Acne/Folliculitis Keloidalis

Naked hair shafts (NHS) are free-floating hair shafts devoid of surrounding epithelium, supporting structures, and/or embedded in inflammation that may result from destruction of hair follicles by scarring processes such as inflammation and fibroplasia. Extensive examination of NHS has not been performed in scalp biopsies of alopecia. We retrospectively evaluated 622 scalp biopsies of alopecia [345 cicatricial alopecias (central centrifugal cicatricial alopecia, lichen planopilaris, discoid lupus erythematosus, acne keloidalis nuchae, and folliculitis decalvans] and 277 non-cicatricial alopecias [alopecia areata, androgenic alopecia, telogen effluvium, and psoriatic alopecia)] for the presence of NHS. NHS occurred in 0.72% (2/277) of non-cicatricial alopecias (1/102 of alopecia areata, 1/150 of androgenic alopecia, 0/17 of telogen effluvium, and 0/8 of psoriatic alopecia) and 20% (72/345) of cicatricial alopecias (27/118 of central centrifugal cicatricial alopecia, 29/109 of lichen planopilaris, 2/75 of discoid lupus erythematosus, 11/16 of acne keloidalis nuchae, and 3/27 of folliculitis decalvans). The presence of NHS was significantly increased in cicatricial alopecias in comparison with non-cicatricial alopecias; P value <0.0001. Among the cicatricial alopecias, 26% (92/345) had mild inflammation and/or fibrosis, of which 9% (9/92) had NHS. There were 73% (253/345) that had moderate to severe inflammation and/or fibrosis, of which 24% (63/253) had NHS, indicating that as the severity of inflammation and fibrosis increases, so does the presence of NHS. NHS rarely occurs in non-cicatricial alopecias. This variation may result from destruction of hair follicles by the inflammatory and scarring processes. The presence of NHS may be a useful adjunctive histopathologic feature in the diagnosis of cicatricial alopecia.

Folliculitis decalvans, first described by Quinquaud in 1888, is the most common form of the neutrophilic primary cicatricial alopecia. Folliculitis decalvans comprise 2.8% of all cases of hair loss and 10.7–11.2% of all cases of cicatricial alopecia. The etiology of the disease remains unclear. It may be a complex combination of a bacterial infection, a hypersensitivity reaction to "superantigens," and a defect in host cell-mediated immunity regulation. Folliculitis decalvans mainly occurs in young and middle-aged adults; there is a male predominance. The most common trichoscopic finding of folliculitis decalvans is tufted hairs. Other trichoscopic features of active folliculitis decalvans are yellowish tubular scaling, follicular pustules, and yellow discharge. The differential diagnosis includes bacterial folliculitis, tinea capitis, dissecting cellulitis, lichen planopilaris, central centrifugal cicatricial alopecia, and erosive pustular dermatosis of the scalp. Folliculitis decalvans require protracted and often repeated treatment. First-line treatment usually consists of tetracyclines.

Central centrifugal cicatricial alopecia (CCCA) is a type of scarring alopecia, which typically starts on the crown and progresses in a centrifugal manner. It predominantly affects women of African descent and often has devastating psychosocial implications. The pathogenesis of CCCA is not fully understood and therapeutic options are limited. It has been proposed that CCCA is a fibroproliferative disorder (FPD) with persistent, low-grade inflammation resulting in end-stage fibrosis. People of African descent are at increased risk of FPDs including uterine leiomyomas (ULs), possibly due to the protective effect of profibrotic alleles against helminthic infections (Dina Y, Okoye GA, Aguh C. Association of uterine leiomyomas with central centrifugal cicatricial alopecia. JAMA Dermatol 2018; 154: 213–14). There are only eight publications to date that explore the potential link between CCCA and ULs. A retrospective cohort study found that Black women with CCCA have nearly five times increased odds of ULs, compared with Black women without CCCA (Dina et al.). Fibroproliferative genes (e.g. platelet-derived growth factor and collagen genes I and III) are upregulated in areas of scalp affected by CCCA, compared with unaffected scalp in the same patients, with an overlap in pathogenic mediators with other FPDs including atherosclerosis and hepatic fibrosis (Aguh C, Dina Y, Talbot CC Jr, Garza L. Fibroproliferative genes are preferentially expressed in central centrifugal cicatricial alopecia. J Am Acad Dermatol 2018; 79: 904–12). These findings may help identify future therapeutic targets to treat CCCA. Current approaches use anti-inflammatory medications to halt disease progression. However, unlike other scarring alopecias, patients with CCCA often have minimal visible scalp inflammation, despite worsening fibrosis and hair loss. Hence, particularly for patients who do not improve with anti-inflammatory treatments, antifibrotic agents may play a therapeutic role in the future, with medications such as losartan and statins demonstrating potential for halting CCCA progression. We conducted a retrospective cohort study among patients with CCCA presenting to our tertiary-centre hair clinic, using Black women with lichen planopilaris (LPP) as a comparator group (n = 20). We found that 80% of Black women with CCCA had reported ULs, compared with 50% of Black women with LPP. In light of this potential association, dermatologists should be encouraged to screen for symptoms of ULs, alongside other FPDs, and counsel patients regarding potential comorbidities associated with CCCA. This may allow for prompt diagnosis of life-altering comorbidities and significantly ameliorate patients’ quality of life.

