin granules in Black hair is also larger than in that of individuals with a fair complexion [1, 6].

There are four major types of hair patterns: helical, spiral, straight, and wavy [1]. The majority of Africans have spiral hair, a quality that makes the hair more difficult to comb [2]. Unlike in Asians or Caucasians, the African hair follicle is sharply curved, contributing to the frizzy, curly appearance of the hair. It also results in the growing hair emerging from the skin obliquely, leading to higher rates of conditions such as pseudofolliculitis barbae [4]. Under magnification, African hair is described as an oval-twisted rod with many random twists and irregular direction changes; in cross section, the shaft has a flattened, elliptical morphology [1, 2]. The diameter of the hair shaft is highly irregular [2], reducing gradually as the hair descends from the scalp. Additionally, there are fewer sebaceous glands in the scalp of individuals of African descent, compared with other ethnicities, resulting in drier, more brittle hair.

Caucasian hair, in contrast, is of a more straight or gently curved shape [5] with an oval cross section and oval follicle [5]. It has a larger diameter than that of African hair [2]. Studies have shown that Caucasian hair grows faster than African hair (0.330 vs 0.259 mm/day, respectively) [7]. However, there is marked similarity in the cuticle thickness, shape and size of scale, and cortical cells between African and Caucasian hair [1].

Structural studies of African hair compared to Caucasian hair have revealed a number of differences. For example, African hair may display microscopic signs of structural damage such as longitudinal fissures leading to splits along the shaft, which ultimately cause more frequent knotting of African hair. A majority of African hairs removed by combing were found to be fractured at the top rather than containing an attached root, indicating breakage of the shaft rather than natural shedding of intact hair during grooming. Additionally, most of the tips of the African hair studied were frayed or serrated, rather than appearing smooth or cut (as during a haircut) [8]. Examination of the in situ hair relationships in African and Caucasian women demonstrated greater intertwining of hairs in African hair, developing into a mat-like structure of hair, whereas Caucasian hair formed fewer single-strand knots and had fewer interlocking knots and weaves between adjacent hairs. These differences may explain why less sebum is able to coat African hair, resulting in drier, less shiny hair with lesser tensile strength [9]. They also explain why there is increased breakage with combing of African hair, making long hair difficult to achieve [7, 10].

Hair density and the total number of hair follicles are notably lower in African than Caucasian hair [1]. Some authors have also observed fewer elastic fibers attaching the hair follicles to the dermis in the Black subjects compared with White subjects [1, 11]. This differential may provide an explanation for certain types of alopecia, such as traction alopecia and central centrifugal cicatricial alopecia [1], which occur more commonly in African than Caucasian women [2, 9]. In addition, African hair has been shown to contain a fewer lipids than Caucasian hair, rendering it more vulnerable to damage by UV radiation [12]. Integral lipids in the hair cuticle offer a degree of protection from UV damage by imparting hydrophobicity, moisturization, and stiffness to hair that reinforces the structural integrity of the strands. These findings, along with reduced tensile strength and less moisture compared to Caucasian hair, explain why African hair is more easily susceptible to breakage [2, 12]. Overall, however, the composition of lipids in the hair is similar across racial type [2].

Hair Care Practices in Women of African Descent

Hair care practices are influenced by current trends as well as ease of style and maintenance [3]. There are several culture-specific hair practices common in women of African descent that induce hair damage either by introducing excess tension at the root of the hair follicles or by direct insult to the hair shaft, potentially contributing to alopecia in women of African descent.

Certain hairstyles subject the hair root to excess tension, including cornrows, weaves, dreadlocks, sisterlocks, braids, and twists. The prolonged periods of traction increase the risk of traction folliculitis and traction alopecia [9, 13]. Cornrows is a hairstyle that involves splitting hair into sections and then braiding it to produce rows of braids (Fig. 11.1). Cornrows and braids are low-maintenance hairstyles that enable less frequent use of chemical or thermal hair treatment and allow the hair a period to “rest” and “grow out” after being chemically relaxed (Fig. 11.2) [13]. A hair weave is a style that sews or glues additional human or synthetic hair to the base of the cornrowed hair, with the added hair worn loose (Fig. 11.3) [9]. Hair wefting is another type of hair extension in which a group of hairs brought together in a band of threads is thereby clipped, glued, or sewn to the hairline for extended periods of time [14]. Dreadlocks, or “locs,” is a hairstyle that involves the hair knotting into individual twist-like structures (Fig. 11.4, left) [9]. This is a permanent style that remains until the hair is cut [13]. The dreadlocks are styled by twisting or “palm-rolling” the root with a balm or wax to lock the hair together [9, 13]. Sisterlocks are created in a similar fashion but with much thinner and smaller locs (Fig. 11.4, right) [13]. While the loc style avoids the use of chemical or heat processing, it induces tension at the roots, especially if the sections are small, the locs over-twisted at the root, or if grown very long [9].

