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Acne keloidalis nuchae - UpToDate

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All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Apr 2020. | This topic last updated: Sep 27, 2018.

INTRODUCTION Acne keloidalis nuchae (AKN) is a common chronic

disorder involving inflammation and scarring of the hair follicles with subsequent development of
keloid-like papules and plaques and scarring alopecia. The characteristic site of involvement is the
posterior scalp and neck. Infrequently, other areas of the scalp are affected.

AKN typically occurs in males of African ancestry, but also occasionally develops in females and in
non-African ethnic groups. AKN may be pruritic, painful, or cosmetically disfiguring, contributing to
negative effects on quality of life.

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The clinical features, diagnosis, and management of AKN will be reviewed here. Other hair and scalp
disorders are reviewed separately. (See "Evaluation and diagnosis of hair loss".)

TERMINOLOGY Although the term "acne keloidalis nuchae" is commonly

used, there are other historical and contemporary descriptors for AKN. Historical terms for this
condition include "sycosis framboesiformis" and "dermatitis papillaris capillitii" [1,2]. Contemporary
alternative names for AKN include "folliculitis keloidalis" [3], "folliculitis nuchae" [4], and "folliculitis
keloidalis nuchae" [5]. In addition, because of the occasional extension to other areas of the scalp,
some authors eliminate "nuchae" from AKN and refer to the disorder as "acne keloidalis."

Multiple authors have suggested that the name "acne keloidalis nuchae" is a misnomer, since the
lesions are neither pathologically acne nor keloids [6]. In contrast to true acne, comedones are never
present in AKN [7]. The scar-like lesions, though they resemble keloids, do not typically recur
following excision like keloids often do [8], and patients typically do not have keloids elsewhere.

EPIDEMIOLOGY AKN is a common disorder. People of African ancestry

with Afro-textured hair are the predominant population at risk. This predilection was evident in a
study of 453 male football players (aged 14 to 27 years); AKN was present in 14 percent of black
players compared with 0 percent of white players [9]. Moreover, studies performed in North America,
the Caribbean, and Africa have found AKN to represent between 0.45 and 9 percent of diagnoses
among black patients evaluated in dermatology clinics [6,10-14].

AKN affects males far more frequently than females, although females occasionally develop AKN
[15,16]. A 20:1 ratio of affected men to women is commonly quoted [17]. In a community selected
group in South Africa, 11 percent of 267 males versus 0.3 percent of 597 females had AKN [18].

Onset of disease typically follows adolescence and is rare after age 50 [19]. The predilection for the
onset of AKN after adolescence was demonstrated in a study of 1042 South African school children
(age 6 to 21 years). The prevalence of AKN was 0.67 percent overall, but among students in the final
year of high school, the prevalence of AKN was 4.7 percent [18].

PATHOGENESIS As evidenced by a multitude of proposed theories with

variable levels of evidence, the cause of AKN has not been definitively established. Pathogenic
mechanisms related to skin injury and aberrant immune responses to common antigens are among
the cited theories.

●Skin injury – Some theories of causality focus on chronic irritation or occlusion of the follicles due
to hair cutting practices (eg, close shaves or close clipping), trauma, friction (eg, rubbing from shirt
collars or helmets), heat, or humidity as a predisposing or exacerbating factor [11,20-23]. Whether
the tool used for hair cutting might influence risk for AKN is unclear; in a South African study, the

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prevalence of AKN did not appear to differ with the use of razors versus clippers [18]. Frequent and
traumatic haircuts have also been proposed as potential contributors, though further study is
necessary to determine whether these are relevant factors [10,18].

External insults are unlikely to be the sole causes of AKN, given the disproportionate number of
cases that occur in men of African descent. The findings of a study of 453 male football players
supports this; in contrast to the increased prevalence of AKN noted in black players, acne
mechanica (an acneiform eruption induced by friction, occlusion, and heat [24]) occurred on the
posterior neck at similar rates in black and white players [9]. A role for the Afro-textured hair type in
AKN has been suggested based upon the increased prevalence in the black population, possibly
related to the curvature and morphology of Afro-textured hair. Although penetration of curved hair
into the skin has been proposed as a potential mechanism [17], this theory has been questioned by
authors who have found no clinical or pathologic evidence of hairs curving and re-entering the skin
[25,26].

●Aberrant immune reaction – Another proposed pathogenic theory is based upon consideration of
AKN as a form of primary cicatricial (scarring) alopecia stimulated by an immune response in the
skin [27]. (See "Evaluation and diagnosis of hair loss", section on 'Cicatricial alopecia'.)

The following sequence of events for the development of AKN as a primary scarring alopecia has
been proposed [27]:

•Intrafollicular antigens (particularly those that are more prevalent after puberty) attract
inflammatory cells to the follicle at the level of the isthmus (figure 1). Proposed antigens include
Demodex, skin flora (eg, bacteria or fungal spores), cosmetics, sebum, and desquamated
keratinocytes.

