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Acne Keloidalis Nuchae

Acne Keloidalis Nuchae is a condition where hair follicles are affected by inflammation. In a general sense, this is known as folliculitis. However, AKN is characterized by a distinct set of symptoms, including scarring and the development of elevated bumps on the skin, known as pustules and papules. Papules are similar in nature to pimples.  Pustules are filled with inflammatory discharge.

Acne Keloidalis Nuchae typically manifests on the occipital scalp and the posterior portion of the neck. Over time, the papules merge forming horizontal plaques. Affected areas are hairless with the exception of minimal hair growth surrounding these plaques. The aberrated shafts are usually broken, ingrown and often tufted (“doll-like.”)

In more advanced stages, this abnormal tissue growth (aka lesions) develops into tumor-like masses.

Aside from the cosmetic disfiguration, patients often experience pain (pruitus), itching and the discharge of pus.

Acne Keloidalis Nuchae most commonly occurs in males of African descent, accounting for 0.45% of all dermatosis cases within the black population.  Hispanics and Asians are also affected, although incidence is relatively minimal. In whites, it is extremely rare. The ratio of males to females with AKN is 20:1.

The overall condition is chronic, permanent and resistant to most forms of treatment.

HISTORY

Acne Keloidalis Nuchae was first documented in the 1800’s. It’s scientific nomenclature underwent several evolutions as noted below:

1860- sycosis framboesiformis (Hebra)

1869- dermatitis papillaris capillittii (Kaposi)

1872- acne keloidalis (Bazin)

CAUSES OF ACNE KELOIDALIS NUCHAE

The specific causal factors of AKN remain unclear. However there are some reasonable postulations on why this condition occurs. These include:

Autoimmune reactions

The general logic behind the  etiology of Acne Keloidalis Nuchae is believed to involve the immune system’s inability to tell the difference between native hair and invading foreign pathogens. This propagates an attack  on the hair shaft as well as the follicle and sebaceous glands. The presence of bacteria further exacerbates this inflammatory reaction.

Coarse, wavy hair can grow back towards the skin’s surface and re-enter the dermis instead of growing outward.  This can then prompt an inflammatory response from the immune system, causing the eruption of papules and pustules seen in Acne Keloidalis Nuchae.

The skin treats these events as an injury and attempts to heal itself through a reactive process called fibrosis. New collagen is produced by cells called fibroblasts. This results in distortion and occlusion of the hair follicle. The hair shaft is retained below the skin’s surface, perpetuating the inflammation and scarring.

Neutrophils and lymphocytes are other constituent cells brought forth by the body’s internal defense system. Their actions lead to the destruction of the hair follicle and sebaceous glands. Plaques made of hypertrophic scar tissue eventually develop from this.

Hertzberg et al suggests that environmental factors may contribute to Acne Keloidalis Nuchae.  For example, shaving and irritation from collars and athletic gear can promote breakage among hair shafts. One study reveals that in 90% of patients, the condition started after using an electric razor to cut the hair.

However, other studies found no evidence of the hair growing back into the skin to support this autoimmune hypothesis.

 

Bacterial Infections

 

Bacterial infections are also thought to be contributing factors. Acne Keloidalis Nuchae is believed by some researchers to be a form of scarring alopecia. In this condition, bacteria damage the hair follicles leading to the manifestation of its symptoms, which include itching, burning, the presence of pustules as well as scarring.

Cylosporine

Cyclosporine is a drug that suppresses the immune system. It is typically given to patients who have received an organ transplant. Cyclosporine works to keep the immune system from attacking the new tissue.

Acne Keloidalis Nuchae has been reported to occur in Caucasian patients after taking cyclosporine.

Mast Cells

 

A high concentration of mast cells in the occipital region of the head is thought to be another possible cause.  These are specialized cells of our immune system which attack foreign invaders.

Having a large number of mast cells in the back of the head might cause the skin  to overreact in the event that hair grows back into the epidermis, leading to the symptoms associated with AKN.

