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CASES FROM ACKERMAN ACADEMY
Year : 2013  |  Volume : 4  |  Issue : 1  |  Page : 50-51  

Central centrifugal cicatricial alopecia


1 Ackerman Academy of Dermatopathology, New York, USA
2 Polley Clinic of Dermatology and Dermatologic Surgery, Wilson, North Carolina, USA

Date of Web Publication3-Jan-2013

Correspondence Address:
Dirk M Elston
Ackerman Academy of Dermatopathology, New York
USA
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5178.105484

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   Abstract 

Central centrifugal cicatricial alopecia is a common cause of progressive permanent apical alopecia. This unique form of alopecia includes entities previously know as "hot comb alopecia," "follicular degeneration syndrome," "pseudopelade" in African Americans and "central elliptical pseudopelade" in Caucasians. The etiology appears to be multifactorial and the condition occurs in all races.

Keywords: Alopecia, central, centrifugal, cicatricial, pseudopelade


How to cite this article:
Blattner C, Polley DC, Ferritto F, Elston DM. Central centrifugal cicatricial alopecia. Indian Dermatol Online J 2013;4:50-1

How to cite this URL:
Blattner C, Polley DC, Ferritto F, Elston DM. Central centrifugal cicatricial alopecia. Indian Dermatol Online J [serial online] 2013 [cited 2019 Dec 16];4:50-1. Available from: http://www.idoj.in/text.asp?2013/4/1/50/105484

Central centrifugal cicatricial alopecia (CCCA) is the term adopted by the North American Hair Research Society (NAHRS) to encompass the previous terms of "hot comb alopecia," "follicular degeneration syndrome," "pseudopelade" in African Americans and "central elliptical pseudopelade" in Caucasians. [1] CCCA is a subcategory of primary, inflammatory cicatricial alopecia, and is the most common form of scarring alopecia in many populations. [2] CCCA presents as progressive apical alopecia. [3] Certain hair care practices have been associated with increased risk for developing CCCA; in a review of 44 patients by McMichael, [4] more women with scarring alopecia had a history of hair weaving and long duration of chemical relaxer usage as compared to those unaffected by scarring alopecia. [5] However, there is developing evidence that the etiology of CCCA may be multifactorial, occurs in all races, and does not always relate to obvious hair care practices. [6]


   Clinical Features Top


The clinical presentation of CCCA appears to be similar between men and women. [5] However, clinically diagnosing CCCA may be challenging because it can resemble female pattern hair loss, alopecia areata, lichen planopilaris, or telogen effluvium. [7] A biopsy is often necessary to confirm the diagnosis. CCCA typically begins and remains most severe on the crown or vertex of the scalp, gradually expanding in a centrifugal fashion [Figure 1]. [8] 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. [9] Patients commonly complain of mild dysesthesia (pruritus, tenderness) in the affected area. [7],[10] The disease generally progresses slowly, but longstanding or severe disease can result in hair loss covering the entire crown of the scalp. [11] This is in contrast to nonscarring (non-cicatricial) alopecia, which is reversible as the follicular epithelium remains intact. [9] CCCA, at its end stages, is irreversible, as the follicular epithelium has been replaced by connective tissues. [12] Additionally, pustules and crusting may be found in patients with superimposed folliculitis decalvans.
Figure 1: Central centrifugal cicatricial alopecia presents with progressive permanent apical alopecia

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   Histological Features Top


CCCA, in both men and women, will display similar histopathological features [6] including contraction of dermal collagen with loss of space between collagen bundles and broad hyalinized fibrous tracts that may contain naked hair shafts [Figure 2]. [13] Premature desquamation of the inner root sheath has also been described as a characteristic feature, but is also present in lichen planopilaris and other forms of scarring alopecia. In elastic Van Geison-stained sections, thickened dermal elastic fibers are present. [14] The elastic sheath surrounding the fibrous tract is preserved [Figure 3] and often duplicated in contrast to lupus erythematosus, lichen planopilaris, and folliculitis decalvans, where there is loss of the elastic sheath. [15] Additionally, CCCA may demonstrate perifollicular mucinous fibrosis and sparse lymphocytic perifollicular inflammation, which primarily occurs at the level of the upper isthmus and lower infundibulum. [2] In advanced lesions, total destruction of the follicular epithelium with retained hair shaft fragments and granulomatous inflammation will be seen.
Figure 2: Central centrifugal cicatricial alopecia is characterized by hyalinization of dermal collagen with broad tree trunk-like fibrous tracts (H and E, ×20)