•The sebaceous gland is either an early target for inflammation or is destroyed secondarily as a
result of the inflammatory process.

•The follicular wall is weakened by the perifollicular inflammation, resulting in spongiosis and a mild
lymphocytic exocytosis.

•The weakened follicular wall allows increased leakage of antigens, resulting in greater
inflammation.

•The process continues with development of concentric lamellar fibroplasia, with the hair shaft
eventually penetrating the follicular wall and entering the dermis, resulting in intense inflammation
and epithelial destruction, furthermore resulting in a follicular scar.

•Hypertrophic scarring develops due to the presence of hair shaft fragments and degenerating
epithelial components in the dermis.

●Other – Other potential contributory factors proposed based upon limited data include
autoimmunity [25], excess androgens or increased sensitivity to androgens [13,28], seborrhea [13],
and medications (eg, cyclosporine [29-31], tacrolimus [32], sirolimus [32], or diphenylhydantoin and

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carbamazepine [33]). As with other proposed contributors, a role for these factors remains to be
confirmed. AKN has also been reported in two patients with keratosis follicularis spinulosa
decalvans, an X-linked genetic disorder that predisposes patients to the development of follicular
hyperkeratosis and inflammation [34,35]. (See "Keratosis pilaris atrophicans", section on 'Keratosis
follicularis spinulosa decalvans'.)

CLINICAL MANIFESTATIONS AKN presents with

variable degrees of inflammation and keloid-like (keloidal) scarring on the posterior lower scalp and
nape of the neck. Occasionally, involvement extends onto the vertex or other areas of the scalp
(picture 1) [36].

Active inflammation in AKN manifests as inflamed papules or pustules (picture 2A-G). Inflammation
may be acute, demonstrating marked erythema, inflamed papules, or papulopustules. Alternatively, a
combination of acute inflammation and chronic inflammation (manifesting as pink to dusky red
papules without pustules or discharge) may be present. The degree of inflammation ranges from
mild to severe and can vary to some extent between episodes of flare in a given individual. Crusting
or excoriation may be evident. In severe cases, draining sinus tracts or suppuration may be present.

Scarring is typically present by the time patients present for medical evaluation. Scarring is
manifested by variably sized keloidal papules, plaques, or nodules at the site of previously involved
hair follicles (picture 2G-K). The keloidal papules of AKN are typically dome-shaped (picture 3). In the
later stages of AKN, patients may present with chronic scarring or scarring alopecia without active
inflammation (picture 1). Tufted "doll hairs," multiple hairs emerging from a single follicular orifice,
may be present (picture 2K).

AKN may be asymptomatic or symptomatic. Pruritus or pain may accompany the skin lesions,
particularly if pustules are present [6,17,20,25].

CLINICAL COURSE AKN is a chronic disease with episodic flares of

worsening inflammation. Effective treatment typically requires a maintenance regimen with
therapeutic adjustments as needed for flares. With time (usually over a variable number of years),
the disease tends to become less active in terms of inflammation. However, keloidal papules and
plaques usually persist unless treated.

HISTOPATHOLOGY Histopathologic findings vary based on the

stage of the disease and the specific clinical features present in the immediate area of the biopsy.
Typically, however, AKN is characterized by the presence of acute and chronic folliculitis, ruptured
pilosebaceous units, and dense dermal (cicatricial-type) fibrosis (picture 4A-E).

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●Early stages – Dense follicular and perifollicular inflammatory infiltrate of lymphocytes and
neutrophils. The dermal fibrosis resembles the collagen fibers seen in typical scar tissue more than
the fibrosis seen in keloid scars [17].

●Later stages – Disrupted and broken hair follicles are surrounded by granulomatous inflammation,
perifollicular abscess formation, and dermal fibrosis [37].

DIAGNOSIS The diagnosis of AKN can usually be made based upon the clinical
findings. Biopsy is rarely necessary. The clinical findings that raise suspicion for a diagnosis of AKN
are firm keloidal papules or plaques on the lower posterior scalp or upper posterior neck, with or
without inflamed follicular papules or papulopustules.

●Patient history – The patient history allows the clinician to collect information that may aid in
supporting or negating a diagnosis of AKN, determining the best approach to management, and
assessing the response to treatment. Knowledge of the following is useful:

•Age of onset (post-adolescence to middle-age is typical for AKN)

•Disease duration and rate of progression

•Symptoms (eg, pain, pruritus, drainage)

•Hair care practices (hair cutting and shaving)

•Exacerbating factors

•Prior treatments (including response to each treatment)

●Physical examination – The entire scalp should be examined. In typical acne keloidalis nuchae, the
area of disease (inflammatory papules, pustules, and keloidal papules or plaques) is limited to the
lower posterior scalp and nape of the neck. Crusting, excoriation, and in severe cases, suppuration,
may be present.