3 - Diagnosis and treatment of scarring alopecia

Cholesterol and related sterols are known to modulate the physical properties of biological membranes and can affect the activities of membrane-bound protein complexes. Here, we report that an early step in protein translocation across the endoplasmic reticulum (ER) membrane is reversibly inhibited by cholesterol levels significantly lower than those found in the plasma membrane. By UV-induced chemical cross-linking we further show that high cholesterol levels prevent cross-linking between ribosome-nascent chain complexes and components of the Sec61 translocon, but have no effect on cross-linking to the signal recognition particle. The inhibiting effect on translocation is different between different sterols. Our data suggest that the protein translocation machinery may be sensitive to changes in cholesterol levels in the ER membrane.

Skin of color, also known as ethnic skin, is described as skin of individuals of African, Asian, Hispanic, Native-American, Middle Eastern, and Pacific Island backgrounds. Differences in hair morphology, hair grooming, cultural practices, and susceptibility to keloid scarring exist within these populations and have been implicated in hair, scalp, and skin disorders. Acne keloidalis (AK), central centrifugal cicatricial alopecia (CCCA), dissecting cellulitis of the scalp (DCS), pseudofolliculitis barbae (PFB), traction alopecia (TA), and keloids are the most prevalent follicular and scarring disorders in skin of color. They have been associated with disfigurement, permanent hair loss, emotional distress, and decreased quality of life. Hair grooming practices, such as the use of chemical relaxers, heat straightening, and tight braiding and weaving can cause scalp irritation and follicular damage and are linked to the pathogenesis of some of these conditions. Consequently, patient education and behavior modifications are integral to the prevention and management of these disorders. Scarring disorders are also of concern in ethnic populations. Keloid scarring is more prevalent in individuals of African, Asian, and Hispanic descent. The scarring alopecia CCCA is almost exclusively seen in patients of African descent. Therapeutic regimens such as intralesional corticosteroids, surgical excision, and laser therapy can be effective for these follicular and scarring disorders, but carry a risk of dyspigmentation and keloid scarring. Ethnic skin and hair may present unique challenges to the clinician, and knowledge of these differences is essential to providing quality care.

Response to Weinstein’s “Reply of Increased risk of vitamin D deficiency and insufficiency in Black patients with central centrifugal cicatricial alopecia.”

Chapter 17 - Erosive Pustular Dermatosis of the Scalp

Chapter 14 - Folliculitis Decalvans

Central centrifugal cicatricial alopecia (CCCA) is a primary lymphocytic scarring alopecia, which predominantly occurs in middle-aged Black women. The benefits and use of shared decision-making (SDM) models in patients with CCCA have yet to be studied. We conducted a cross-sectional survey to investigate the role of SDM in the management of CCCA. SDM processes were evaluated using the nine-item Shared Decision-Making Questionnaire (SDM-Q-9), the Decisional Regret Scale (DRS) and the Control Preferences Scale (CPS). Between June and August 2023, members of the Scarring Alopecia Foundation were emailed and invited to participate; n = 87/1192 members responded. The CPS responses suggested that most patients (48%, n = 41/87) preferred to share responsibility with their doctor for treatment decisions. The SDM-Q-9 (scored 0-45) and DRS (scored 0-100) demonstrated mean (SD) scores of 27.5 (11.5) and 38.4 (19.4), with higher and lower scores, respectively, demonstrating greater patient satisfaction with SDM. SDM is useful for patients involved in complex decision-making when several medically reasonable treatment alternatives are available. Patients with CCCA may benefit from this model, and investment in resources such as patient decision aids and SDM training for dermatologists will aid integration into clinical practice. Together, the CPS, DRS and SDM-Q-9 results suggested that patients with CCCA preferred to be involved in their treatment management decisions, regardless of their sociodemographic characteristics.

