Central centrifugal cicatricial alopecia in Black men: A case series highlighting key clinical features in this cohort

Abstract

Central centrifugal cicatricial alopecia (CCCA) is the most common cicatricial alopecia in Black patients and results in progressive hair loss and profound impact on well-being, associated with depression and poorer quality of life.1Adotama P. Shapiro J. Lo Sicco K. et al.Standardizing the clinical and dermatoscopic exam for patients with afro-textured hair and scarring alopecia: a scoping review and clinical guide.JEADV Clin Pract. 2022; 1: 31-37Crossref Google Scholar,2Gathers R.C. Lim H.W. Central centrifugal cicatricial alopecia: past, present, and future.J Am Acad Dermatol. 2009; 60: 660-668Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar Given the progressive nature of this condition, early and accurate diagnosis and treatment initiation are essential. Although CCCA has been well studied in Black women, there is a dearth of information regarding its manifestations in Black men. Furthermore, its history, physical examination, and trichoscopy patterns have been well described in female cohorts with CCCA; however, there is less information on special considerations for men with this condition. Given the predilection for CCCA to occur in female patients, providers may have a delayed diagnosis of CCCA in men with hair loss in the vertex scalp or may misdiagnose their condition for more common forms of hair loss such as androgenetic alopecia (AGA). Although AGA may exist concomitantly with CCCA, especially in male patients, and therapies, such as minoxidil, can be used to increase hair density in both conditions, it is important to identify CCCA correctly to initiate additional anti-inflammatory therapy as soon as possible. Herein, we present 3 cases of biopsy-supported CCCA in Black men, along with a review of pertinent clinical, trichoscopic, and histopathologic findings of CCCA. All patients gave consent for their photographs and medical information to be published in print and online and with the understanding that this information may be publicly available. A 41-year-old man presented to a hair clinic with a chief complaint of hair loss for 1.5 years. He reported hair breakage and balding on the crown of his scalp, with associated hair shedding. He wore his hair in waist-length locks in a mid-ponytail for >18 years and reported washing it every 2 weeks with various shampoos. He had a family history of progressive hair loss in his maternal grandmother. Physical examination revealed a normal frontal hairline but decreased hair density and obliteration of the follicular ostia on the vertex scalp (Fig 1). A honeycomb pigmented pattern, decreased hair density, and peripilar gray halos were observed using trichoscopy of the vertex (Fig 2). A 4-mm punch biopsy revealed a decreased number of terminal anagen follicles, with fibrosis, a lymphocytic infiltrate, and loss of sebaceous glands. Clinicopathologic correlation was consistent with CCCA. Treatment with twice-daily minoxidil solution 5%, daily metformin compounded solution 10%, and clobetasol solution 0.05% was initiated. Later in the treatment course, he started 1 mg of oral finasteride daily and received regular 5-mg/mL intralesional triamcinolone acetonide injections.Fig 2Peripilar white/gray halos (arrows) with mild surrounding scale (white arrows), honeycomb pattern with small white dots in between, and increased distance between hair follicles, with decreased follicular ostia.View Large Image Figure ViewerDownload Hi-res image Download (PPT) A 36-year-old man presented to a hair clinic with a chief complaint of asymptomatic hair loss along the crown of his scalp for 3 years. He had a history of seborrheic dermatitis, which he managed with antifungal shampoos and supplements. Physical examination revealed significant thinning along the vertex scalp, with a retained frontal hairline (Figs 3 and 4). Trichoscopy demonstrated follicular miniaturization, a honeycomb pigmented pattern, and peripilar white halos. A scalp biopsy revealed a decrease in the size and number of terminal hair follicles, prominent sebaceous lobules, and foci with prominent perifollicular fibrosis and inflammation. Given the clinicopathologic correlation, the patient was diagnosed with CCCA and started on twice-daily minoxidil solution 5%.Fig 4Patient 2 with hair loss on the vertex scalp, with retention of the frontotemporal hairline, at initial presentation.View Large Image Figure ViewerDownload Hi-res image Download (PPT) A 37-year-old man presented to a hair clinic with a chief complaint of hair loss on the scalp for 8 years. His initial symptoms of scalp flaking and pruritus gradually progressed to generalized hair loss and a focal alopecic patch on the vertex scalp (Fig 5). He reported a history of high-tension hairstyles, including locks and braids. He had a strong family history of total baldness in his brother, father, and