Although AKN occasionally extends to other areas of the scalp [36], findings of follicular papules,
inflammation, suppuration, or scarring on other areas of the scalp should prompt consideration of
alternative diagnoses, such as dissecting cellulitis of the scalp, lichen planopilaris, follicular
cutaneous T cell lymphoma, tinea capitis, or another disorder. (See 'Differential diagnosis' below.)

●Biopsy – Biopsy is usually unnecessary unless the presentation is atypical. If a biopsy is necessary
for diagnosis, a 3 to 4 mm punch biopsy should be performed. Vertical sectioning of the pathology
specimen is usually sufficient for recognizing pathologic characteristics of AKN. If the presentation
is atypical or there are overlapping features suggestive of an alternate diagnosis, two biopsies may
be performed to minimize the risk of misdiagnosis due to sampling error.

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It is essential that the biopsy be of sufficient depth to include the base of the follicle. It is also
important to include a suspected keloidal papule. Biopsy solely of areas of severe inflammation or
frank suppuration in the absence of keloidal papules may not be helpful.

●Other tests – If superinfection is a possibility (extensive erythema, suppuration, pain, drainage,

odor, or systemic symptoms such as fever), bacterial culture should be obtained. The need for other
tests is determined by the differential diagnosis. For example, if tinea capitis is suspected, a
potassium hydroxide (KOH) preparation or fungal culture is indicated. (See 'Differential diagnosis'
below.)

DIFFERENTIAL DIAGNOSIS The diagnosis of AKN is

usually straightforward, based on the clinical appearance and location of lesions. However, there are
several disorders that may merit consideration if faced with an atypical presentation of AKN.

●Acne mechanica – Acne mechanica is a frictional or traumatic folliculitis. The disorder may
present on the nape of the neck or in any hair-bearing area subject to rubbing or friction. Unlike AKN,
acne mechanica is usually transient and does not present with keloidal papules or plaques. Often, a
specific trigger may be identifiable: eg, shaving, friction, or trauma from clothing or other apparel (eg,
hat or helmet).

●Bacterial folliculitis – Bacterial folliculitis may occur due to bacterial colonization in the setting of
minor trauma (eg, shaving) or follicular occlusion followed by secondary infection. Patients present
with multiple follicle-based inflammatory papules and pustules (picture 5). Unlike AKN, keloidal
papules should not be present.

●Molluscum contagiosum – Molluscum contagiosum presents with skin-colored papules that may
resemble small keloidal papules (picture 6A-B). The nape of the neck is an uncommon location for
molluscum contagiosum; however, infection in this site could occur due to transmission via infected
hair clippers or self-inoculation from other areas of the body. Close examination may help to
differentiate molluscum contagiosum lesions from keloidal papules; molluscum lesions frequently
exhibit central umbilication and are likely to be accompanied by lesions elsewhere on the body.
Inflammation is usually minor or absent, unless molluscum become inflamed or secondarily
infected. Biopsy findings should easily differentiate molluscum from AKN [6]. (See "Molluscum
contagiosum", section on 'Clinical features'.)

●Folliculitis decalvans –Folliculitis decalvans is an uncommon form of cicatricial alopecia that

typically presents with one or more confluent areas of scarring alopecia and multiple pustules that
are often located at the periphery of the areas of alopecia (picture 7A-B). Tufted hairs may be
present. Unlike AKN, multifocal involvement of the scalp is common and keloidal papules and
plaques are not features of this disease. (See "Folliculitis decalvans".)

●Dissecting cellulitis of the scalp – Dissecting cellulitis of the scalp is an uncommon form of
cicatricial alopecia in which patients develop follicular papules, pustules, fluctuant nodules, and
abscesses on the scalp that result in scarring (picture 8A-B). Young men of African descent are most

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frequently affected. Keloidal papules and plaques are not a feature of dissecting cellulitis of the
scalp. (See "Dissecting cellulitis of the scalp".)

●Lichen planopilaris – Lichen planopilaris should be easy to distinguish from AKN. Affected patients
present with fine perifollicular papules with scale and erythema at the base of affected hairs. The
perifollicular papules are smaller than those typically seen in AKN. Macules or patches of alopecia
may be present (picture 9). Pustules should not be present, nor should there be keloidal papules or
plaques. Lichen planopilaris can be located anywhere on the scalp. (See "Lichen planopilaris",
section on 'Clinical presentation'.)

Although a variant of lichen planopilaris, frontal fibrosing alopecia, typically affects the frontal and
temporal hairline with variable extension to the crown and temporoparietal scalp, in lichen
planopilaris the lower posterior scalp would not typically be affected in the absence of lesions
elsewhere on the scalp. (See "Lichen planopilaris", section on 'Frontal fibrosing alopecia'.)