Fig. 11.1
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Cornrow hairstyle. (Reproduced with permission from Ref. [15])

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Fig. 11.2
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(Was 4) Tight braids inducing folliculitis and early traction alopecia. (Reproduced with permission from Ref. [15])

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Fig. 11.3
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(Was 2) Sew-in weave hairstyle. (Reproduced with permission from Dr. Yolanda Lenzy [13])

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Fig. 11.4
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(Was 3) Left: Traditional locs. Right: sisterlocks. (Reproduced with permission from Dr. Yolanda Lenzy [13])

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Straight hair, a sought-after style in this group due to its increased manageability, is achieved either by thermal or chemical means [2, 9]. Thermal techniques, such as hot combing or pressing, introduce heat to the shaft leading to short-term rearrangement of hydrogen bonds within hair strands. Hot combing is a technique introduced in the early twentieth century that has become less popular since the advent of chemical straighteners [3]. After washing and drying, an oil or pomade is first applied throughout the hair, and then a hot metal comb heated to 300 to 500 °F is pulled through the length of the hair. The thermal straightening effect is temporary, lasting until the hair is washed [3]. This method is associated with damage to the hair shaft as well as scalp burns, potentially causing acquired trichorrhexis nodosa [3]. The use of hot combing plus braiding is a common hairstyle initiated as early as childhood or adolescence [1].

Hair straightening is also achieved with the use of a flat iron, which consists of two flat ceramic plates heated to 180–450 °F pressed along the length of the hair [2]. Despite temperatures nearly as high as hot combing, flat irons may result in less damage due to even heating and better temperature control [9]. Furthermore, as opposed to oils and pomades used with hot combs, silicone heat-protectant lubricators are often used with flat irons to protect the hair from damage [9]. In addition to these two methods, women also use curling irons, hair rollers, and hair dyes, all of which may contribute to hair damage [1].

Chemical straightening, a method used by 90% of African American women at some point, is much more commonly used than hot combing. Unlike the temporary effect of thermal straightening, chemical straightening rearranges disulfide bonds within the shaft to produce permanently straight hair. Chemical relaxers are highly alkaline substances containing sodium hydroxide or guanidine hydroxide, the latter used in no-lye formulas [9, 16]. This method, often performed up to 6–12 times per year, is seen as an easier way to reduce the curliness of the hair [3]. However, it is not without risks, as it frequently leads to frail, damage-susceptible hair, irritant contact dermatitis, and acquired trichorrhexis nodosa, especially with improper use [1]. The resultant hair fragility may be due to a lower cysteine content in relaxed hair, a component of the disulfide bond that is essential for hair strength [9]. Use with concurrent permanent hair dye can induce breakage [17]. The Brazilian keratin treatment, another method of chemical straightening lasting about 2–2.5 months, involves covering the hair with a liquid keratin and formaldehyde preparation and sealing the solution to the hair with a flat iron. Formaldehyde exposure during the use of Brazilian keratin is the main health risk to both the client and the hair stylist, and the heat from the flat iron is also damaging the hair [2, 4].

African American women typically shampoo once every 1–2 weeks, compared with the every-other-day or once-weekly washes in Caucasian women [7]. In addition to naturally low hair sebum levels, necessitating less frequent washing, shampooing often may contribute to dry hair and risk for breakage. Less frequent shampooing also allows longer preservation of the time-consuming and expensive hairstyles [2]. This is of importance to dermatologists, as treatments involving shampooing or wetting the hair may not be adopted easily in this group [4]. Furthermore, infrequent shampooing allows for product buildup, which contributes to hair shaft dryness and reduced tensile strength. Product buildup may also lead to or worsen scalp conditions such as seborrheic dermatitis or cause irritant contact dermatitis [4, 7]. The use of emollients may provide a false impression of healthy hair and also result in less frequent shampooing [3].

Alopecia

Alopecia is a condition that has a wide range of etiologic subtypes, certain forms of which are far more common in women of color. Alopecia can be categorized into two major groups, non-scarring or scarring (also referred to as “cicatricial”). Non-scarring alopecias of special focus to the WOC population include traction alopecia, trichorrhexis nodosa, and seborrheic dermatitis, the latter of which can be associated with hair loss. It is important to be familiar with non-scarring forms of alopecia as prompt diagnosis can have a profound impact on the clinical course and total eventual hair loss associated with these diseases. Examples of cicatricial alopecias include central centrifugal cicatricial alopecia, discoid lupus erythematosus, lichen planopilaris, and frontal fibrosing alopecia. Generally, management of these diseases focuses on symptom relief and halting the progression of hair loss. Unfortunately, the underlying cause and strategies for management of many alopecias remains to be studied and delineated with greater confidence [18].

Traction Alopecia

Traction alopecia (TA) is a pattern of traumatic hair loss highly associated with hair care practices and increased hair follicle vulnerability [14]. It occurs most commonly in females of African ethnicity [2, 6, 19]. The primary insult occurs when tension is applied to the hair for prolonged periods or in a repetitive manner [2]. Regularly styling the hair in a manner that involves tight pulling is the central risk factor to TA. The combination of traction and chemically relaxed or dyed hair significantly increases the risk of TA, although the frequency of chemical use has not been found to affect the risk [2, 6, 19]. Genetics, too, may play a role; TA has been found to occur at higher rates with a family history of androgenetic alopecia [15]. The use of shampoo and other hair products does not appear to increase risk of TA [15].

