Acne Keloidalis Nuchae

Acne keloidalis (also spelled "cheloidalis") nuchae is an unusual skin condition affecting the nape of the neck. It commonly affects adult Afro-Caribbean males with curly hair, but it can occur in any individual. The condition may persist for many years. Acne Keloidalis Nuchae, or hair bumps, arises at the juncture of the scalp and the back of the neck (nape). Often seen in African American men, this problem can arise when hair, at the nape of the neck is cut or shaved quite short, typically less than 1/4 inch in length. In curly haired individuals, the hair is able to corkscrew back into the hair follicle where it causes irritation, much like an acne lesion. In the occasional unfortunate person prone to developing keloids, (firm raised scar tissue), this inflamed bump turns into a permanent ball of scar tissue. In severe cases, hair can be permanently lost in areas of widespread inflammation. Treatment is best aimed at preventing the ingrown hair in the first place. And if the ingrown hair does arise, it is equally important to prevent the development of inflammation.

Initially, itchy round small bumps appear within or close to the hair-bearing area of the back of the neck (occipital scalp). Sometimes there are pustules around the hair follicles (folliculitis). As time goes on the bumps become small scars and then the small scars may greatly enlarge to become keloids. The scars are hairless and can form a band along the hairline.

The cause of acne keloidalis nuchae is not known. It may be a form of dermatitis or a form of acne. In most cases there are ingrown hairs, which irritate the wall of the hair follicle resulting in inflammation.

Frequency In the US: ACNE KELOIDALIS is said to represent 0.45% of all dermatoses affecting black persons.

Mortality/Morbidity: Acne keloidalis is a medically benign but often psychologically devastating dermatosis. Squamous cell carcinoma has developed secondary to radiation therapy in rare cases. Chronic pruritus, scarring, and drainage may occur. Without therapy, the lesions may continue to enlarge and new ones may appear. Scarring alopecia of the involved scalp is a common finding.

Race: Acne keloidalis most often occurs in African Americans. Hispanics are the next most common group, followed by Asians and (least often) whites.

Sex: While the early literature inferred that ACNE KELOIDALIS occurred only in males, it is now known to occur in females. The male-to-female ratio is approximately 20:1.

Age: Onset usually occurs in early adulthood, but some cases do develop during adolescence. Onset prior to puberty or after the 50 years of age is extremely rare.

History: Early papular lesions usually are asymptomatic, but pustular lesions often are itchy and occasionally may be painful.

*Large lesions often are painful.

*Older lesions with abscesses and sinuses may emit an odorous discharge.

*Hats, shirts, jackets, and sweaters may irritate the involved area, thereby causing patients to alter preferred style of dress.

*Even if large lesions are asymptomatic, they often are a cause of great cosmetic concern.

Background: Acne keloidalis (ACNE KELOIDALIS) refers to the occurrence of keloid-like papules and plaques on the occipital scalp and posterior neck, almost exclusively in African-American men. These patients initially usually develop a chronic folliculitis and perifolliculitis of the occipital scalp and posterior neck, which heal with keloid-like lesions, sometimes with discharging sinuses. They often coalesce to form one or several large plaques, which gradually enlarge for years. The lesions often are painful and cosmetically disfiguring.

Kaposi first described acne keloidalis in 1869 as dermatitis papillaris capillitii. The disease previously had been known in Hebra’s clinic and is described and pictured in Hebra’s atlas under the name “sycosis framboesiformis.” Three years after Kaposi’s publication, Bazin named the condition "acne keloidalis." Since then, it has appeared in the literature under a variety of names.

Little can be added clinically to Adamson's description of ACNE KELOIDALIS in 1914. "The eruption occurs upon the back of the neck in the form of a raised transverse band at the lower margin of the hairy scalp. The band is usually dusky red in color, smooth and firm to the touch in fact, of keloidal aspect and consistence. It is hairless except at its upper margin, which is abrupt, broken into nodules and fringed with hair in tufts, like aigrettes, or the bunches of bristles in a brush. There may be pustules or crusted nodules here and there along the upper border. The lower margin slopes gradually to the normal skin. Usually there are no comedones or follicular pustules of acne when the patient comes under observation, and there may or may not be a history of acne of the face in youth. Often the patient complains of itching at the site of the eruption."

Cause: The exact cause of ACNE KELOIDALIS still is speculative. Injury produced by short haircuts (especially when the posterior hairline is shaved with a razor, a practice common in African American men) and curved hair follicles (analogous to pseudofolliculitis of the beard in African Americans) may be the precipitating factors. Other frequently suggested etiologic possibilities are constant irritation from shirt collars, chronic low-grade bacterial infections, and an autoimmune process (ACNE KELOIDALIS usually responds to systemic steroid therapy). The use of antiepileptic drugs and an increased number of mast cells in the occipital region also have been incriminated.

Sperling et al's recent findings indicate that ACNE KELOIDALIS is a primary form of scarring alopecia. Many of the histologic findings closely resemble those found in certain other forms of scarring alopecia. They claim that overgrowth of microorganisms does not play an important role in the pathogenesis of ACNE KELOIDALIS. They also found no association between pseudofolliculitis barbae and ACNE KELOIDALIS.