●Follicular cutaneous T cell lymphoma (CTCL) – Follicular CTCL may present with follicular papules
with alopecia, alopecic patches or plaques with hyperkeratotic papules, keratosis pilaris-like lesions,
or comedonal or milia-like lesions. Conventional lesions of mycosis fungoides may or may not be
present [38]. Alternate presentations include papules, plaques, and nodules on the head and trunk
[39]. If follicular CTCL is a realistic concern, biopsy for definitive diagnosis is essential. Multiple
biopsies may be helpful in this clinical scenario. (See "Variants of mycosis fungoides", section on
'Folliculotropic mycosis fungoides'.)

●Tinea capitis – Tinea capitis may present with kerion, an acute inflammatory reaction to tinea
capitis that presents as a localized plaque with erythema, pustules, alopecia, and boggy induration
(picture 10A-B). Cervical lymphadenopathy is usually present. In most cases, the clinical distinction
between AKN and fungal infection should be straightforward. Kerion presents acutely and keloidal
papules are not a feature of tinea capitis. A fungal culture should be obtained if kerion is a
possibility.

MANAGEMENT Treatment data on AKN are limited and there are no

consensus guidelines regarding therapy [40]. Decisions for treatment are usually guided by clinician
experience and modified based upon the clinical presentation and patient preferences. The lack of
consensus is evident in the widely variable treatment suggestions made by a panel of
dermatologists in a review of AKN [4].

Our approach to the treatment of AKN based upon the limited available evidence and clinical
experience is reviewed here. Other approaches to treatment may be reasonable.

Approach to treatment — Our management of AKN consists of measures

directed at improving the clinical manifestations and symptoms of AKN. Our initial interventions
typically include counseling of patients to avoid potential exacerbating factors and the
administration medication to improve active inflammation. Minimizing the appearance of scars may

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be addressed concurrently or following the control of active inflammation. (See 'Avoidance of

exacerbating factors' below and 'Treatment of inflammation' below and 'Treatment of keloidal
scarring' below.)

Avoidance of exacerbating factors — Although the cause of

AKN is not definitively known, a variety of factors have been proposed as exacerbating factors for
the condition (see 'Pathogenesis' above). In an attempt to minimize disease exacerbations, we
encourage patients to [6,17,36,41]:

●Avoid picking, rubbing, or scratching the affected area

●Discontinue close shaving, trimming, or razor- or clipper-edging of the posterior hairline

●Avoid irritation from tight-fitting hats, helmets, or high-collared shirts

However, the efficacy of such measures on AKN has not been studied.

During this discussion, patients often express concern that AKN resulted from contact with infected
barber tools. Therefore, we also educate patients that AKN is not an infectious condition.

Treatment of inflammation — Topical or systemic medications with

antiinflammatory properties represent the primary method of improving inflammatory
manifestations of AKN (inflammatory papules and pustules). The treatment and prevention of
secondary infection may also help to reduce symptoms and minimize exacerbations.

Mild to moderate inflammation — Patients who present with small

inflammatory papules or pustules often can achieve improvement in symptoms with topical therapy.
The antiinflammatory effects of topical corticosteroids are useful for this purpose. We often
combine topical corticosteroid therapy with topical antibacterial therapy or topical retinoid therapy in
an attempt to gain additional benefit.

Topical corticosteroids — Despite the frequent use of topical corticosteroids for AKN,

there are few data on efficacy. Some support stems from an open-label study in which 20 African-
American men and women with mild to moderate AKN (defined as fewer than 50 papules or
pustules) were treated with topical clobetasol propionate 0.05% foam (applied twice daily for two
weeks alternating with no therapy for two weeks) over an eight-week period [42]. At the end of
treatment, mean papule and pustule counts were significantly reduced compared with baseline.
Patients who continued to have active disease after this period were treated with betamethasone
valerate 0.12% foam (a less potent topical corticosteroid) for an additional four weeks. However, a
significant difference in response was not noted between 8 and 12 weeks.

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When treating patients with AKN, we typically prescribe a high-potency (group 1 or group 2 (table 1))
topical corticosteroid and instruct the patient to apply the corticosteroid once or twice daily to the
affected area. Because we usually simultaneously prescribe a topical antibiotic or topical retinoid,
we most frequently ask patients to use the topical corticosteroid once daily (see 'Adjunctive
therapies' below). The topical corticosteroid is applied before bed. In order to minimize risk for
corticosteroid side effects, we instruct patients to use the topical corticosteroid in two-week cycles
that alternate with two-week treatment-free intervals. Topical corticosteroids are available in a
variety of vehicles (eg, ointments, foams, gels, creams, solutions), which can be selected based upon
patient preference. We find that many of our patients prefer ointments or lotions, as these
formulations tend to be more soothing and cosmetically acceptable.

Of note, in the setting of severe suppuration or many pustules on the scalp, suggesting
superinfection, we delay topical corticosteroid treatment. Topical corticosteroid therapy may begin
following treatment with oral antibiotics and resolution of symptoms suggestive of infection or
suppuration. (See 'Moderate to severe inflammation' below.)