mother. Trichoscopic examination revealed perifollicular erythema, peripilar gray halos, and perifollicular scale in the focal alopecic patch. A 4-mm scalp biopsy was notable for perifollicular fibrosis, with loss of sebaceous glands, a lymphohistiocytic inflammatory infiltrate, and focal areas of premature loss of the inner root sheath (Fig 6), which was most consistent with CCCA. The patient was diagnosed with CCCA, given the biopsy findings and clinical corollary, and started on a compounded cream consisting of 12% minoxidil with 0.05% clobetasol in a petrolatum emulsion 3 times weekly, 100-mg doxycycline hyclate capsules once daily, and regular 5-mg/mL intralesional triamcinolone acetonide injections.Fig 6Follicle at center exhibiting premature desquamation of the inner root sheath. The inner root sheath of this follicle has desquamated below the level of the isthmus and shows perifollicular concentric fibrosis. There is an adjacent fibrous follicular scar to the right, a sequela of follicular dropout.View Large Image Figure ViewerDownload Hi-res image Download (PPT) CCCA is a primary scarring alopecia that disproportionately impacts adult women of African ancestry. It classically begins at the vertex scalp and expands centrifugally with loss of follicular ostia as the disease progresses. Most cases of CCCA have been reported in the late second or third decade of life in Black women,2Gathers R.C. Lim H.W. Central centrifugal cicatricial alopecia: past, present, and future.J Am Acad Dermatol. 2009; 60: 660-668Abstract Full Text Full Text PDF PubMed Scopus (69) Google Scholar whereas few cases are known in men, which could result from underdiagnosis in the male population. Hair grooming practices, such as chemical relaxers, hot combs, and tight hairstyles, have been linked to the etiology of CCCA, although multiple studies have not shown consistent findings.3Olsen E.A. Callender V. McMichael A. et al.Central hair loss in African American women: incidence and potential risk factors.J Am Acad Dermatol. 2011; 64: 245-252Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar The pathophysiology of CCCA is not entirely understood; however, it is believed to be multifactorial. In some families, the inheritance pattern is similar to that of an autosomal dominant trait.4Aguh C. Dina Y. Talbot Jr., C.C. Garza L. Fibroproliferative genes are preferentially expressed in central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2018; 79: 904-912Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar Additionally, missense mutations leading to decreased expression of peptidyl arginine deiminase type III have been found in scalp biopsies of women with CCCA.5Malki L. Sarig O. Romano M.T. et al.Variant PADI3 in central centrifugal cicatricial alopecia.N Engl J Med. 2019; 380: 833-841Crossref PubMed Scopus (74) Google Scholar Along with fibroproliferative disorders, type 2 diabetes mellitus, hyperlipidemia, and breast cancer have also recently been shown to be associated with CCCA.3Olsen E.A. Callender V. McMichael A. et al.Central hair loss in African American women: incidence and potential risk factors.J Am Acad Dermatol. 2011; 64: 245-252Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar,4Aguh C. Dina Y. Talbot Jr., C.C. Garza L. Fibroproliferative genes are preferentially expressed in central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2018; 79: 904-912Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar CCCA clinically manifests as a patch of hair thinning at the midline portion of the vertex scalp, progressing over time in a centrifugal pattern (Figs 1, 3, and 5). Atypical presentations, with irregular patchy alopecia in the occipital or temporal scalp, have also been reported.6De Souza B. Tovar-Garza A. Uwakwe L.N. McMichael A. Bitemporal scalp hair loss: differential diagnosis of nonscarring and scarring conditions.J Clin Aesthet Dermatol. 2021; 14: 26-33PubMed Google Scholar Our patients demonstrated the classic presentation with thinning at the vertex scalp and a retained frontal hairline (Figs 4 and 5). In early disease, unexplained hair breakage on the crown can be one of the first signs and can occasionally be accompanied by a burning sensation, pain, tenderness, dysesthesia, or pruritus of the scalp.6De Souza B. Tovar-Garza A. Uwakwe L.N. McMichael A. Bitemporal scalp hair loss: differential diagnosis of nonscarring and scarring conditions.J Clin Aesthet Dermatol. 