Clinical Challenge: Firm Papules and Hair Loss

Acne keloidalis nuchae or folliculitis keloidalis nuchae is a chronic inflammatory condition consisting of keloid-like papules and plaques that eventually manifest in scarring alopecia.1 It was first described by Kaposi in 1869 as dermatitis papillaris capillitia and was later coined acne...

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Acne keloidalis nuchae or folliculitis keloidalis nuchae is a chronic inflammatory condition consisting of keloid-like papules and plaques that eventually manifest in scarring alopecia.¹ It was first described by Kaposi in 1869 as dermatitis papillaris capillitia and was later coined acne keloidalis nuchae.^2,3 The name is misleading, however, as this is not a form of acne.⁴

A majority of cases arise in people of African descent and the disorder occurs infrequently in White patients.^5,6 The condition is 20 times more common in men than women, suggesting that androgens may play a role in development.⁷ It rarely manifests before puberty or after middle age.

Acne keloidalis nuchae is induced by chronic inflammation or occlusion of the hair follicles. Injury to the skin and hair follicles as a result of hair cutting can lead to disease.⁸ Hair texture and propensity for ingrown hair allow for extrafollicular and transfollicular penetration of the skin favoring the development of acne keloidalis nuchae. An aberrant immune reaction resulting in chronic inflammation also plays a role in pathogenesis.⁹

Acne keloidalis nuchae is classified by the North American hair research society as a mixed form of primary cicatricial alopecia.¹⁰ Characteristic presentation is that of firm inflammatory papules and pustules on the occiput and/or nape of the neck that spread laterally.¹¹ The onset of symptoms is preceded by pruritis several hours to days prior, commonly following irritation to the area. Coalescence and fibrosis of papules lead to formation of keloidal scarring plaques. Due to the pruritic nature, patients often scratch at the lesions causing inflammation and development of secondary bacterial infections. In some cases, recurrent folliculitis leads to development of patchy scarring alopecia.

The diagnosis is clinical although atypical cases may warrant culture. Dermatoscopic features of early lesions may show hair shafts trapped in individual papules.¹ Biopsy is rarely indicated but may be done to exclude conditions such as psoriasis and folliculitis decalvans.¹² Histopathology often reveals a neutrophilic and lymphocytic infiltrate surrounding the isthmus of the hair follicle and naked hair shafts in the dermis.¹³

Management of acne keloidalis nuchae is difficult. Medical treatment includes the use of topical or intralesional steroids, retinoids, and topical or oral antibiotics.^4,11 A recent case report documented successful treatment with halobetasol and tazarotene.¹⁴ Surgical removal of refractory areas is another option.¹⁵

Patients should be advised to avoid close shaving and frequent haircuts. Providers should also educate patients about the use of antimicrobial cleansers, wearing loose clothing around the neck to prevent rubbing, and avoidance of pomades and hair grease.¹⁶

Alexandra Stroia, BS, is a medical student at the Lake Erie College of Osteopathic Medicine. 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. Ogunbiyi A. Acne keloidalis nuchae: prevalence, impact, and management challenges. Clin Cosmet Investig Dermatol. 2016;9:483-489. doi:10.2147/CCID.S99225
2. Kaposi M. Ueber die sogennante Framboesia und mehrere andere arten von papillaeren neubildungen der Haut. Arch Dermatol Syphilol. 1869;3382-423.
3. Fox H. Folliculits keloidalis a better term than dermatitis papillaris capillitii.  Arch Derm Syphilol. 1947;55(1):112. doi:10.1001/archderm.1947.01520010116015
4. Labib A, Salfity L, Powell B. Acne keloidalis nuchae: a staged reconstruction. Cureus. 2021;13(9):e18173. doi:10.7759/cureus.18173
5. Kantor GR, Ratz JL, Wheeland RG. Treatment of acne keloidalis nuchae with carbon dioxide laser. J Am Acad Dermatol. 1986;14(2 Pt 1):263-267. doi:10.1016/S0190-9622(86)70031-5
6. Azurdia RM, Graham RM, Wesmann K, Guerin DM. Acne keloidalis in caucasian patients on cyclosporine following organ transplantation. Br J Dermatol. 2000;143(2):465-467. doi:10.1046/j.1365-2133.2000.03694.x
7. Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. 2003;21(4):645-653. doi:10.1016/S0733-8635(03)00079-2
8. Adotama P, Tinker D, Mitchell K, Glass DA, Allen P. Barber knowledge and recommendations regarding pseudofolliculitis barbae and acne keloidalis nuchae in an urban setting. JAMA Dermatol. 2017;153(12):1325-1326. doi:10.1001/jamadermatol.2017.3668
9. Sperling LC, Homoky C, Prat L, Sau P. Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol. 2000;136:479-484. doi:10.1001/archderm.136.4.479
10. Olsen EA, Bergfeld WF, Cotsarelis G, et al. Summary of North American Hair Research Society (NAHRS)-sponsored workshop on cicatricial alopecia, Duke Medical Centre, February 10 and 11, 2001. J Am Acad. 2003;48(1):103-110. doi:10.1067/mjd.2003.68
11. Maranda EL, Simmons BJ, Nguyen AH, Lim VM, Keri JE. Treatment of acne keloidalis nuchae: a systematic review of the literature. Dermatol Ther (Heidelb)). 2016;6(3):363-378. doi:10.1007/s13555-016-0134-5
12. Al Aboud DM, Badri T. Acne keloidalis nuchae. In: StatPearls [Internet]. StatPearls Publishing; 2022. Updated May 1, 2022. Accessed August 15, 2022. https://www.ncbi.nlm.nih.gov/books/NBK459135/
13. Bernárdez C, Molina-Ruiz AM, Requena L. Histologic features of alopecias: part II: scarring alopecias. Actas Dermosifiliogr. 2015;106(4):260-270. doi:10.1016/j.ad.2014.06.016.
14. Marushchak O, Tan KJ, Encarnacion MR, Clark L, Golant A. Acne keloidalis nuchae successfully treated with halobetasol 0.01% and tazarotene 0.045%. J Skin. 2022;6(4): 311-314. doi:10.25251/skin.6.4.6
15. Galarza LI, Azar CA, Al Hmada Y, Medina A. Surgical management of giant acne keloidalis nuchae lesions. Case Reports Plast Surg Hand Surg. 2021;8(1):145-152. doi:10.1080/23320885.2021.1982392
16. Ludmann P. Acne keloidalis nuchae: self-care. American Academy of Dermatology. Accessed August 15, 2022. https://www.aad.org/public/diseases/a-z/acne-keloidalis-nuchae-self-care?utm_campaign=DW+Academy+Insider.