We reevaluate patients every four to six weeks to assess the response to treatment and to observe
for adverse effects. We expect a response to topical corticosteroid therapy (noticeable reduction in
severity of papules and pustules) within the first six to eight weeks of treatment, followed by
continued improvement with continued therapy. Once sufficient improvement is achieved, we
discontinue the topical corticosteroid with the plan to restart therapy if symptoms recur. If little
improvement is evident after six to eight weeks treatment, we transition to other therapies. (See
'Moderate to severe inflammation' below and 'Refractory inflammation' below.)

Potential adverse effects of topical corticosteroids include cutaneous atrophy and

hypopigmentation at the treatment site. Therefore, patients should be cautioned to apply the
corticosteroid only to the affected areas of the scalp and to avoid applying the corticosteroid for
extended periods without medical supervision. Additional adverse effects of topical corticosteroids
are reviewed separately. (See "Topical corticosteroids: Use and adverse effects", section on 'Adverse
effects'.)

Adjunctive therapies — We usually combine topical corticosteroid therapy with an

additional agent in an attempt to augment the response to treatment. For patients with a prominent
pustular component, we usually add a topical antimicrobial. For patients with primarily inflammatory
papules, we usually add a topical retinoid. Although topical antibacterials and topical retinoids are
commonly used therapies, the efficacy of these agents for AKN has not been evaluated in research
studies.

Topical antibacterial agents — Although AKN is not a primarily infectious condition,

clinical experience suggests that topical antibacterial agents are sometimes useful for improving
AKN. These agents may help to minimize disease exacerbations via the treatment and prevention of
secondary infection. The antiinflammatory effects of antibacterials may also be of benefit.

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We typically prescribe topical clindamycin solution, applied to the affected area once daily in the
morning. Application of the topical corticosteroid can be done at night. Alternative topical products
include erythromycin, benzoyl peroxide, benzoyl peroxide/clindamycin combination gel, or benzoyl
peroxide/erythromycin combination gel. The comparative efficacy of different topical antimicrobial
agents for AKN is unknown. Gel, solution, foam, or pledget formulations of topical antibacterial
agents may be additionally useful for their drying effects on pustules.

Potential adverse effects of topical antibacterial agents include allergic or irritant reactions. If gels,
solutions, or pledget formulations are too irritating, lotion formulations are an alternative. Patients
using benzoyl peroxide should be informed of the potential for the product to bleach clothing or bed
sheets.

Topical retinoids — It is theorized that topical retinoids may help to improve AKN by
normalizing follicular keratinocyte maturation and desquamation, thereby decreasing follicular
blockage. Topical retinoids also have antiinflammatory properties [43,44].

Topical retinoids available for use in the United States include tretinoin, adapalene, and tazarotene.
The efficacy of these agents for AKN has not been compared; therefore relative efficacy is unclear.

Irritation is a common side effect of topical retinoids, particularly at the start of therapy. We typically
use tretinoin cream, beginning with the lowest strength (0.025%) applied every other day, and
increase to daily application as tolerated. The strength of tretinoin can be increased as well,
depending on response. Topical tretinoin should be applied at night, as the drug is subject to
photodegradation. Therefore, when treating patients with topical tretinoin and a topical
corticosteroid, we ask the patient to apply the topical corticosteroid in the morning. Adapalene and
some specialized formulations of tretinoin are more light stable than standard tretinoin; however, in
practice, we typically still apply these at night. If beneficial, topical retinoid therapy can be continued
indefinitely.

Use of topical retinoids during pregnancy is discouraged. In particular, tazarotene, considered one of
the more effective topical retinoids in the context of acne treatment, is contraindicated in pregnancy
(category X (table 2)). The adverse effects of topical retinoids are reviewed in greater detail
separately. (See "Treatment of acne vulgaris", section on 'Adverse effects'.)

Moderate to severe inflammation — When patients present with

numerous inflammatory lesions, extensive pustulation or suppuration, or larger inflammatory
lesions, we alter the approach to therapy. Oral antibiotics and intralesional corticosteroids can be
useful for improving signs and symptoms of AKN in these patients. These drugs also represent a
second-line approach for patients with milder disease who fail to improve with topical therapy. In the
absence of infection, we usually combine oral antibiotic treatment with topical or intralesional
corticosteroid therapy.

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Oral antibiotics — Oral antibiotics are primarily reserved for severe flares of inflammation.
Tetracycline derivatives are most frequently prescribed for AKN because of their Gram positive
coverage and antiinflammatory effects. However, if superinfection is a concern (extensive erythema,
suppuration, pain, drainage, odor, or systemic symptoms such as fever), bacterial culture and
sensitivities should be obtained, and initial antibiotic therapy should be adjusted accordingly.

As with most other treatments for AKN, the efficacy of oral antibiotics for AKN has not been studied.
Use of these agents is based upon clinical experience that suggests benefit for improving
inflammation.