Histopathology

Histology early on shows a normal density of hair follicles, retained hair sebaceous glands, higher numbers of telogen and catagen hairs, and trichomalacia. Over time, the number of terminal follicles will be fewer, although vellus hairs appear unaffected. Loss of hair follicles is accompanied by concentric fibrosis [14]. Overall, there is little inflammation in TA. Biopsy of transverse sections of scalp showing a low-power pattern of miniaturization and follicular dropout with retained sebaceous glands should point toward a diagnosis of TA [15]. This method is preferable over vertical sections in differentiating TA from scarring alopecias such as cicatricial alopecia. TA is the only type of alopecia that has a reversible early stage. Likewise, it is the only type of hair loss that is preventable with the potential for eradication [15]. A proposed staging system delineates TA into a reversible “prealopecia stage,” a reversible stage with alopecia, and a stage with permanent, irreversible alopecia [14].

Etiology

The patient history may point to the use of tight hairstyles that put tension on the hair root or hair treatments that increase the vulnerability to traction-related damage. The physician should evaluate for a history of tight ponytails, buns, chignons, braids, twists, weaves, cornrows, dreadlocks, sisterlocks, and hair wefts in addition to the usage of religious hair coverings [2, 14]. Wefting has recently been shown to cause TA in some cases [14]. Some patients with TA report symptoms with hairdressing, which may include scalp tenderness, stinging, crusting, and follicular papules [15]. Finally, a history of treatment with hair dyes or chemical relaxers may be readily apparent or elicited upon questioning the patient.

Clinical Features

Physical exam findings are dependent on the clinical stage of TA. At the earliest stage, scalp traction results in perifollicular erythema, which may evolve into follicular papules or pustules. These findings are most prominent in the area with the greatest traction. However, the patient may not be aware of these lesions as they can be missed without close inspection [15]. As the traction continues, affected hair shafts become enveloped by a yellow-white material called a peripilar cast (Fig. 11.5) [2, 20, 21]. Peripilar casts on the hair shaft indicate that TA is ongoing or persistent [15]. Beyond this point, clinically evident hair loss develops. In the late stages of TA, dermal scarring and permanent alopecia develop. The scalp at this stage demonstrates a reduction of follicular ostia in affected areas [2, 20].

Fig. 11.5
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Peripilar casts. (Reproduced with permission from Ref. [21])

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The most common locations subject to traction and the development of TA are the marginal hairline (along the frontal and temporal scalp) and anterior and superior to the ears [2, 14]. The “fringe sign,” which can aid in the diagnosis of TA, refers to hair loss at the marginal hairline with some preserved hairs along the frontal and/or temporal hairline (Fig. 11.6) [2, 20]. Less common variants of TA have been found to affect other areas of the scalp (Fig. 11.7). When due to wefted hair extensions, TA may present with a “horseshoe” pattern (Fig. 11.8) [14]. Reports of TA in Hispanic women with a history of tight ponytail use and ballerinas and Japanese women commonly styling their hair into a tight bun have demonstrated hair loss localized to the temporal or occipital scalp [22, 23]. TA may also appear in linear, curved, or geometric shapes [15].

Fig. 11.6
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Fringe sign. (Reproduced with permission from Ref. [15])

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Fig. 11.7
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Sisterlocks on a scalp affected by traction alopecia. (Image courtesy of Oma Agbai, MD)

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Fig. 11.8
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Horseshoe traction alopecia. Alopecia of occipital and temporoparietal scalp corresponds to area where patient used glued-in hair wefts. (Reproduced with permission from Ref. [14])

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Differential Diagnosis

Not all TA patients present with a clear-cut history of high tension hairstyles. In the case of unclear history of hair tension or unclear clinical presentation, other diagnoses to consider include androgenetic alopecia, telogen effluvium, trichotillomania, and central centrifugal cicatricial alopecia. Long-term or repetitive hair wefting may induce TA that appears similar to scarring alopecia [14]. Consider frontal fibrosing alopecia (FFA) and alopecia areata (AA) ophiasis pattern in cases confined to the marginal hairline [24]. Unlike TA, AA and FAA may also involve the eyebrows, body hair, skin, and nails. Follicular markings, which are decreased in TA, remain present in AA and are absent in FFA. Accurate diagnosis requires biopsy [15]. See Table 11.1 for a comparison of the clinical features of TA, AA, and FAA.

Table 11.1 A comparison of clinical features of traction alopecia, alopecia areata, and frontal fibrosing alopecia
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Management

Management of TA is also dependent on the stage of the disease. A proposed prevention strategy involves notifying at-risk patients to limit tension hairstyles to a short time duration (maximum 2 weeks) infrequent use, created in a painless manner and on natural hair [15]. In early stages, TA is reversible; thus, the clinician plays an important role in counseling the patient of the risk of progressive, permanent hair loss [25]. Once TA is noted, the patient should avoid chemical relaxers, dyes, and heat and lessen or avoid tension in their hairstyles. Early intervention includes suppression of follicular inflammation with topical and/or intralesional corticosteroids [26]. This may be combined with topical minoxidil [2, 19]. Oral or topical antibiotics can be beneficial at this stage given their anti-inflammatory properties [26]. Once there is scarring of the dermis or permanent alopecia, treatment necessitates surgical interventions such as hair transplantation [26, 27].