ACNE KELOIDALIS NUCHAE TREATMENT

It is important for patients to address symptoms immediately once the lesions appear to help prevent long term disfigurement which is difficult to correct. There are a number of different options depending on the clinical stage of the Acne Keloidalis Nuchae. Examples of treatments for AKN include:

  1. Specialized shampoos which should contain ingredients such as benzoyl peroxide, alpha hydroxy acids or tar to address any existing bacteria as well as itching. This should be administered daily for early stage symptoms.
  2. Topical steroids, which have anti inflammatory properties. For example, Retin-A (retinoic acide) can be combined with a corticosteroid (class 2 or 3) cream for a twice a day treatment. This is also an option for early stage onset of Acne Keloidalis Nuchae.
  3. A topical antibiotic (e.g. clindamycin or erythromycin) can be used when drainage is present or when pustules start to crust.
  4. Oral prednisone, along with systemic antibiotics can be used for instances where large abcesses or drainage is present.  The recommended period is 4-5 days.
  5. Isotretinoin may be an intervention to consider for rapidly progressing manifestations of  symptoms.
  6. Triamcinolone acetonide can be injected into papules affected by Acne Keloidalis Nuchae lesions.  Recommended concentrations can vary from 3-5 mg/mL to 40 mg/mL). Since this may be painful for the patient, a mixture of lidocaine-prilocaine should be administered 2 hours before the triamcinolone acetonide using an occlusion made of plastic film.
  7. Cryotherapy has been effective for certain cases. This intervention involves a series of freezing and thawing to treat affected areas.
  8. Radiation therapy works to completely remove the hair shafts responsible for triggering the problematic symptoms.

Overall, deciding on what treatment to use depends on the clinical stage of AKN. Topical medications and injections can be used for mild or moderate papulopustular lesions. The more advanced stages characterized by plaques and tumors will need to be removed by surgical exicision.

AKN Surgical Excision

 

Acne-Keloidalis-Nuchae-65


 

The tumor and plaque stage of Acne Keloidalis Nuchae can be reliably treated through surgical excision which removes the affected area. The excision must be deep enough to remove the inflamed follicles as well as the keloidal tissue.  Common surgical techniques used include:

  • A combination of excision and grafting
  • Excision using a primary closure technique
  • Excision with secondary intention healing
  • Staged excision with primary closure
  • Electrosurgical excision
  • Excision with trychophytic closure

Using skin grafts to replace the tissue affected by Acne Keloidalis Nuchae tumors and plaques is a technique that is viable in theory. However, according to Beckett, Lawson and Cohen et al (2011) the outcome produces poor cosmetic results since the new skin usually does not match the surrounding areas.

Excision with primary closure is a technique where the affected area is surgically cut out, usually in an ellipse shape. The two sides of the excised skin are then stitched together. However, this method will leave a very large, prominent scar.

Excision with secondary healing allows the surgical wound to heal for a period of about 6-10 weeks following the excision of the Acne Keloidalis Nuchae tissue mass. The scar is flatter and much smaller.

The surgical excision can also be closed off in stages. This is referred to as staged excision with primary closure. The purpose of this technique is to help the patient’s head move more freely. This movement can be restricted if the excised area is stitched immediately after removing the affected area.

Electrosurgical excision uses an electrified wire to remove the specific areas of tissue affected by advanced stages of Acne Keloidalis Nuchae. The resulting wound is allowed to heal on its own by applying gauze and petroleum. Patients are also given antibiotics during this recovery time to help prevent infections. The benefit of this technique is reduced levels of bleeding. The heat causes blood to develop  into a solid state, a process known as hemostasis. And there is no need to tie off any blood vessels.

Excisions with trychophytic closure can reposition specific lines of hair growth close to the resulting scar. As the hair grows, this line becomes far less visible.

Case Study of Acne Keloidalis Nuchae Excision with Trychophytic Closure

 

Dr. Umar a board certified surgical dermatologist,  combines his background in hair transplant surgery with his expertise on Acne Keloidalis Nuchae to actually cure the condition in selected individuals using a variety of surgical techniques including:

  • posterior hairline migration
  • serial excisions
  • trichophytic closure

The trychophytic closure technique is illustrated in the following case study video.

Steroid injections did not work for this particular patient who has suffered from Acne Keloidalis Nuchae for eight years. Dr. Umar was able to excise this area of tissue and strategically promote the  growth of hair over the scar.

The results are illustrated through the before and after pictures in this video.

Here is a second case study of a patient treated with surgical excision and trychophytic closure to remove the tumor like mass. This patient had Acne Keloidalis Nuchae for 6 years and was taking prescription oral medication for six months without experiencing any results. Dr. Umar managed to reposition and lower the posterior hair line of this patient so that the scar would eventually be covered by his hair.

Acne Keloidalis Nuchae can occur in different forms of severity depending on how long symptoms have persisted in the absence of any form of treatment. Fortunately, there are different forms of treatment associated with different stages. Surgical excision can offer much hope, especially for more advanced cases of AKN. However it is nonetheless important to consult with a medical practitioner to determine the most ideal direction to take for individual cases.

Read more about a dermatologist who can help with your AKN.

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