We typically treat adults with AKN with oral doxycycline (100 mg twice daily) for several weeks to
several months depending on the response to treatment. Minocycline (75 to 100 mg twice daily) is
an alternative, but there is increased risk of adverse events. We reevaluate patients after six to eight
weeks of treatment and expect to see at least a partial response at this period. Once inflammation is
controlled, we taper the doxycycline as tolerated to the lowest dose that maintains the response to
treatment. The goal is to eventually discontinue oral antibiotic therapy and maintain improvement
with the topical therapies used for milder inflammation. (See 'Mild to moderate inflammation' above.)

If flares occur during or after tapering, we instruct patients to restart doxycycline 100 mg twice daily.
Other antibiotics that have been used for AKN include cephalosporins, clindamycin, and
erythromycin.

Doxycycline and minocycline are generally well tolerated. Common side effects of tetracycline
derivatives include photosensitivity and gastrointestinal distress. In addition, minocycline has been
associated with vertigo, skin pigmentation, serum sickness, drug reaction with eosinophilia and
systemic symptoms (DRESS), hepatic abnormalities, and a lupus-like syndrome. (See "Treatment of
acne vulgaris", section on 'Tetracyclines'.)

Intralesional corticosteroids — As in many other inflammatory skin conditions,

intralesional corticosteroid injections may be useful for treating persistent inflammatory papules
that cannot be resolved with topical therapy. We typically inject triamcinolone acetonide (2.5 to 5
mg/mL) into inflamed papules and reevaluate in four weeks. Cutaneous atrophy and
hypopigmentation are among the most common side effects of this therapy. (See "Intralesional
corticosteroid injection", section on 'Side effects, complications, and pitfalls'.)

Intralesional corticosteroid injection can also be useful for softening the keloid-like papules of AKN.
(See 'Intralesional corticosteroids' below.)

Refractory inflammation — Some patients with AKN experience continued

progression of disease despite treatment with the above therapies. Successful treatment of such
patients with surgery, laser hair removal, or oral isotretinoin has been reported; however, efficacy
data are limited.

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Surgery — Because AKN is typically localized, surgical excision can be used to remove the
affected tissue. Recurrence is possible, particularly if the excision is insufficiently deep to remove
hair follicles. Surgery for AKN is discussed below. (See 'Surgery' below.)

Laser hair removal — A pilot study in which 16 patients with AKN were given five laser
treatments with a long pulsed 1064 nm neodymium:yttrium aluminum garnet (Nd:YAG) laser at four-
week intervals (fluence 35 to 45 J/cm2, pulse duration 10 to 30 msec) suggests benefit of laser hair
removal for AKN [3]. Improvement was detected in all patients, and the study found statistically
significant decreases in papule count, plaque count, and plaque size after treatment. Significant
softening of keloidal plaques was also observed. The best results were seen in patients with early
papular lesions compared with later stage disease with keloidal plaques, suggesting that such laser
treatment is most beneficial prior to the development of advanced disease. Recurrence of active
AKN (characterized by a few papular lesions) developed in 2 of 10 patients who were reevaluated
after one year; however, these patients responded well to additional laser treatment.

Improvement following treatment with a long pulsed 1064 nm Nd:YAG laser has also been
documented in a case report [45]. Treatment with other depilation lasers may also be effective; at
least two patients have improved following treatment with an 810 nm diode laser [46].

Reduced hair density in the treatment site is an expected side effect of this treatment. Other
potential side effects of laser therapy include dyspigmentation, blistering, and scarring.

Oral isotretinoin — Although case reports suggest that oral isotretinoin may help some
patients with severe, treatment-resistant inflammation, the limited data on efficacy and the multiple
side effects of isotretinoin (teratogenicity, hyperlipidemia, xerosis, visual changes, etc) prevent a
recommendation for routine use for AKN. One case report describes a man with both keratosis
follicularis spinulosa decalvans and AKN who experienced improvement in AKN during treatment
with 20 mg of oral isotretinoin daily (0.25 mg/kg per day) [34]. The AKN on the vertex demonstrated
rapid improvement in inflammation within weeks; however, the reduction in inflammation on the
nuchal area was less dramatic. Improvement was maintained for at least one year with a reduced
dose isotretinoin (20 mg every two to three days). (See "Keratosis pilaris atrophicans", section on
'Keratosis follicularis spinulosa decalvans'.)

In a second case report, six months of oral isotretinoin (initial dose 0.6 mg/kg per day for three
months, then 0.3 mg/kg per day for three months) was associated with resolution of papules in a
patient with AKN [47]. A recurrence after the end of treatment responded to a second course of
isotretinoin (0.1 mg/kg per day).

Isotretinoin is teratogenic and should not be given to pregnant women. In the United States,
participation in the iPLEDGE program, a computer-based risk management program designed to
reduce fetal exposure to isotretinoin, is required for prescribing the drug. The multiple adverse
effects of isotretinoin are reviewed separately. (See "Oral isotretinoin therapy for acne vulgaris",
section on 'Adverse effects'.)