Acquired Trichorrhexis Nodosa

Trichorrhexis nodosa (TN) is a form of alopecia resulting from the loss of cuticle cells on the hair shaft. The congenital form of TN is rare, while acquired TN is much more common and caused by trauma to the hair during grooming practices [2, 28]. Without the protective cuticle, the cortical fibers of the hair shaft become fragile and fray longitudinally [2].

Etiology

Causes of acquired TN include a variety of hair care practices that induce trauma to the hair. Repeated chemical treatment, such as the use of relaxers, dyes, bleaches, perms, or shampoos have been implicated in TN. Relaxers have been found to directly weaken the hair cuticle and compromise the hair shaft itself by reducing the cysteine levels [2, 19]. Thermal hair tools including flat irons and hot combs damage the cuticle leading to increased fragility and breakage. Aggressive hair brushing or combing also introduces damage to the cuticle and contribute to TN. Medical conditions such as iron deficiency anemia and hypothyroidism may also lead to TN [2, 28].

Clinical Features

Hair affected by TN is characterized by a dry, lusterless, brittle quality with whitish spots along the shaft [2, 28]. TN is most often localized to the distal shaft, with focal narrowing observed along the length of the shaft. A normal hair cuticle contains cells organized in a neat fashion similar to roof tiles. Under magnification, cuticle cells affected by TN appear disrupted, and the underlying cortical fibers split into many small fibers [2, 28].

Management

Minimization or cessation of traumatizing processes is the priority in managing TN. The use of hot combs and flat irons should be limited to once weekly or less, temperatures under 175 °C, and use on dry hair [2, 29]. Frequency of chemical relaxers and dye use should be minimized, and these products should be applied by a licensed hair stylist. The combination of dyeing and chemically relaxing hair should be avoided if possible as it leads to a notably increased risk of hair breakage. In routine hair care, efforts should be made to reduce friction by the use of conditioners, hair oils, and combs with straight elongated bristles [2].

Central Centrifugal Cicatricial Alopecia

Central centrifugal cicatricial alopecia (CCCA) is a form of scarring alopecia affecting the crown and vertex of the head. It is most often seen in women of African ethnicity, with many fewer cases observed in men of African descent. Known historically by names such as “hot comb alopecia” and “follicular degeneration syndrome,” CCCA is the most common cause of permanent hair loss in this ethnic group [9, 30]. The disease most often presents in middle age [30]. It is characterized by hair loss originating at the vertex of the scalp that proceeds centrifugally, often in a symmetric manner [30].

Etiology

The cause of CCCA is not known. Although it was initially believed to be caused by trauma from hot comb use, subsequent studies have failed to demonstrate a causal effect between hot comb use and CCCA [9]. Studies have also failed to find correlation between chemical relaxer use and CCCA but have yielded mixed results with regard to traction-inducing hairstyles [9, 19, 30,31,32]. One theory suggests that CCCA originates as female pattern hair loss (FPHL), given the pattern I and pattern II distribution of hair loss, which is subsequently negatively influenced by hair grooming practices. Although CCCA and FPHL may appear similar, in late stages CCCA distinguishes itself by scarring and follicular dropout [30]. Other investigatory findings point toward a potential hereditary component, either due to a genetic predisposition or shared exposures, although more evidence is warranted in this area. Interestingly, studies show that scalp sebum maintains a pro-inflammatory state even in unaffected hair, with a greater amount of IL-1 alpha than IL-1 antagonist. It is theorized that after an initial event of premature desquamation of the inner root sheath (PDIRS), the inner follicle is more susceptible to invasion by cosmetics and microorganisms, with eventual inflammation and scarring [9].

Histopathology

CCCA is characterized by chronic perifollicular lymphocytic inflammation leading to eventual scarring of the scalp. Early in the course of the disease, hair follicles are surrounded by a lymphocytic infiltrate and perifollicular fibroplasia. PDIRS is a central histologic finding in CCCA, although it can also be found in other types of primary scarring alopecia. There is also a reduction in the number of terminal hair follicles with a concurrent increase in fibrous tracts [9]. Early biopsies may be interpreted as “lichen planopilaris” or “pseudopelade,” given the scarring and follicular destruction [30]. An active phase in which the affected area increases in size may last years but ultimately ceases naturally [30]. Late in the disease, pathological findings include obliteration of pilosebaceous units, dermal scarring, and dermal lymphocytic and plasma cell infiltrate. Biopsy of end-stage CCCA will demonstrate fibrosis with little remaining inflammation [30]. Although these findings are useful in the diagnosis of CCCA, they are not unique to this disease and cannot distinguish it from other primary scarring alopecias such as lichen planopilaris, discoid lupus erythematosus, and folliculitis decalvans [9, 30]. Therefore, every effort should be made to evaluate the patient early in the disease course and to correlate the histopathologic findings with the patient’s history and clinical presentation [30].

