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Acne Keloidalis Nuchae Clinical Presentation

  • Author: Elizabeth K Satter, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Feb 11, 2016
 

History

Importantly, note the duration of AKN, 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. See the image below.

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. See the image below.

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. See the image below.

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. See the image below.

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[13]

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.[14, 15]

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]

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Contributor Information and Disclosures
Author

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.

Specialty Editor Board

David F Butler, MD 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: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, 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: Received grant/research funds from Genentech for investigator.

Chief Editor

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: Nothing to disclose.

Additional Contributors

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.

Acknowledgements

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.

References
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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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