Section 2. Acneiform Disorders

A 16-year-old boy (Figure 112-1) with severe nodulocystic acne and scarring presents for treatment. After trying oral antibiotics, topical retinoids, and topical benzyl peroxide with no significant benefit, the patient and his mother request isotretinoin (Accutane). After 4 months of isotretinoin, the nodules and cysts cleared and there remained only a few papules (Figure 112-2). He is much happier and more confident about his appearance. The skin cleared fully after the last month of isotretinoin.

Acne is an obstructive and inflammatory disease of the pilosebaceous unit predominantly found on the face of adolescents. However, it can occur at any age and often involves the trunk in addition to the face.

Acne vulgaris affects more than 80% of teenagers, and persists beyond the age of 25 years in 3% of men and 12% of women.1

The four most important steps in acne pathogenesis:

1. Sebum overproduction related to androgenic hormones and genetics.

2. Abnormal desquamation of the follicular epithelium (keratin plugging).

3. Propionibacterium acnes proliferation.

4. Follicular obstruction, which can lead to inflammation and follicular disruption.

Neonatal acne is thought to be related to maternal hormones and is temporary (Figure 112-3).

Acne can be precipitated by mechanical pressure as with a helmet strap (Figure 112-4) and medications such as phenytoin and lithium (Figure 112-5).

There are some studies that suggest that consumption of large quantities of milk (especially skim milk) increase the risk for acne in teenagers.2

Clinical Features

Morphology of acne includes comedones, papules, pustules, nodules, and cysts.

• Obstructive acne = comedonal acne = noninflammatory acne and consists of only comedones (Figure 112-6).
• Open comedones are blackheads and closed comedones are called whiteheads and look like small papules.
• Inflammatory acne has papules, pustules, nodules, and cysts in addition to comedones (Figure 112-5).

Typical Distribution

Face, back, chest, and neck.

Laboratory Studies

None unless you suspect androgen excess and/or polycystic ovarian syndrome (PCOS).3 SOR A Obtain testosterone and DHEA-S levels if you suspect androgen excess and/or PCOS.

Consider follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels if you suspect PCOS.

• Acne conglobata is an uncommon and unusually severe form of acne characterized by multiple comedones, cysts, sinus tracks, and abscesses. The inflammatory lesions and scars can lead to significant disfigurement.4 Sinus tracks can form with multiple openings that drain foul-smelling purulent material (Figures 112-7, 112-8 and 112-9). The comedones and nodules are usually found on the chest, the shoulders, the back, the buttocks, and the face. In some cases acne conglobata is part of a follicular occlusion triad including hidradenitis and dissecting cellulitis of the scalp (Figure 112-9).
• Acne fulminans is characterized by sudden onset ulcerative crusting cystic acne, mostly on the chest and back (Figures 112-10 and 112-11).5 Fever, malaise, nausea, arthralgia, myalgia, and weight loss are common. Leukocytosis and elevated erythrocyte sedimentation rate are usually found. There may also be focal osteolytic lesions. The term acne fulminans may also be used in cases of severe aggravation of acne without systemic features.5
• Rosacea can resemble acne by having papules and pustules on the face. It is usually seen in older adults with prominent erythema and telangiectasias. Rosacea does not include comedones and may have ocular or nasal manifestations (Chapter 113, Rosacea). Rosacea fulminans or pyoderma faciale has features of severe acne and rosacea (Figure 112-12).
• Folliculitis on the back may be confused with acne. Look for hairs centrally located in the inflammatory papules of folliculitis to help distinguish it from acne. Acne on the back usually accompanies acne on the face as well (Chapter 117, Folliculitis).
• Acne keloidalis nuchae consists of papules, pustules, nodules, and keloidal tissue found at the posterior hairline. It is most often seen in men of color after shaving the hair at the nape of the neck (Chapter 114, Pseudofolliculitis and Acne Keloidalis Nuchae).
• Actinic comedones (blackheads) are related to sun exposure and are seen later in life (Figure 112-13).

Treatment is based on type of acne and severity. Categories to choose from are topical retinoids, topical antimicrobials, systemic antimicrobials, hormonal therapy, oral isotretinoin, and injection therapy.