Clinical Features

Patients with CCCA present with hair loss originating on the crown or vertex of the head, spreading outward in a centrifugal pattern (Fig. 11.9) [9, 30]. The patient may describe burning, itching, or tenderness in the affected area. However, not all patients experience symptoms and thus often present late after slow, insidious hair loss is noticed by others [31]. Early stages may demonstrate erythema or follicular pustules; late disease lacks observable inflammation. As the disease progresses, the number of visible follicles decreases, and the scalp appears smooth and shiny. Some individual strands may remain [9]. For a comparison of dermoscopic features of CCCA, lichen planopilaris, and frontal fibrosing alopecia, see Table 11.2.

Fig. 11.9
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Central centrifugal cicatricial alopecia. (Image courtesy of Oma Agbai, MD)

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Table 11.2 Dermoscopic features of cicatricial alopecias
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Differential Diagnosis

Lichen planopilaris (LPP) can occur anywhere on the scalp but may look similar to CCCA especially in cases that begin on the vertex with centrifugal spread. Unlike CCCA, affected areas of LPP originate as hyperkeratotic follicular papules with perifollicular erythema. The lesions progress radially, ultimately resulting in dappled areas of scarred hair loss surrounded by unaffected hair. Frontal fibrosing alopecia (FFA) targets the eyebrows and frontotemporal hairline. It presents later than CCCA, most often in postmenopausal women in their 60s or 70s. It is slowly progressive and may also spread down the inferior temporoparietal hairline (“ophiasis-like pattern”). In some patients it may occur with nearby LPP or discoid lupus erythematosus [30, 33]. Folliculitis decalvans is characterized by patches of scarring alopecia surrounded by pustules. The hair loss begins in distinct round or oval areas but may merge into a large central area, mimicking CCCA. Lastly, discoid lupus erythematosus typically affects young to middle-aged females. While it characterized affects the sun-exposed face, ears, trunk, and limbs, the scalp may be involved. Some patients present with only scalp lesions, which can range from papules to sclerotic plaques. Affected areas are well circumscribed, erythematous, scaling, and pruritic. They may also display atrophy, telangiectasia, dyspigmentation, and follicular plugging. Key in distinguishing this process from CCCA is its tendency to recur within the confines of a previous patch [30].

Management

There is no established definitive treatment of CCCA. Therapy should be aimed at symptom relief and halting the progression of the disease [26]. Hair regrowth, if achieved, should be viewed as an extra benefit, though not assured. Patients should be advised to decrease or avoid trauma to the scalp during hair grooming. This includes decreasing the use of thermal hair appliances and loosening tight braids and weaves. Intervals between reapplication of relaxers should be increased as much as possible (at least every 8–12 weeks). Some sources recommend temporary cessation of heat or chemical processing and the avoidance of occlusive scalp greases to allow the scalp to heal [26, 30].

Medical management involves daily topical corticosteroids until symptom remission, with subsequent maintenance at 3 days per week. Intralesional steroids aimed at the periphery of the hair loss (including unaffected areas for prevention) can be administered at a dose range of 2.5–10 mg/mL every 4–8 weeks, for 6 months or longer [26, 31, 34]. Topical minoxidil should be tried for the potential of avoiding future scarring and nurturing recovering follicles; once inflammation has cleared, topical 2% or 5% solution or 5% foam can be used [30]. The use of oral tetracyclines and antimalarial medications for their anti-inflammatory properties has been reported with variable success [9]. Prior to initiating antibiotics, any pustules should be cultured to evaluate for a fungal infection or resistant Staphylococcus. In late-stage disease, surgical intervention may be considered. Hair transplantation should only be initiated once inflammatory infiltrate has cleared and hair loss has not progressed for at least 12 months [30]. While keloids are not as common a complication as previously thought, the scarring of the scalp due to CCCA renders transplantation more complicated and decreases the survival of the hair grafts [26].

Lichen Planopilaris

Lichen planopilaris (LPP) is an uncommon primary scarring alopecia of unknown etiology. It is considered a follicular variant of lichen planus [35]. Chronic lymphocytic inflammation in LPP targets the upper third of the hair follicle, with eventual destruction of the follicular stem cells and permanent hair loss [36]. Of new patients presenting with hair loss, LPP is estimated to account for 1.15–7.59% of cases [37]. It occurs more often in women and commonly presents during 30 and 60 years of age [35, 37]. Frontal fibrosing alopecia (FFA) and Graham-Little-Piccardi-Lassueur syndrome (GLPLS) are clinical variants of LPP. FFA , a scarring of alopecia presenting as a receding band the frontotemporal hairline, will be discussed in the following section. GLPLS is recognized by the combination of scarring patches of scalp alopecia, spinous follicular papules on the limbs and trunk, and a non-scarring alopecia in the axillary and pubic areas [35].

Histopathology

Biopsy demonstrates perivascular infiltrate with a lichenoid dermatitis enveloping the follicular infundibulum and isthmus [30]. Early lesions feature destruction of the sebaceous epithelium and arrector pili muscles; as the disease progresses, follicles may fuse, and the inner sheath may degenerate. In late disease, inflammation is less prominent, and perifollicular hyalinization and lamellar fibrosis are notable in the upper and lower dermis and follicle [35, 38]. With the aid of dermoscopy, perifollicular scaling can be visible on the proximal hair shaft of active lesions [35]. Lesions later in the course may be characterized by fibrotic white dots, absence of follicular ostia, pili torti, and honeycomb hyperpigmentation [35].

