Acne Keloidalis Nuchae

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Acne keloidalis nuchae (AKN) is a condition characterized by follicular-based papules and pustules that form hypertrophic or keloid-like scars. AKN typically occurs on the occipital scalp and posterior neck and develops almost exclusively in young, African-American men. ^[1] The term acne keloidalis nuchae is somewhat of a misnomer because the lesions do not occur as a result of acne vulgaris, but rather a folliculitis. Moreover, histologically lesions are not keloidal. ^[2]

Acne keloidalis nuchae was first recognized as a discrete entity in the late 1800s. Hebra was the first to describe and document this condition in 1860, under the name sycosis framboesiformis. Subsequently in 1869, Kaposi described this same condition as dermatitis papillaris capillitii. ^[3] The term acne keloidalis was then given to this condition in 1872 by Bazin, and, since that time, this is the name most often used in the literature. ^[2]

Lesions initially manifest as mildly pruritic follicular-based papules and pustules on the nape of the neck. Chronic folliculitis ultimately leads to development of keloid-like plaques. AKN develops in hair bearing skin areas, and broken hair shafts, tufted hairs, and ingrown hairs can be identified within and at the margins of the plaques themselves. Lesions can grow over time and become disfiguring and painful. In advanced cases, abscesses and sinus tracts with purulent discharge may develop. Unlike true acne vulgaris, comedones are not a common feature of AKN.

The exact etiology of acne keloidalis nuchae (AKN) is unclear. It is thought that chronic irritation from coarse, curly hairs in the skin leads to inflammation and development of these lesions. This hypothesis is supported by the fact that close shaving and chronic rubbing of the area by clothing or athletic gear make AKN worse. In a study of 453 high school, college, and professional American football players, 13.6% of African American athletes had acne keloidalis nuchae, as opposed to none of the Caucasian athletes. ^[4] It has also been shown that men who have haircuts more frequently than once a month are at higher risk of developing acne keloidalis nuchae. ^[5]

Pseudofolliculitis barbae (PFB) is a similar condition that occurs commonly in African Americans. In PFB, it has been proposed that close shaving of coarse, curved hairs facilitates the reentry of the free end of the hair into the skin (via either extrafollicular or transfollicular penetration), which then invokes a chronic foreign-body inflammatory response.

While ingrowing hairs may account for small papules, they do not sufficiently explain the progressive scarring alopecia that occurs in some patients. These patients with scarring alopecia often exhibit recurrent crops of small pustules and may have a condition akin to folliculitis decalvans. Chronic low-grade bacterial infection, autoimmunity, and some types of medication (eg, cyclosporine, diphenylhydantoin, carbamazepine) have also been implicated in the pathogenesis in some patients. ^{[6, 7]}

Sperling et al classify acne keloidalis nuchae as a primary form of inflammatory scarring alopecia and suggest that overgrowth of microorganisms does not play an essential role in the pathogenesis of AKN. They also found no association between pseudofolliculitis barbae and acne keloidalis nuchae. ^[8]

After extensive histological and ultrastructural studies of AKN lesions, Herzberg et al proposed that a series of events must happen in order for acne keloidalis nuchae to occur, namely the following ^[9] :

The initial process begins as acute perifollicular inflammation followed by weakening of the follicular wall at the level of the lower infundibulum, the isthmus, or both.

The naked hair shaft is then released into the surrounding dermis, which acts as a foreign-body and incites further acute and chronic granulomatous inflammation. This process is clinically manifested by small follicular-based papules and pustules. The nape of the neck has almost twice the number of mast cells compared with the anterior scalp and therefore may contribute to the pruritic sensation in this location. ^[10]

Subsequently, fibroblasts deposit new collagen and fibrosis ensues.

Distortion and occlusion of the follicular lumen by the fibrosis results in retention of the hair shaft in the inferior aspect of the follicle, thereby perpetuating the granulomatous inflammation and scarring. This stage is marked by plaques of hypertrophic scar and irreversible alopecia. 

Suggested etiologies include the following:

Close shaving of the neck: This often exacerbates the condition. The sharp, curved hairs reenter the skin and invoke an acute inflammatory response.

Constant irritation from shirt collars or athletic gear: This irritation causes shearing of the hairs.