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Figure 3: Central centrifugal cicatricial alopecia is characterized by preservation of the elastic sheath surrounding the fibrous tracts (H and E, ×100)

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   Treatment Top


Therapeutic options are limited. Hair styles that require heat treatment or produce traction should be avoided. Ceasing the use of relaxers is also suggested, as this practice correlates with increased incidence of disease. [1] For those patients who have crops of pustules, suggesting superimposed folliculitis decalvans, topical corticosteroids, and oral tetracyclines can be of benefit. Hair transplantation can be useful in patients with advanced disease; however, CCCA presents a unique challenge for hair transplantation as the presence of scarring can decrease the transplanted graft survival rate. [4]

 
   References Top

1.Olsen EA, Callender V, McMichael A, Sperling L, Anstrom KJ, Shapiro J, et al. Central hair loss in African American women: Incidence and potential risk factors. J Am Acad Dermatol 2011;64:245-52.  Back to cited text no. 1
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2.Jean B, Jorizzo JL, Rapini RP. Dermatology. London: Mosby; 2003.  Back to cited text no. 2
    
3.Kyei A, Bergfeld WF, Piliang M, Summers P. Medical and environmental risk factors for the development of central centrifugal cicatricial alopecia: A population study. Arch Dermatol 2011;147:909-14.  Back to cited text no. 3
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4.McMichael AJ. Ethnic hair update: Past and present. J Am Acad Dermatol 2003;48:S127-33.  Back to cited text no. 4
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5.Davis EC, Reid SD, Callender VD, Sperling LC. Differentiating central centrifugal cicatricial alopecia and androgenetic alopecia in African American men: Report of three cases. J Clin Aesthet Dermatol 2012;5:37-40.  Back to cited text no. 5
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6.Summers P, Kyei A, Bergfeld W. Central centrifugal cicatricial alopecia - an approach to diagnosis and management. Int J Dermatol 2011;50:1457-64.  Back to cited text no. 6
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7.Sperling LC, Sau P. The Follicular degeneration syndrome in black patients. 'Hot comb alopecia' revisited and revised. Arch Dermatol 1992;128:68-74.  Back to cited text no. 7
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8.Callender VD, McMichael AJ, Cohen GF. Medical and surgical therapies for alopecias in black women. Dermatol Ther 2004;17:164-76.  Back to cited text no. 8
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9.Gathers RC, Jankowski M, Eide M, Lim HW. Hair grooming practices and central centrifugal cicatricial alopecia. J Am Acad Dermatol 2009;60:574-8.  Back to cited text no. 9
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10.Sperling LC, Sau P. The follicular degeneration syndrome in black patients. Arch Dermatol 1992;128:68-74.  Back to cited text no. 10
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11.Whiting DA, Olsen EA. Central centrifugal cicatricial alopecia. Dermatol Ther 2008;21:268-78.  Back to cited text no. 11
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12.Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg 2006;25:41-50.  Back to cited text no. 12
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13.Miteva M, Tosti A. 'A detective look' at hair biopsies from African-American patients. Br J Dermatol 2012;166:1289-94. Available from: Academic Search Elite, Ipswich, MA.  Back to cited text no. 13
    
14.Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol 2005;53:1-37.  Back to cited text no. 14
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15.Elston DM, McCollugh ML, Warschaw KE, Bergfeld WF. Elastic tissue in scars and alopecia. J Cutan Pathol 2000;27:147-52.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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