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Treatment of keloidal scarring — The major therapeutic options

for the treatment of keloidal scarring in AKN are intralesional corticosteroid injections and surgery.

Papules and small plaques — Our preferred first-line treatment for small

keloidal scars is intralesional corticosteroid injection.

Intralesional corticosteroids — As with true keloids, intralesional corticosteroid

injection can be useful for softening and shrinking keloidal papules or plaques of AKN. (See
"Intralesional corticosteroid injection".)

We typically use triamcinolone acetonide in concentrations of 5 to 40 mg/mL depending on the size

of the scarred area. Keloidal papules may improve with doses as low as 5 mg/mL, whereas large,
thick plaques may require higher doses to achieve noticeable improvement. Injections can be
repeated every four to six weeks and the dose can be progressively titrated upward based upon the
response. At least a partial clinical response (at least some softening of the lesions) is expected
within one to three treatments. (See "Intralesional corticosteroid injection".)

Intralesional injections can be uncomfortable. When necessary, application of a topical analgesic

(eg, topical lidocaine) prior to treatment can help to reduce discomfort. Alternatively, the
corticosteroid may be diluted in lidocaine. Spraying the site with liquid nitrogen prior to injection may
also help to reduce pain of injection and may ease injection into the tissue; however,
hypopigmentation is a risk of liquid nitrogen, particularly in individuals with type IV to VI skin. We
typically do not use liquid nitrogen for analgesia.

Larger plaques — Patients with extensive scarring or scars that fail to improve

adequately with intralesional corticosteroid therapy may benefit from surgical therapy to remove the
affected area.

Surgery — Surgical therapies are indicated for the treatment of extensive scarring seen in
plaque or tumor stage AKN. Once large plaques have developed, surgical options are the only
effective recourse. Surgery can also be used to remove the site of disease in patients with active
disease that is poorly responsive to medical therapy. (See 'Refractory inflammation' above.)

●Procedures – AKN has been successfully treated with scalpel excision, electrosurgical excision,
and laser excision. Regardless of the surgical technique selected, the following measures may
optimize results:

•Deep excision – The depth of excision must extend below the base of the hair follicles to reduce
risk of recurrence [11,48,49]. Some authors suggest excising to the level of the deep subcutaneous
tissue or muscular fascia, given the propensity for hair follicles to extend into the subcutaneous fat
[48].

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•Horizontal excision – Orienting the excision horizontally with intent to incorporate the posterior
hairline may yield the best cosmetic results [48].

Options for closure of excision wounds include primary closure and healing by secondary intention.

•Primary wound closure – Primary wound closure provides immediate closure of the wound and
simplifies wound care for the patient. However, high tension on the closure may result in spreading
of the scar, hypertrophic scarring, or restricted head movement. Therefore, the preoperative
evaluation should include an assessment of the excision size and surrounding skin laxity to
determine if the wound can be comfortably closed. If the affected area is too large to excise in a
single procedure, staged excision with primary closure or secondary intention healing are options
[48].

•Secondary intention healing – Secondary intention healing eliminates concern for excessive wound
tension that may occur with primary wound closure, but requires an average of 6 to 12 weeks for the
wound to close. Scars often contract to a smaller size than the initial excised area, yielding improved
cosmesis after healing [50]. One review found data to suggest that fewer recurrences occur with
secondary intention healing than with primary closure; however, further study would be necessary to
confirm this finding [50]. The authors postulated that the secondary intention healing process might
effectively eliminate hair-bearing tissue or that the inflammatory response or process of prolonged
healing might have other beneficial effects on AKN [50].

Split thickness skin grafting also has been described but is considered less than optimal, since the
scar is usually depressed and texturally different (shiny and smooth) compared with the surrounding
skin [48]. In addition, the color of the grafted skin usually does not match the surrounding skin.

●Evidence – Although surgery is considered an effective treatment for AKN, few studies have
evaluated its efficacy. One of the largest studies evaluating surgical intervention for AKN involved 25
men with AKN refractory to medical therapy [48]. The patients had extensive, coalescent keloidal
plaques on the nuchal scalp. Twenty patients underwent complete excision with primary closure of
the surgical defect. The remainder had lesions too large for this procedure and had either a two-
stage excision with primary closure (four patients) or excision with secondary-intention healing (one
patient). One year after the procedure, the surgeon and the patients both rated cosmetic results as
good to excellent. Complications included mild recurrence of small papules and pustules in 15
patients (managed with topical corticosteroids) and hypertrophic scars in four patients who had
excision of large lesions and primary wound closure in a single procedure. In this particular study,
the four patients who underwent electrosurgical excision seemed to have a greater requirement for
pain medication than patients whose excisions were performed with a scalpel. However, good
tolerance of electrosurgery has also been reported [8].

