Acne Keloidalis Nuchae Clinical Presentation

  • Author: Philip R Letada, MD; Chief Editor: William D James, MD   more...
 
Updated: Aug 5, 2011
 

History

Importantly, note the duration of acne keloidalis nuchae (AKN), the duration of the acute flare, past therapeutic successes and failures, present medications, hair grooming techniques, and any known allergies. Regardless of symptomology, in general the lesions are cosmetically bothersome.

Early papular lesions are usually asymptomatic, but pustular lesions are often pruritic and occasionally painful. Large lesions can be painful. Abscesses and sinuses may be present and may emit purulent, malodorous discharge. Hats, shirts, jackets, and sweaters can irritate the involved area.

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Physical

Early lesions manifest as firm, dome-shaped, follicular-based papules that are 2-4 mm in diameter. The papules are predominately located on the occipital region and nape of the neck. Pustules may be present, but often only excoriated papules can be identified because the lesions are often pruritic or they become traumatized when the hair is groomed.

As the disease progresses, more papules and pustules appear and, over time, can coalesce to form larger plaques.

Numerous acne keloidalis papules and plaques in a Numerous acne keloidalis papules and plaques in a white man with straight hair.

Ultimately, keloidlike plaques arranged in a bandlike distribution at or below the posterior part of the hairline can be identified.

A large acne keloidalis plaque in a bandlike distrA large acne keloidalis plaque in a bandlike distribution at the posterior occiput in an African American man.

The plaques are usually only a few centimeters in diameter, but they can be greater than 10 cm in diameter.

A large acne keloidalis plaque on the occipital reA large acne keloidalis plaque on the occipital region in an African American patient. This man also had perifolliculitis of the scalp and acne conglobata (the follicular occlusion triad).

Scarring alopecia eventually ensues. In chronic lesions, broken or tufted (“doll-like”) hairs may be seen within or at the periphery of the plaque.

Numerous papules that have coalesced into a large Numerous papules that have coalesced into a large plaque, within which are tufts of hairs with several hair shafts exiting the same follicular orifice.

Abscesses with draining sinuses also may be present.

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Causes

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 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]

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Contributor Information and Disclosures
Author

Philip R Letada, MD  Resident Physician, Department of Dermatology, Naval Medical Center San Diego

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.

Coauthor(s)

Elizabeth Kline Satter, MD, MPH  Chairman, Department of Dermatology, Naval Medical Center San Diego

Elizabeth Kline Satter, MD, MPH is a member of the following medical societies: Alpha Omega Alpha and American Medical Women's Association

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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

Disclosure: Nothing to disclose.

John G Albertini, MD  Consulting Staff, Dermatologic Surgery, The Skin Surgery Center; Program Director, ACGME Accredited Fellowship in Procedural Dermatology

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

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

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

Disclosure: Elsevier Royalty Other

Acknowledgments

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, A. Paul Kelly, MD, to the development and writing of this article.

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

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

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

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

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

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

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

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

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

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

  11. 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. Oct 1993;32(10):714-6. [Medline].

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

  13. 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].

  14. 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].

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

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

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

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

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

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

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

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

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Numerous acne keloidalis papules and plaques in a white man with straight hair.
A large acne keloidalis plaque in a bandlike distribution at the posterior occiput in an African American man.
A large acne keloidalis plaque on the occipital region in an African American patient. This man also had perifolliculitis of the scalp and acne conglobata (the follicular occlusion triad).
Numerous papules that have coalesced into a large plaque, within which are tufts of hairs with several hair shafts exiting the same follicular orifice.
A dense plasma cell infiltrate surrounding a hair follicle.
Naked hair shafts embedded within a fibrotic dermis.
 
 
 
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