Medications for Acne Therapy

In a review of 250 comparisons, the Agency for Healthcare Research and Quality found 14 had evidence of level A.6 These comparisons demonstrated the efficacy over vehicle or placebo control of topical clindamycin, topical erythromycin, benzoyl peroxide, topical tretinoin, oral tetracycline, and norgestimate/ethinyl estradiol.4 Level A conclusions demonstrating equivalence include: Benzoyl peroxide at various strengths was equally efficacious in mild/moderate acne; adapalene and tretinoin were equally efficacious.6 SOR A

Topical

• Benzoyl peroxide—Antimicrobial effect (gel, cream, lotion) (2.5%, 5%, 10%) 10% causes more irritation and is not more effective.1 SOR A
• Topical antibiotics—Clindamycin and erythromycin are the mainstays of treatment.
• Erythromycin—Solution, gel.3 SOR A
• Clindamycin—Solution, gel, lotion.3 SOR A
• Benzamycin gel—Erythromycin 3%, benzoyl peroxide 5%.3 SOR A
• BenzaClin gel—Clindamycin 1%, benzoyl peroxide 5%.3 SOR A
• Dapsone 5% gel.7 SOR B

Retinoids:

• Tretinoin (Retin-A) gel, cream, liquid, micronized.1 SOR A
• Adapalene gel—Less irritating than tretinoin.1 SOR A
• Tazarotene—Strongest topical retinoid with greatest risk of irritation.8 SOR A

Topical retinoids will often result in skin irritation during the first 2 to 3 months of treatment, but new systematic reviews do not demonstrate that they worsen acne lesion counts during the initial period of use.2

• Azelaic acid—Useful to treat spotty hyperpigmentation and acne (Figure 112-14).3 SOR B

Systemic

• Oral antibiotics.
  • Tetracycline 500 mg qd-bid—Inexpensive, absorbed best on an empty stomach.3 SOR A
  • Doxycycline 40 to 100 mg qd bid—Inexpensive, well tolerated, can take with food and increases sun sensitivity.3 SOR A
  • Minocycline 50 to 100 mg qd bid—Expensive, not proven to be better than other systemic antibiotics including tetracycline.3,9 SOR A
  • Erythromycin 250 to 500 mg bid—Inexpensive, frequent gastrointestinal (GI) disturbance but can be used in pregnancy.3 SOR A
  • Trimethoprim/sulfamethoxazole DS bid—Effective but risk of Stevens-Johnson syndrome is real. Reserve for short courses in particularly severe and resistant cases.3 SOR A

Oral azithromycin has been prescribed in pulse dosing for acne in a number of small poorly done studies and has not been found to be better than oral doxycyline.10

• Isotretinoin (Accutane) is the most powerful treatment for acne. It is especially useful for cystic and scarring acne that has not responded to other therapies.3 SOR A Dosed at approximately 1 mg/kg per day for 5 months. Women of childbearing age must use two forms of contraception. Monitor for depression.
• The U.S. Food and Drug Administration requires that prescribers of isotretinoin, patients who take isotretinoin, and pharmacists who dispense isotretinoin all must register with the iPLEDGE system (www.ipledgeprogram.com).
• Hormonal treatments:
  • Oral contraceptives only for females—Choose ones with low androgenic effect.3 SOR A FDA-approved oral contraceptives are Ortho Tri-Cyclen, Yaz, and Estrostep. Other oral contraceptives with similar formulations also help acne in women even though these have not received FDA approval for this indication. Note Yaz and Yasmin have progestin drospirenone, which is derived from 17α-spirolactone. It shares an antiandrogenic effect with spironolactone.
  • Spironolactone may be used for adult women when other therapies fail.3,11,12 This may be especially useful if the patient has hirsutism. Standard dosing is 50 to 200 mg/day. May start with 25 mg bid and monitor for hyperkalemia. The risk of hyperkalemia increases with a higher dose. Titrate up as needed and tolerated.3 SOR B A recent systematic review failed to show a benefit for spironolactone in acne even though it was found to decrease hirsutism.13
  • One small prospective study of 27 women with severe papular and nodulocystic acne used a combination of EE/DRSP (Yasmin) and spironolactone 100 mg daily. Eighty-five percent of subjects were entirely clear of acne lesions or had excellent improvement and there was no significant elevation of serum potassium.14

Complementary and Alternative Therapy

Tea tree oil 5% gel.15 SOR B

• Steroid injection therapy
• For painful nodules and cysts. SOR C Be careful to avoid producing skin atrophy.