2021; 14: 26-33PubMed Google Scholar,7Shah S.K. Alexis A.F. Central centrifugal cicatricial alopecia: retrospective chart review.J Cutan Med Surg. 2010; 14: 212-222Crossref PubMed Scopus (18) Google Scholar Clinical manifestations of inflammation are highly variable among patients; it can be apparent with follicular papules, pustules, perifollicular erythema, and scaling.8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar Notably, 2 of the 3 patients in our case series reported ongoing or prior locks, a high-tension styling practice, which may have potentially propagated inflammation and progression of the disease, although more studies are needed to confirm this observation. As with our patients, most people with CCCA do not seek medical care for several months or even years as hair loss progresses, making an early diagnosis of the disease less common.7Shah S.K. Alexis A.F. Central centrifugal cicatricial alopecia: retrospective chart review.J Cutan Med Surg. 2010; 14: 212-222Crossref PubMed Scopus (18) Google Scholar Clinical examination often reveals loss of follicular ostia between hair shafts, which is characteristic of cicatricial alopecias.8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar The most common and specific trichoscopy finding is peripilar white or gray halos, which was seen in all our patients (Fig 2). Other trichoscopy findings include honeycomb pigmented network interrupted by irregular white patches, follicular papules and pustules, perifollicular erythema, and scale.8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar The peripilar white-gray halos seen using trichoscopy are often correlated with the histologic finding of perifollicular fibrosis surrounding the outer root sheath.9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google Scholar Additional histologic features of CCCA include lymphocytic infiltrate, follicular dropout, and premature degeneration of the inner root sheath (Fig 6).9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google Scholar CCCA and AGA have overlapping clinical and trichoscopic features that can lead to misdiagnosis (Table I).8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar, 9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google Scholar, 10Rossi A. Ferranti M. Magri F. et al.Clinical and trichoscopic graded live visual scale for androgenetic alopecia.Dermatol Pract Concept. 2022; 12e2022078PubMed Google Scholar, 11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google Scholar Both CCCA and AGA can present initially as hair thinning on the vertex scalp; however, the limited extent of hairline recession in CCCA contrasts the more extensive recession of the frontotemporal scalp in AGA (Figs 4 and 5). Unlike the Norwood Hamilton scale for male pattern hair loss, wherein patients demonstrate progressive bitemporal thinning prior to notable vertex scalp hair loss, our series of men had relative retention of the temporal hairline. This may be a useful clue for distinguishing CCCA from AGA clinically, considering their potential overlap and concurrent existence, because AGA is the leading form of alopecia in men. Patients with CCCA may also present with symptoms of itch, pain, or burning. Perifollicular erythema, follicular papules, and scaling are also more suggestive of a scarring process, with deep-red or violaceous hues often seen in darker skin types. The lack of inflammatory features in AGA can prompt clinicians to consider other types of alopecia, such as CCCA, while formulating a comprehensive differential. These 2 forms of alopecia can also be distinguished based on trichoscopy findings because the absence of follicular ostia is key to the scarring process in CCCA.7Shah S.K. Alexis A.F. Central centrifugal cicatricial alopecia: retrospective chart review.J Cutan Med Surg. 2010; 14: 212-222Crossref PubMed Scopus (18) Google Scholar There are no established diagnostic criteria for CCCA12Okereke U.R. Simmons A. Callender V.D. Current and emerging treatment strategies for hair loss in women of color.Int J Womens Dermatol. 2019; 5: 37-45Crossref PubMed Scopus (17) Google Scholar; therefore, clinicopathologic correlation is often necessary to differentiate AGA and CCCA or confirm the diagnosis in a concomitant case of both and aid in individualized management options for better outcomes. Although AGA may exist concomitantly with CCCA, especially in male patients, and therapies, such as minoxidil, can be used to increase hair density in both conditions, it is important to identify CCCA correctly to initiate additional anti-inflammatory therapy as soon as possible.Table IComparison of androgenetic alopecia and central centrifugal cicatricial alopecia in Black menAGACCCAClinical historyFamily history of hair loss consistent with Norwood scaleHistory of high-tension hairstyle practices (cornrows, braids, extensions, weaves with sewn-in or glued-on hair), use of hot combs, and frequent use of hair relaxers3Olsen E.A. Callender V. McMichael A. et al.Central hair loss in African American women: incidence and potential risk factors.J Am Acad Dermatol. 2011; 64: 245-252Abstract Full Text Full Text PDF PubMed Scopus (61) Google ScholarFamily history of hair loss in similar pattern Hair care practices Family historyClinical featuresBitemporal or frontal hairline recession in addition to thinning of temporal and vertex scalpInflammatory features not presentDistribution: focal, vertex/crown of the scalp, with retained frontal hairlinePain, burning sensation, tenderness, dysesthesia, and pruritus8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar Distribution Inflammatory featuresTrichoscopic featuresFollicular miniaturization10Rossi A. Ferranti M. Magri F. et al.Clinical and trichoscopic graded live visual scale for androgenetic alopecia.Dermatol Pract Concept. 