Herzberg et al provided another explanation based on extensive transverse microscopy, histochemistry, and electron microscopy. Herzberg et al described the following hypothetical sequence of inflammatory events that take place in acne keloidalis:

"The acute inflammation, whether it begins in the sebaceous gland or elsewhere in the region of the deep infundibular or isthmus levels, is a cause or the result of a weakened follicular wall at these levels. This enables the release of hair shafts into the surrounding dermis. The "foreign" hairs incite further acute and chronic granulomatous inflammation. The localized granulomatous inflammation manifests itself clinically as a papular lesion. Fibroblasts lay down collagen and scars form in the region of the inflammation. Distortion and occlusion of the follicular lumen by fibrosis leads to hair retention in the inferior follicle and further smoldering granulomatous inflammation and scarring. The scarring and granulomatous inflammation manifest themselves clinically as keloid-like scars and plaques."

They also found that the follicular lymphocytic infiltrate contained a mixed B- and T-cell population and that the plasma cell immunoglobulins are of a polyclonal nature.

Thus, the exact cause of ACNE KELOIDALIS still is speculative. Injury produced by short haircuts (especially when the posterior hairline is shaved with a razor, a practice common in African American men) and curved hair follicles (analogous to pseudofolliculitis of the beard in African Americans) may be the precipitating factors. Frequently suggested possibilities include:

*Constant irritation from shirt collars. *Chronic low-grade bacterial infections.

*An autoimmune process (ACNE KELOIDALIS can respond to systemic steroid therapy).

*Use of antiepileptic drugs. *An increased number of mast cells in the occipital region

Physical:

*Acne keloidalis starts after puberty as firm, dome-shaped, follicular papules 2-4 mm in diameter.

*The papules develop on the nape of the neck and/or on the occipital scalp.

*Pustules also may be present in the same areas, but usually are short-lived because the tops are scratched off in response to pruritus or they are traumatized when the patient's hair is combed and/or brushed.

*Comedones are not present (in contradistinction to acne).

*More papules may appear and the papules enlarge as the disease progresses.

Treatment

Unfortunately acne keloidalis often persists despite a variety of treatments. The following measures are sometimes helpful:

Make sure clothing does not rub the back of the neck
Oral antibiotics for secondary infection
Topical steroids
Steroids injected into the lesions (intralesional)
Oral isotretinoin
Radiotherapy
Laser vaporization
Surgery (i.e. surgically removing all the affected skin)

Hair should be kept at least over 1/4 inch in length.

Once the scar has formed, it is very difficult to eradicate it. The use of steroid injections into the larger lesions can help shrink it down, but for very tiny bumps, it is often impossible to get rid of these. If possible, simple camouflage by covering the area with long hair is the best way to hide the condition. The use of steroid impregnated tapes, and steroid ointments can also be used in place of the injections.

For disfiguring cases, excision of the scarred area can be performed by a competent surgeon.

*The first line of defense against acne keloidalis is prevention. People who have this problem should not get their occipital hair line edged with a razor or wear tight fitting shirts or other clothing that will cause mechanical irritation of their posterior hairline. Initiating therapy quickly lowers the patient's chances of developing large lesions.

*Twice a day treatment with a combined retinoid like Retin-A or Tazorac and a corticosteroid gel may be sufficient to relieve all symptomatology and flatten the existing lesions. This mixture seems to be somewhat more effective than steroids alone. . Ask for a prescription if you do not have these medications.

*When pustules, crust formation, or drainage is present, you may use topical clindamycin gel (Clindagel) on a twice-daily basis until the pustules abate and the inflammation subsides. Ask for a prescription if you do not have this medication.

*Once healing has taken place, continue to apply the retinoid steroid mixture to the occipital scalp twice daily to help prevent recurrence.

Surgical Care:

Larger linear lesions may be excised deeply below the hair follicles with primary closure

*The postoperative site often will splay open to the diameter of the initial excision.

*For large lesions that cannot be excised and closed primarily, the area of acne keloidalis can be excised to the fascia or to the deep subcutaneous tissue and left to heal secondarily.

*The postoperative site will be cosmetically unacceptable initially, and the patient will experience pain and discomfort for the first few days.

*Complete wound healing takes 8-12 weeks.

*In 2-3 months you may resume the retinoid steroid mixture to the occipital scalp twice daily to help prevent recurrence.

*A follicular papule or pustule occasionally will develop along the border of the linear scar. Treat all inflammatory lesions with topical clindamycin until the infection subsides.

*Excision with grafting is not as cosmetically acceptable because it results in a large depressed non–hair-bearing area.

*Healed second intention scars are almost imperceptible especially when the occipital hair is allowed to grow long.

*Dr. Jacobs does not give corticosteroid injections prior to complete wound closure.

Deterrence/Prevention:

*Males who play football should make sure their helmets fit properly and do not cause posterior scalp irritation. *Discontinue wearing garments that rub or irritate the posterior scalp and neck.

*Avoid having the posterior hairline shaved with a razor as part of a haircut, especially in African Americans. Discontinue wearing possible offending garments. Tell the barber not to shave the posterior hairline.

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