• Dilute 0.1 cc of 10 mg/cc triamcinolone acetonide (Kenalog) with 0.4 cc of sterile saline for a 2 mg/cc suspension.
• Inject 0.1 cc with a 1-cc tuberculin syringe into each nodule using a 30-gauge needle (Figure 112-15).

Acne Therapy by Severity

Comedonal acne (Figure 112-6).

• Topical retinoid or azelaic acid.
• No need for antibiotics or antimicrobials—Do not need to kill P. acnes.

Mild papulopustular

• Topical antibiotics and benzoyl peroxide.
• Topical retinoid or azelaic acid.
• May add oral antibiotics if topical agents are not working.

Papulopustular or nodulocystic acne—moderate to severe—inflammatory

• Topical antibiotic, benzoyl peroxide, and oral antibiotic.
• Oral antibiotics are often essential at this stage.
• Topical retinoid or azelaic acid.
• Steroid injection therapy—For painful nodules and cysts.

Severe cystic or scarring acne

• Isotretinoin if there are no contraindications.
• Steroid injection therapy—For painful nodules and cysts.

Acne fulminans (Figures 112-9, 112-10, 112-11)

• Start with systemic steroids (prednisone 40 to 60 mg/day—approximately 1 mg/kg per day).16 SOR C
• Systemic steroid treatment rapidly controls the skin lesions and systemic symptoms. The duration of steroid treatment in one Finnish series was 2 to 4 months to avoid relapses.16 SOR C
• Therapy with isotretinoin, antibiotics, or both was often combined with steroids, but the role of these agents is still uncertain.16 SOR C
• One British series used oral prednisolone 0.5 to 1 mg/kg daily for 4 to 6 weeks (thereafter slowly reduced to zero).17 SOR C
• Oral isotretinoin was added to the regimen at the fourth week, initially at 0.5 mg/kg daily and gradually increased to achieve complete clearance.17 SOR C
• Consider introducing isotretinoin at approximately 4 weeks into the oral prednisone if there are no contraindications. SOR C

Acne conglobata and pyoderma faciale may be treated like acne fulminans but the course of oral prednisone does not need to be as long. SOR C

Combination Therapies

• Combination therapy with multiple topical agents can be more effective than single agents.3 SOR B
• Topical retinoids and topical antibiotics are more effective when used in combination than when either are used alone.3 SOR B
• Benzoyl peroxide and topical antibiotics used in combination are effective treatment for acne by helping to minimize antibiotic resistance.3 SOR B
• The adjunctive use of clindamycin/benzoyl peroxide gel with tazarotene cream promotes greater efficacy and may also enhance tolerability.18
• Combination therapy with topical retinoids and oral antibiotics can be helpful at the start of acne therapy. However, maintenance therapy with combination tazarotene and minocycline therapy showed a trend for greater efficacy but no statistical significance vs. tazarotene alone.19

Medication Cost

The most affordable medications for acne include topical benzoyl peroxide, erythromycin, clindamycin, and oral tetracycline and doxycycline. The most expensive acne medications are the newest brand-name combination products of existing topical medication. These medications are convenient for those with insurance that covers them (Epiduo contains benzoyl peroxide and adapalene; Ziana contains clindamycin and tretinoin).

Newer Expensive Modes of Therapy

Intense pulsed light and photodynamic therapy (PDT) use lasers, special lights, and topical chemicals to treat acne.20-22 These therapies are very expensive and the data do not suggest that these should be first-line therapies at this time. Light and laser treatments have been shown to be of short-term benefit if patients can afford therapy and tolerate some discomfort. These therapies have not been shown to be better than simple topical treatments.2

One comparative trial demonstrated that PDT was less effective than topical adapalene in the short-term reduction of inflammatory lesions.2

Isotretinoin requires monthly follow-up visits but other therapies can be monitored every few months at first and then once to twice a year. Keep in mind that many treatments for acne take months to work, so quick follow-up visits may be disappointing.

Adherence with medication regimens is crucial to the success of the therapy. Adequate face washing twice a day is sufficient. Do not scrub the face with abrasive physical or chemical agents. If benzoyl peroxide is not being used as a leave-on product, it can be purchased to use for face washing.

Patient Resources

• PubMed Health has a good patient education information—http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001876/.