Clinical Presentation

Lichen planopilaris may manifest as multifocal patches or diffuse areas of hair loss throughout the scalp. The lesional margins spread outward creating smooth, pale, atrophic polygonal alopecic areas (Figs. 11.10 and 11.11). Follicular markings are absent. The hairs at the margins display perifollicular erythema and scaling [30, 36, 38]. Lichen planus on the body may occur prior to, during, or after LPP affects the scalp. The trunk and limbs may also display follicular hyperkeratosis. Symptoms are variable and can include moderate to severe discomfort, pruritus, pain, or burning. Active disease can be determined with a positive pull test for anagen hairs. LPP tends to progress slowly, often leaving enough hair for styling to conceal the alopecic areas [38].

Fig. 11.10
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Lichen planopilaris in a mature African American woman. (Image courtesy of Oma Agbai, MD)

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Fig. 11.11
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Overlapping lichen planopilaris and frontal fibrosing alopecia in a mature Asian woman. (Image courtesy of Oma Agbai, MD)

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Differential Diagnosis

LPP can resemble alopecia areata, particularly in the early stages of disease. The differential diagnosis for LPP also includes discoid lupus, CCCA, and folliculitis decalvans, necessitating a close look at clinical, dermoscopic, and histologic findings [35]. Histologically, subepidermal lymphocytic infiltrate in LPP surrounds the isthmus and infundibulum (the upper portion) of the hair follicle but does not penetrate the deeper follicle as seen in alopecia areata [35]. Common dermoscopic features of alopecia areata include dystrophic hairs, yellow dots, cadaverized hairs (appearing as black dots), and exclamation mark hairs [39]. LPP is more commonly characterized by perifollicular scales [35].

Management

Current treatment modalities are aimed at slowing the disease progression and symptom relief [38]. Treatment includes high-potency topical steroids such as clobetasol propionate. However, topical therapy with corticosteroids or immunomodulators such as pimecrolimus and tacrolimus are often ineffective as monotherapy [36]. Oral immunosuppression is often required, with hydroxychloroquine as a common choice [30, 36]. Areas of active disease can be treated with intralesional corticosteroids such as triamcinolone acetonide suspension 10 mg/ml. Oral corticosteroids, azathioprine, antimalarial medications, dapsone, isotretinoin, and cyclosporine may also be used [30, 38]. Recent literature suggests that mycophenolate mofetil is safe and effective in the treatment of recalcitrant LPP [36].

Frontal Fibrosing Alopecia

Frontal fibrosing alopecia (FFA), a clinical variant of LPP, is a primary scarring alopecia associated with progressive regression of the frontotemporal hairline [2]. Despite the differing overarching hair loss patterns of alopecia, FFA and LPP are similar in histology and certain clinical features. FFA most often affects women of postmenopausal age and occurs more commonly in Caucasian and Asian women, although it has been reported in women of African descent [2, 40]. It can also occur in men and premenopausal women [2].

Etiology

Although FFA appears to be increasingly common, little is known about the etiology or pathology of this condition [41]. Given the predilection for postmenopausal women and recent studies demonstrating benefit from antiandrogenic therapy, it is theorized that FFA may be due to a hormonal cause [41]. Other findings point toward a potential autoimmune etiology, as FFA has been associated with other autoimmune disorders such as vitiligo and thyroid dysfunction. A high prevalence of hypothyroidism in some studies has prompted the suggestion to include thyroid studies in the workup of FFA [42, 43]. The paucity of data and low apparent prevalence of FFA in African Americans may be due to misdiagnosis as traction alopecia, a common cause of hair loss in this group [2, 40]. Given significant overlapping clinical histories and pathologic features between FFA and TA, making the correct diagnosis can be challenging [40].

Histopathology

Scalp biopsy in FFA appears histologically identical to that of lichen planopilaris, necessitating clinicopathologic correlation [41]. A lymphocytic infiltrate surrounds the infundibulum and isthmus of the hair follicle. Long-term inflammation ultimately leads to follicular destruction and fibrosis [23].

Clinical Presentation

FFA is characterized by a band-like receding frontotemporal hairline and that may also involve hair loss of the eyebrows, eyelashes, axillary region, pubic region, limb, scalp, and vellus hairs (Figs. 11.11 and 11.12). Eyebrow loss, which can be the presenting sign, is another central feature and occurs in up to 75% of patients [2, 41, 44, 45]. The patient may initially develop pruritus, noninflammatory perifollicular papules, and red follicular spots on the forehead [2, 41, 43, 45]. Perifollicular erythema, follicular keratotic plugs, and follicular dropout visible on dermoscopy are indicative of active disease [2, 46]. Follicular inflammation is associated with and eventually replaced by scarring with a loss of follicular markings [23, 47]. The presence of one or a few terminal hairs on the forehead despite the regression of the marginal hairline, the “lonely hair sign,” is another clue pointing to a diagnosis of FFA [48]. Clinical course is variable but commonly progresses slowly until self-stabilization [43, 45, 47].

