Acne Keloidalis Nuchae Clinical Presentation
- Author: Elizabeth K Satter, MD, MPH; Chief Editor: William D James, MD more...
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.
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.
Ultimately, keloidlike plaques arranged in a bandlike distribution at or below the posterior part of the hairline can be identified. See the image below.
The plaques are usually only a few centimeters in diameter, but they can be greater than 10 cm in diameter. See the image below.
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.
Abscesses with draining sinuses also may be present.
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
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.
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