Clinical Challenge: Thinning Hair in Woman

Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia that primarily affects Black women.1 It was originally described in 1968 as “hot comb alopecia” in a series of Black women in the United States who straightened their hair with a hot...

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Central centrifugal cicatricial alopecia (CCCA) is a scarring alopecia that primarily affects Black women.¹ It was originally described in 1968 as “hot comb alopecia” in a series of Black women in the United States who straightened their hair with a hot comb and petrolatum products.² This nomenclature has been discarded following reports of the disorder in women who never used these hair products and treatments.³ Prevalence increases with age; most women are affected in the late second or third decades of life.⁴

Diagnosing CCCA may be challenging because it can resemble female pattern hair loss, alopecia areata, tinea capitis, lichen planopilaris, discoid lupus erythematosus, or telogen effluvium.⁴ It classically presents with a round area of hair loss beginning at the vertex of the scalp and spreading centrifugally sparing the lateral and posterior scalp.⁵ Findings on clinical examination may include soft scalp on palpation, mild hyperpigmentation around the hair follicles, mild burning, tenderness, itching confined to areas of hair loss, and islands of unaffected hairs with polytrichia seen within affected areas.³

The etiology of CCCA is not well understood. Familial occurrence has been documented with susceptibility to the disease likely inherited in an autosomal dominant manner.⁶A defect in the peroxisome proliferator-activated receptor-gamma (PPAR-γ) has been implicated in the lymphocytic cicatricial alopecia associated with lichen planopilaris and may play a role in CCCA.⁶

Dermoscopy can be useful in diagnosis and identification of the best location for scalp biopsy.⁷ The most specific and sensitive dermatoscopic finding for CCCA is a peripilar gray/white halo.⁸ Dermatocopic findings of a honeycomb-pigmented network, pinpoint white dots, hair shaft variability, perifollicular edema, and interfollicular-pigmented asterisk-like brown macules have also been reported.⁷

Histopathologically, the predominant inflammatory cellular infiltrate of CCCA is a CD 4-predominant T-cell infiltrate with increased Langerhans cells extending into the lower hair follicle.⁵ Later stages are associated with follicular epithelium destruction and retention of fragments of hair shaft along with granulomatous inflammation followed by replacement of follicular epithelium with connective tissues and fusion of the infundibulum.³

Central centrifugal cicatricial alopecia is a scarring alopecia. The goal of treatment is to preserve available hair and prevent further progression.⁹ Topical steroids or intralesional triamcinolone acetonide are often considered first-line therapy.³ Other therapeutic options include doxycycline, topical calcineurin inhibitors, vitamins, herbal treatments, minoxidil, cyclosporine, and mycophenolate mofetil.^9,10

Alexandra Stroia, BS, is a medical student at the Lake Erie College of Osteopathic Medicine in Erie, Pennsylvania; Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.

References

1. Malki L, Sarig O, Romano MT, et al. Variant PADI3 in central centrifugal cicatricial alopecia. N Engl J Med. 2019;380(9):833-841. doi:10.1056/NEJMoa1816614

2. LoPresti P, Papa CM, Kligman AM. Hot comb alopecia. Arch Dermatol. 1968;98:234-238.

3. Gabros S, Masood S. Central centrifugal cicatricial alopecia. In: StatPearls. StatPearls Publishing; July 20, 2021. https://www.ncbi.nlm.nih.gov/books/NBK559187/

4. Summers P, Kyei A, Bergfeld W. Central centrifugal cicatricial alopecia – an approach to diagnosis and management. Int J Dermatol. 2011;50(12):1457-1464. doi:10.1111/j.1365-4632.2011.05098.x

5. Flamm A, Moshiri AS, Roche F, et al. Characterization of the inflammatory features of central centrifugal cicatricial alopecia. J Cutan Pathol. 2020;47(6):530-534. doi:10.1111/cup.13666

6. Dlova NC, Jordaan FH, Sarig O, Sprecher E. Autosomal dominant inheritance of central centrifugal cicatricial alopecia in black South Africans. J Am Acad Dermatol.2014;70(4):679-682.e1

7. Herskovitz I, Miteva M. Central centrifugal cicatricial alopecia: challenges and solutions. Clin Cosmet Investig Dermatol. 2016;9:175-181. doi:10.2147/CCID.S100816

8. Miteva M, Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia. J Am Acad Dermatol. 2014;71(3):443-449. doi:10.1016/j.jaad.2014.04.069

9. Ezekwe N, King M, Hollinger JC. The use of natural ingredients in the treatment of alopecias with an emphasis on central centrifugal cicatricial alopecia: a systematic review. J Clin Aesthet Dermatol. 2020;13(8):23-27.

10. Price VH. The medical treatment of cicatricial alopecia. Semin Cutan Med Surg. 2006;25(1):56-59. doi:10.1016/j.sder.2006.01.008