Fig. 11.12
figure 12

Frontal fibrosing alopecia. (Image courtesy of Oma Agbai, MD)

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Differential Diagnosis

The most important confounding diagnosis in FFA is traction alopecia [40], which is a common cause of alopecia in this group. Please refer to Table 11.1 for a comparison of clinical features of FFA and TA. Lichen planopilaris is similar to FFA, although the pattern of hair loss is distinct. Eyebrow loss, axillary hair loss, and follicular keratotic papules are features common in both conditions. Histologic appearance of the two conditions is equivalent, precluding this technique as a method of differentiating the two conditions [47]. FFA may also be difficult to distinguish from the ophiasis pattern of alopecia areata, with both conditions involving band-like hair loss in the fronto-parieto-temporal regions. Both may affect the eyebrows, although this occurs most consistently in FFA. Close clinical examination of FFA will demonstrate scarring with follicular loss, perifollicular erythema, and follicular keratotic plugs. In cases where biopsy is required, the ophiasis pattern of alopecia areata exhibits peribulbar lymphocytic infiltration, follicular miniaturization, and telogen arrest; the ophiasis pattern specifically may show fewer hair follicles and replacement by fibrosis. Histology of FFA more reliably exhibits fibrosis and scaring with a lichenoid interface infiltrate around the upper follicle in active lesions [49].

Lastly, cutaneous sarcoidosis not only may mimic alopecia but has been known to stimulate scarring alopecia. Sarcoidosis-induced alopecia has been reported most commonly in African American women between 23 and 78 years of age and most often presents simultaneously with facial, pulmonary, and lymph node involvement [50]. When occurring on the scalp, cutaneous sarcoidosis usually presents as localized with indurated papules, plaques, and erythema with scale [50]. These lesions may resemble not only FFA but also CCCA, discoid lupus, and lichen planopilaris, making biopsy essential for diagnosis. On biopsy, FFA will demonstrate granulomas surrounding the hair follicle, while sarcoidosis demonstrates noncaseating granulomas present in the dermis with or without follicles [51]. Evaluation for systemic sarcoidosis is indicated in patients with cutaneous sarcoidosis-induced alopecia [50].

Management

Efforts to establish effective therapy for FFA have been elusive, and there is currently no gold standard treatment [2, 41]. The current goal of management is to prevent further hair loss. Without any intervention, the marginal hairline recedes at a rate of 0.95–1.08 cm/year [2, 40, 46] and tends to self-stabilize [43, 45, 47]. Varying success has been reported with the use of topical and intralesional corticosteroids, topical calcineurin inhibitors, hydroxychloroquine, mycophenolate mofetil, and oral 5-alpha-reductase inhibitors [2, 44, 45, 47]. Some authors suggest a trial of triamcinolone acetonide 20 mg/ml in patients with evidence of inflammatory activity [47]. Recent evidence suggests that oral finasteride and dutasteride may be a beneficial maintenance therapy after the use of intralesional corticosteroids has cleared any perifollicular erythema or follicular hyperkeratosis [2, 41, 43]. Furthermore, PPAR-gamma agonists may be a promising treatment option for frontal fibrosing alopecia and lichen planopilaris.

Discoid Lupus Erythematosus

Discoid lupus erythematosus (DLE) is a clinical variant of cutaneous lupus erythematosus (CLE) and is one of the most prevalent causes of scarring alopecia [52]. In those presenting solely with discoid lesions, an estimated 17–30% will ultimately show signs of systemic lupus erythematosus (SLE). 8–28% of patients with diagnosed SLE exhibit discoid lesions. Discoid lesions limited to the head indicate a lower likelihood of future SLE (5%) compared with discoid lesions below the neck (20%) [53]. DLE generally affects the face, ears, neck, and scalp, with over half of patients presenting with scalp lesions [52]. DLE occurs more commonly in both people of color and females [54, 55]. Importantly, quality of life studies in CLE have demonstrated poorer outcomes in females, patients of color, and those of low socioeconomic status [55].

Histopathology

Histopathology shows vacuolization of the basal epidermis and increased dermal mucin. Direct immunofluorescence shows granular deposition of IgG and C3 along the follicular epithelium and dermoepidermal junction [56]. Lesions also demonstrate basement membrane thickening [57].

Clinical Features

Lesions typically appear as erythematous plaques with follicular plugging, scale, central hypopigmentation, and epidermal atrophy [52, 56]. One author has observed two African American patients with DLE presenting hyperpigmented scaly plaques of the scalp (Agbai, unpublished clinical observation). A classic sign on trichoscopy is follicular red dots, but branching vessels, large yellow dots, and speckled brown discoloration may also be seen [56]. Patients may be asymptomatic or experience tenderness or pruritus in affected areas [56]. Late-stage lesions eventually develop into atrophic, fibrotic areas with absent follicular markings and central hypopigmentation in the late stages (Fig. 11.13) [52]. Without treatment this condition leads to irreversible alopecia.