Successful treatment with carbon dioxide laser excision followed by secondary-intention healing
was described in a series that included six patients treated with this modality [49]. No patients
experienced recurrences during follow-up periods of 3 to 47 months. Two patients developed slight
hypertrophic scarring that responded well to intralesional injection of corticosteroids.

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●Postsurgical interventions – Intralesional corticosteroid injections are useful for softening and
flattening hypertrophic scars and can be used if such scars develop after surgery [51]. Some authors
suggest use of intralesional corticosteroid injections beginning at the time of suture removal from
sutured wounds, as is often done after the excision of true keloids [36]. However, the effect of
prophylactic intralesional corticosteroid injections on patient outcomes has not been specifically
evaluated for AKN. Of note, intralesional corticosteroid injections may inhibit wound contraction and
should not be performed in wounds that will heal by secondary intention. (See "Keloids and
hypertrophic scars", section on 'Surgical excision and perioperative therapies'.)

Studies have yielded mixed results on the efficacy of topical imiquimod for the prevention of
postsurgical recurrence of true keloids [51]. The value of this intervention in AKN is unclear.

Emerging therapy
Ultraviolet light — Ultraviolet (UV) light can induce anti-fibrotic effects in skin by
inducing matrix metalloproteinase production as well as antiinflammatory effects [52]. The findings
of a small, split-scalp randomized trial of patients with papular lesions of AKN suggest that targeted
UVB phototherapy may be a useful therapy [52]. Patients were randomly assigned to targeted UVB
treatment three times per week to the affected area on either the right or left side of the scalp for
eight weeks. The UVB device emitted 290 nm to 320 nm light, with peaks at 303 nm and 313 nm.
After eight weeks, both sides of the scalp were treated. After the initial eight week period, the mean
lesion count on the treated sides decreased significantly, while the counts on the untreated side did
not change. Further improvement was demonstrated in sites that received an additional eight weeks
of UVB treatment.

UVB therapy was well tolerated; side effects were mild, including a transient burning sensation,
erythema, and hyperpigmentation. However, the need for multiple clinical visits can make
phototherapy inconvenient for some patients.

Radiotherapy — Radiotherapy led to resolution of active disease in a patient with

treatment-refractory AKN [53]. The disease did not recur during a follow-up period of 20 months.

SUMMARY AND
RECOMMENDATIONS
●Acne keloidalis nuchae (AKN) is a common chronic disorder characterized by inflammation and
keloid-like scarring on the posterior scalp or posterior upper neck. Most cases of AKN occur in post-
adolescent males of African descent. Less frequently, other individuals develop AKN. (See
'Epidemiology' above.)

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●The cause of AKN is unclear. Proposed contributory factors have included possession of Afro-
textured hair, close shaving or clipping of hair, and factors that may irritate or occlude hair follicles
on the posterior scalp and neck. Some authors consider AKN a form of primary scarring alopecia.
(See 'Pathogenesis' above.)

●The major clinical findings of AKN include inflammatory papules, pustules, and keloid-like papules
or plaques on the posterior scalp and nape of the neck. The severity of involvement varies widely.
Patients may be asymptomatic or symptomatic. Symptomatic patients may experience pain or
pruritus. (See 'Clinical manifestations' above.)

●The diagnosis of AKN usually can be made based upon the clinical evaluation. Clinical findings that
strongly suggest a diagnosis of AKN include firm keloid-like papules or plaques on the lower
posterior scalp or nape of the neck, with or without inflamed papules or pustules. Biopsy is not
typically necessary, but can be useful for confirming the diagnosis in atypical cases. (See 'Diagnosis'
above.)

●Data on treatments for AKN are limited. Treatment goals include reducing inflammation,
minimizing exposure to potential exacerbating factors, and improving the appearance of scarred
areas. Early treatment is important to minimize risk for severe scarring. (See 'Approach to treatment'
above.)

●For patients with mild to moderate inflammation in sites of AKN (small inflammatory papules or
pustules), we suggest high-potency topical corticosteroid therapy (Grade 2C). We typically use a
topical antibacterial agent or topical retinoid as adjunctive therapy. (See 'Mild to moderate
inflammation' above.)

●For patients with more severe inflammation, we suggest oral antibiotic therapy as initial treatment
(Grade 2C). We typically use oral doxycycline. If secondary infection is suspected, cultures and
sensitivity testing should be performed and antibiotic therapy should be prescribed as appropriate.
In the absence of infection, we often simultaneously treat with a topical corticosteroid. Intralesional
corticosteroid injections also can be useful for treatment of persistent, noninfectious inflammation.
(See 'Moderate to severe inflammation' above.)

●Scarring from AKN can result in significant cosmetic disfigurement. For patients with keloidal
papules or small plaques who desire cosmetic improvement, we suggest intralesional corticosteroid
injection as initial treatment (Grade 2C). For patients with larger plaques or keloidal scars that do not
improve sufficiently with intralesional corticosteroid injection, we suggest surgical excision (Grade
2C). (See 'Treatment of keloidal scarring' above.)

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