2022; 12e2022078PubMed Google Scholar,11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarPeripilar sign (brown halo)11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarYellow dots (empty follicles)10Rossi A. Ferranti M. Magri F. et al.Clinical and trichoscopic graded live visual scale for androgenetic alopecia.Dermatol Pract Concept. 2022; 12e2022078PubMed Google ScholarAnisotrichosis10Rossi A. Ferranti M. Magri F. et al.Clinical and trichoscopic graded live visual scale for androgenetic alopecia.Dermatol Pract Concept. 2022; 12e2022078PubMed Google ScholarIncreased proportion of vellus hairs11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarFocal atrichia10Rossi A. Ferranti M. Magri F. et al.Clinical and trichoscopic graded live visual scale for androgenetic alopecia.Dermatol Pract Concept. 2022; 12e2022078PubMed Google ScholarHoneycomb pigmented network (normal)8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarPeripilar white-gray halo8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarFollicular dropout8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarFollicular papules and pustules8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarPerifollicular erythema and scale8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarFollicular miniaturization8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarHoneycomb pigmented network, often interrupted by white patches8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google ScholarHistologic featuresProgressive miniaturization of terminal hair follicles11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarIncreased number of vellus and telogen hairs11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarReduction of anagen-to-telogen ratio (<5:1)11El-Domyati M. Attia S. Saleh F. Abdel-Wahab H. Androgenetic alopecia in males: a histopathological and ultrastructural study.J Cosmet Dermatol. 2009; 8: 83-91Crossref PubMed Scopus (46) Google ScholarLymphocytic infiltrates8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar,9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google ScholarPerifollicular fibrosis8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar,9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google ScholarFollicular dropout8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar,9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google ScholarPremature degeneration of the inner root sheath8Miteva M. Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia.J Am Acad Dermatol. 2014; 71: 443-449Abstract Full Text Full Text PDF PubMed Scopus (51) Google Scholar,9Miteva M. Tosti A. Dermoscopy guided scalp biopsy in cicatricial alopecia.J Eur Acad Dermatol Venereol. 2013; 27: 1299-1303PubMed Google ScholarAGA, Androgenetic alopecia, CCCA, central centrifugal cicatricial alopecia. Open table in a new tab AGA, Androgenetic alopecia, CCCA, central centrifugal cicatricial alopecia. Our 3 cases reveal that although CCCA has been predominant in Black women, it should also be considered in Black men. Our cases highlight a key clinical distinguishing feature of male CCCA, demonstrating notable thinning on the vertex scalp with relative maintenance of the hairline, a feature that is yet to be noted in previous literature and can help clinicians more easily differentiate this form of scarring alopecia in Black men from AGA. To our knowledge, there have only been 12 reported cases of CCCA in Black men in the literature.13Davis E.C. Reid S.D. Callender V.D. Sperling L.C. Differentiating central centrifugal cicatricial alopecia and androgenetic alopecia in African American men: report of three cases.J Clin Aesthet Dermatol. 2012; 5: 37-40PubMed Google Scholar, 14Sperling L.C. Skelton III, H.G. Smith K.J. Sau P. Friedman K. Follicular degeneration syndrome in men.Arch Dermatol. 1994; 130: 763-769Crossref PubMed Google Scholar, 15Rondina A. Gathers R.C. A case of central centrifugal cicatricial alopecia in an African American man.J Am Acad Dermatol. 2009; 60: AB102Google Scholar The paucity of published evidence of CCCA cases in Black men raises the following question: how common is misdiagnosis of CCCA for other alopecias? Our case series demonstrates the need for clinicians to thoroughly review symptoms as well as clinical and trichoscopic findings and have an appropriate threshold for scalp biopsy when there is concern for a scarring process. Our cases add to the limited, but now growing, evidence of CCCA in Black men and are a call for future studies to explore the clinical characteristics of CCCA and diagnostic outcomes in Black men. None disclosed.