Lichen planopilaris (LPP), frontal fibrosing alopecia (FFA) and central centrifugal cicatricial alopecia (CCCA) are primary cicatricial alopecias. While CCCA has been linked to increased uterine fibroid risk, similar associations for LPP and FFA remain unexplored. This study found that classic LPP, but not the FFA, was associated with a higher risk of ovarian cysts but not uterine fibroids. These results suggest hormonal dysregulation in LPP and a distinct fibroproliferative mechanism from CCCA, underscoring its link to gynaecological comorbidities.

Acne keloidalis nuchae (AKN) is a chronic scarring folliculitis which usually occurs in young adult males of African descent. Studies have suggested that AKN may be associated with other dermatologic conditions and even general medical disorders. The aim of this study was to identify cutaneous and extracutaneous associated disorders and to examine risk factors in our population for developing AKN. The study was a retrospective, descriptive, and analytical study conducted at the Dermatology Outpatient department of the University Hospital of the West Indies. Data were obtained from the medical records of patients diagnosed over a 15-year period (2000-2014). There were 1031 new patients during the study period. Of these, 43 (4.2%) had AKN. The male to female ratio for AKN was approximately 7:1. Pseudofolliculitis barbae was associated with keloidal plaques on the scalp (OR = 6.22, P = 0.036). Also, when the duration of AKN was divided into two groups (0-5 years and greater than 5 years), there was an association between keloidal plaques and duration of greater than 5 years (OR = 7.5, P = 0.032). However, when the odds ratio was adjusted, only the duration of AKN remained significantly associated with keloidal plaques (OR = 7.08, P = 0.047). Chronic scalp folliculitis (P = 0.001) and the presence of any component disease of the metabolic syndrome (OR = 14, P = 0.008) and specifically hypertension (OR = 6.75, P = 0.036) were significantly associated with the extension of the lesions beyond the nape and occipital scalp. Pseudofolliculitis barbae, chronic scalp folliculitis, and aspects of the metabolic syndrome may be associated with acne keloidalis nuchae.

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Background:Central centrifugal cicatricial alopecia (CCCA) is a type of scarring alopecia exclusively seen in women of African descent. The etiology is unknown and epidemiologic studies including data on comorbidities in patients with CCCA are limited. Our primary objective was to identify possible etiologic and lifestyle associations in patients with CCCA.Materials and Methods:A retrospective chart review was conducted for patients diagnosed with CCCA between January 1, 2013, and January 1, 2018, at a university dermatology outpatient clinic. Controls consisted of age-, sex-, and race-matched African–American women diagnosed with other hair loss conditions. Data from 74 cases and 96 controls were collected and analyzed via logistic regression. In addition, a phone survey was conducted in the CCCA cohort asking specific questions related to their condition and quality of life.Results:A significant relationship was found between CCCA and previous pregnancies as well as the use of chemical relaxers: patients with CCCA were 11.71 times more likely to have had a previous pregnancy (P < 0.001) and 12.37 times more likely to have used chemical relaxers in the past (P < 0.001). Association with uterine fibroids was found not statistically significant (P > 0.05).Conclusion:We identified an association between previous pregnancies and use of chemical relaxers in patients diagnosed with CCCA when compared to controls. These findings may help to plan prospective studies aiming at establishing a more concrete link between hormones and CCCA.

The term “skin of color” identifies individuals of racial groups with skin darker than Caucasians, such as Asians, Africans, Native Americans, and Pacific Islanders. Patients with skin of color often have distinctive cutaneous and hair characteristics, disorders and reaction patterns. There are skin disorders that disproportionately affect skin of color patients, such as keloids, hidradenitis suppurativa, and central centrifugal cicatricial alopecia – these conditions are underresearched and the underlying causes remain poorly understood. Recent progress in our understanding of the mechanisms underlying these diseases has begun to emerge. In this talk, I will discuss these diseases with an emphasis on keloids; the racial differences in their epidemiology and the recent progress in the genetic and molecular understanding of these conditions.