Chronic low-grade bacterial infections

An autoimmune process

Use of antiepileptic drugs or cyclosporine

An increased number of mast cells in the occipital region ^[11]

Reports have linked acne keloidalis nuchae (AKN) with keratosis follicularis spinulosa decalvans, a rare X-linked disorder in which individuals have a genetic predisposition toward follicular hyperkeratosis and subsequent inflammation. ^{[12, 13]}

A genetic risk factor has been identified in a subset of men with PFB. A substitution mutation in the 1 A alpha-helical segment of the hair-follicle-specific keratin 75 (formerly K6hf) was found in 36% of men with PFB compared with 9% of controls. This single-nucleotide polymorphism predisposes patients for PFB since it is associated with structural weakness of the hair follicle. ^[10]

Acne keloidalis nuchae (AKN) is said to represent 0.45% of all dermatoses affecting black persons. ^[14]

Acne keloidalis nuchae is most prevalent in African Americans; however, it has occasionally been reported in Hispanics and Asians, and, rarely, in whites. 

Although early literature inferred that acne keloidalis nuchae only affects males, it is now known to occur in females, with a male-to-female ratio of approximately 20:1. ^{[10, 15]}

Most cases occur in persons aged 14-25 years. Lesions manifesting prior to puberty or in persons older than 50 years is unusual. ^[8]

The prognosis is good if acne keloidalis nuchae (AKN) is treated early and properly. However, once major scarring develops, therapy is more difficult and morbidity is increased. The plaques of acne keloidalis nuchae slowly expand over time, and, although medically benign, acne keloidalis nuchae can be a psychologically devastating condition. Chronic pruritus and drainage may occur, and, ultimately, scarring alopecia may ensue.

Educate patients on the postulated underlying causes of acne keloidalis nuchae (AKN). Advise patients to discontinue wearing possible offending garments.Instruct patients to tell their barbers not to shave the posterior part of their hairline. Counsel patients about scalp folliculitis and how to care for it.

Dinehart SM, Herzberg AJ, Kerns BJ, Pollack SV. Acne keloidalis: a review. J Dermatol Surg Oncol. 1989 Jun. 15(6):642-7. [Medline].

Gloster HM Jr. The surgical management of extensive cases of acne keloidalis nuchae. Arch Dermatol. 2000 Nov. 136(11):1376-9. [Medline].

Adamson HG. Dermatitis papillaris capillittii (Kaposi). Acne keloid. Br J Dermatol. 1914. 26:69-83.

Knable AL Jr, Hanke CW, Gonin R. Prevalence of acne keloidalis nuchae in football players. J Am Acad Dermatol. 1997 Oct. 37(4):570-4. [Medline].

Khumalo NP, Jessop S, Gumedze F, Ehrlich R. Hairdressing and the prevalence of scalp disease in African adults. Br J Dermatol. 2007 Nov. 157(5):981-8. [Medline].

Grunwald MH, Ben-Dor D, Livni E, Halevy S. Acne keloidalis-like lesions on the scalp associated with antiepileptic drugs. Int J Dermatol. 1990 Oct. 29(8):559-61. [Medline].

Wu WY, Otberg N, McElwee KJ, Shapiro J. Diagnosis and management of primary cicatricial alopecia: part II. Skinmed. 2008 Mar-Apr. 7(2):78-83. [Medline].

Sperling LC, Homoky C, Pratt L, Sau P. Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol. 2000 Apr. 136(4):479-84. [Medline].

Herzberg AJ, Dinehart SM, Kerns BJ, Pollack SV. Acne keloidalis. Transverse microscopy, immunohistochemistry, and electron microscopy. Am J Dermatopathol. 1990 Apr. 12(2):109-21. [Medline].

Alexis A, Heath CR, Halder RM. Folliculitis keloidalis nuchae and pseudofolliculitis barbae: are prevention and effective treatment within reach?. Dermatol Clin. 2014 Apr. 32 (2):183-91. [Medline].

George AO, Akanji AO, Nduka EU, Olasode JB, Odusan O. Clinical, biochemical and morphologic features of acne keloidalis in a black population. Int J Dermatol. 1993 Oct. 32(10):714-6. [Medline].

Goh MS, Magee J, Chong AH. Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae. Australas J Dermatol. 2005 Nov. 46(4):257-60. [Medline].

Janjua SA, Iftikhar N, Pastar Z, Hosler GA. Keratosis follicularis spinulosa decalvans associated with acne keloidalis nuchae and tufted hair folliculitis. Am J Clin Dermatol. 2008. 9(2):137-40. [Medline].

Kundu RV, Patterson S. Dermatologic conditions in skin of color: part II. Disorders occurring predominately in skin of color. Am Fam Physician. 2013 Jun 15. 87(12):859-65. [Medline].

Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. 2003 Oct. 21(4):645-53. [Medline].

Luz Ramos M, Munoz-Perez MA, Pons A, Ortega M, Camacho F. Acne keloidalis nuchae and tufted hair folliculitis. Dermatology. 1997. 194(1):71-3. [Medline].

Adegbidi H, Atadokpede F, do Ango-Padonou F, Yedomon H. Keloid acne of the neck: epidemiological studies over 10 years. Int J Dermatol. 2005 Oct. 44 Suppl 1:49-50. [Medline].

Quarles FN, Brody H, Badreshia S, et al. Acne keloidalis nuchae. Dermatol Ther. 2007 May-Jun. 20(3):128-32. [Medline].

Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. 2003 Oct. 21 (4):645-53. [Medline].

Okoye GA, Rainer BM, Leung SG, Suh HS, Kim JH, Nelson AM, et al. Improving acne keloidalis nuchae with targeted ultraviolet B treatment: a prospective, randomized, split-scalp comparison study. Br J Dermatol. 2014 Nov. 171 (5):1156-63. [Medline].

Layton AM, Yip J, Cunliffe WJ. A comparison of intralesional triamcinolone and cryosurgery in the treatment of acne keloids. Br J Dermatol. 1994 Apr. 130(4):498-501. [Medline].

Millán-Cayetano JF, Repiso-Jiménez JB, Del Boz J, de Troya-Martín M. Refractory acne keloidalis nuchae treated with radiotherapy. Australas J Dermatol. 2015 Jul 29. [Medline].

Kantor GR, Ratz JL, Wheeland RG. Treatment of acne keloidalis nuchae with carbon dioxide laser. J Am Acad Dermatol. 1986 Feb. 14(2 Pt 1):263-7. [Medline].

Shah GK. Efficacy of diode laser for treating acne keloidalis nuchae. Indian J Dermatol Venereol Leprol. 2005 Jan-Feb. 71(1):31-4. [Medline].

Esmat SM, Abdel Hay RM, Abu Zeid OM, Hosni HN. The efficacy of laser-assisted hair removal in the treatment of acne keloidalis nuchae; a pilot study. Eur J Dermatol. 2012 Sep-Oct. 22(5):645-50. [Medline].

Califano J, Miller S, Frodel J. Treatment of occipital acne keloidalis by excision followed by secondary intention healing. Arch Facial Plast Surg. 1999 Oct-Dec. 1(4):308-11. [Medline].

Glenn MJ, Bennett RG, Kelly AP. Acne keloidalis nuchae: treatment with excision and second-intention healing. J Am Acad Dermatol. 1995 Aug. 33(2 Pt 1):243-6. [Medline].

Bajaj V, Langtry JA. Surgical excision of acne keloidalis nuchae with secondary intention healing. Clin Exp Dermatol. 2008 Jan. 33(1):53-5. [Medline].

Elizabeth K Satter, MD, MPH Dermatologist and Dermatopathologist

Elizabeth K Satter, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Medical Womens Association

Disclosure: Nothing to disclose.

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

John G Albertini, MD Private Practice, The Skin Surgery Center; Clinical Associate Professor (Volunteer), Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine; President-Elect, American College of Mohs Surgery

John G Albertini, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: QualDerm Partners; Novascan<br/>Have a 5% or greater equity interest in: QualDerm Partners – North Carolina.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.

Joshua A Zeichner, MD Assistant Professor, Director of Cosmetic and Clinical Research, Mount Sinai School of Medicine; Chief of Dermatology, Institute for Family Health at North General

Joshua A Zeichner, MD is a member of the following medical societies: American Academy of Dermatology, National Psoriasis Foundation

Disclosure: Received consulting fee from Valeant for consulting; Received grant/research funds from Medicis for other; Received consulting fee from Galderma for consulting; Received consulting fee from Promius for consulting; Received consulting fee from Pharmaderm for consulting; Received consulting fee from Onset for consulting.

A Paul Kelly, MD Chief, Clinical Professor, Department of Internal Medicine, Division of Dermatology, King/Drew Medical Center, Charles Drew University of Medicine and Science

A Paul Kelly, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society for Dermatologic Surgery, National Medical Association, and Pacific Dermatologic Association

Disclosure: Nothing to disclose.

Philip R Letada, MD Dermatologist, Associates in Dermatology, Hampton, VA

Philip R Letada, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Association of Military Dermatologists

Disclosure: Nothing to disclose.

Acne Keloidalis Nuchae

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