Fig. 11.13
figure 13

Hyperpigmented papules within an alopecic patch in a middle-aged African American woman with discoid lupus erythematosus. (Image courtesy of Oma Agbai, MD)

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Differential Diagnosis

Differentiating alopecia caused by DLE from that of LPP can be challenging, as the two share several clinical and histological characteristics. The presence of follicular plugging and central hypopigmentation in alopecic areas is suggestive of DLE, whereas perifollicular erythema and scale favor a diagnosis of LPP. Interface dermatitis on histology is more telling of LPP as opposed to DLE. Perivascular inflammation is more suggestive of LPP than DLE unless it is deep and dense. Excess mucin in LPP is located perifollicularly, while in DLE it is in the dermis. Thickening of the basement membrane in LPP is minimal but prominent in DLE [57].

Management

Early treatment is essential as scarring of the hair follicles leads to permanent alopecia [56]. Smoking cessation and the use of sunscreen may exert a protective and preventive effect against the formation of new lesions [56]. Management of early DLE includes the use of potent topical, intralesional, and oral corticosteroids. Oral antimalarials and topical calcineurin inhibitors are also effective [52, 56]; calcineurin inhibitors are particularly useful on affected areas showing thinning or atrophy [56]. Mycophenolate mofetil, methotrexate, retinoids, dapsone, and thalidomide have also been used but lack sufficient data [56].

Seborrheic Dermatitis

Seborrheic dermatitis (SD) is a chronic inflammatory condition of the scalp, face, and upper chest that leads to erythema, flaking, scaling, and pruritus [58]. While not classically associated with hair loss, some evidence shows a connection between the long-term subclinical inflammation in chronic SD and increased telogen shedding. Cicatricial hair loss has also been reported in some patients with SD [59]. Awareness of the relationship between seborrheic dermatitis and hair loss is significant in the care of WOC as seborrheic dermatitis numbers are among the top five dermatologic diagnoses in African American patients. Caucasian and Asian patients, by contrast, experience seborrheic dermatitis much less frequently, with SD being the ninth most common dermatologic diagnosis in Asians and not among the top ten in Caucasians [2, 60]. The general incidence of SD is 1–3% in the postpubertal population, with a higher proportion of cases in men [58].

Histopathology

In patients with SD, the fungus Malassezia is thought to play a pathogenic role in disruption of seborrheic skin [58]. It is also theorized that an inflammatory reaction is stimulated in the SD scalp when local deposits of immunoglobulins attract and activate leukocytes. Chronic, subclinical inflammation near the follicle is believed to interrupt the hair cycle in the short term but cause scarring in the long term [59]. Common features of scalp biopsy in a case report of patients with SD and hair loss included a mild spongiotic dermatitis, focal parakeratosis of hair follicles, parakeratosis in the deep follicular infundibulum, and scattered perivascular and perifollicular lymphocytes. Mild chronic perifolliculitis with fibrosis was also observed. Unlike other cicatricial alopecias, the patients described in this report demonstrated a mild, diffuse scarring with follicular dropout. No lichenoid inflammation, trichomalacia, or any features of androgenetic alopecia were noted [59].

Clinical Features

Seborrheic dermatitis can affect the scalp, face, retroauricular area, and upper chest. In adults with scalp involvement, it appears as areas of mild desquamation or greasy, honey-colored crusts with associated hair loss (Fig. 11.14). A scaly, erythematous margin of SD from the scalp can appear at the frontotemporal hairline, called “corona seborrheica.” Pruritus often also accompanies SD of the scalp [58]. Table 11.3 demonstrates differing pigmentary alterations seen in patients of color and Caucasian patients, as well as differing management modalities in these two populations.

Fig. 11.14
figure 14

Seborrheic dermatitis associated with alopecia. (Image courtesy of Oma Agbai, MD)

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Table 11.3 Pigmentary findings and management considerations in patients of color compared with Caucasian patients with seborrheic dermatitis
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Differential Diagnosis

Patients with tinea capitis, especially children, may present similarly, with scaly patches of hair loss. However, the patches will contain black dots that are short, broken hairs. Diagnosis of tinea can be made with potassium hydroxide microscopic preparation of the hair shaft or scalp scale or fungal culture [58]. Seborrheic dermatitis must also be distinguished from scalp psoriasis, which presents as sharply demarcated erythematous or hyperpigmented plaques with thick micaceous scale.

Management

SD is a chronic condition that requires ongoing management. Goals of treatment include alleviating pruritus, reducing or eliminating visible signs of SD, and long-term maintenance. Topical antifungal and anti-inflammatory therapies are used to target Malassezia excess and scalp inflammation. Common agents also used are coal tar, lithium gluconate or succinate, and phototherapy. If the affected area is large or the lesions are recalcitrant to topical therapy, systemic agents may be used. See Table 11.4 for specific treatment recommendations [58].

Table 11.4 Treatment of seborrheic dermatitis [58]
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Conclusion

Certain forms of alopecia have unique clinical presentations in WOC. Furthermore, the relative incidence of different forms of alopecia in WOC is distinct from those affecting Caucasian patients. A better understanding of the diagnostic and management nuances in this patient population may optimize diagnostic accuracy, treatment adherence, and management outcomes.