Provider Resources

• Usatine R, Pfenninger J, Stulberg D, Small R. Dermatologic and Cosmetic Procedures in Office Practice. Philadelphia: Elsevier; 2012—Covers how to do acne surgery, steroid injections for acne, chemical peels, PDT and laser treatment for acne. It is also available as an app: www.usatinemedia.com.

A 34-year-old woman with extensive papulopustular rosacea (Figures 113-1, 113-2, 113-3) has a history of easy facial flushing since her teen years. Her face has been persistently redder in the past 5 years and she is bothered by this. She acknowledges that her mom has similar redness in her face and that she is from northern European heritage. In the last 6 months, since her daughter was born, she has developed many “pimples.” Physical examination reveals papules, pustules, and telangiectasias. No comedones are seen. She knows that the sun makes it worse but finds that many sunscreens are irritating to her skin. The patient is started on oral tetracycline daily and 0.75% metronidazole cream to use once daily. She agrees to wear a hat and stay out of the sun during the middle of the day. She will continue to look for a sunscreen she can tolerate. She knows that precipitating factors for her include hot and humid weather, alcohol, hot beverages, and spicy foods. She will do her best to avoid those factors.

Rosacea is an inflammatory condition of the face and eyes that mostly affects adults. Most commonly the face becomes reddened over the cheeks and nose and this is often accompanied by telangiectasias and a papulopustular eruption.

Rosacea is also called acne rosacea.

• Common in fair-skinned people of Celtic and northern European heritage.
• Women are more often affected than men.
• Men are more prone to the extreme forms of hyperplasia, which causes rhinophymatous rosacea (Figures 113-4 and 113-5).

• Although the exact etiology is unknown, the pathophysiology involves nonspecific inflammation followed by dilation around follicles and hyperreactive capillaries. These dilated capillaries become telangiectasias (Figures 113-6 and 113-7).
• As rosacea progresses, diffuse hypertrophy of the connective tissue and sebaceous glands ensues (Figures 113-4 and 113-5).
• Alcohol may accentuate erythema, but does not cause the disease. Rosacea runs in families.
• Sun exposure may precipitate an acute rosacea flare, but flare-ups can happen without sun exposure.
• A significant increase in the hair follicle mite Demodex folliculorum is sometimes found in rosacea.1 It is theorized that these mites play a role because they incite an inflammatory or allergic reaction by mechanical blockage of follicles.

Genetics, Demodex infestation,1 sun exposure.

Clinical Features

Rosacea has four stages or subtypes:

1. Erythematotelangiectatic rosacea (Figures 113-6 and 113-7)—This stage is characterized by frequent mild to severe flushing with persistent central facial erythema.

2. Papulopustular rosacea (Figures 113-1, 113-2, 113-3, 113-8 and 113-9)—This is a highly vascular stage that involves longer periods of flushing than the first stage, often lasting from days to weeks. Minute telangiectasias and papules start to form by this stage, and some patients begin having very mild ocular complaints such as ocular grittiness or conjunctivitis. These patients may have many unsightly pustules with severe facial erythema. They are more prone to develop a hordeolum (stye) (see Chapter 13, Hordeolum and Chalazion).

3. Phymatous or Rhinophymatous rosacea (Figures 113-4 and 113-5)—Characterized by hyperplasia of the sebaceous glands that form thickened confluent plaques on the nose known as rhinophyma. This hyperplasia can cause significant disfigurement to the forehead, eyelids, chin, and nose. The nasal disfiguration is seen more commonly in men than women. W. C. Fields is famous for his rhinophyma and intake of alcohol. Rhinophyma can occur without any alcohol use, as seen in the patient in Figure 113-5.

4. Ocular rosacea (Figures 113-10, 113-11, 113-12)—An advanced subtype of rosacea that is characterized by impressive, severe flushing with persistent telangiectasias, papules, and pustules. The patient may complain of watery eyes, a foreign-body sensation, burning, dryness, vision changes, and lid or periocular erythema. The eyelids are most commonly involved with telangiectasias, blepharitis, and recurrent hordeola and chalazia (Figures 113-10 and 113-11). Conjunctivitis may be chronic. Although corneal involvement is least common, it can have the most devastating consequences. Corneal findings may include punctate erosions, corneal infiltrates, and corneal neovascularization. In the most severe cases, blood vessels may grow over the cornea and lead to blindness (Figure 113-12).

Typical Distribution

Rosacea occurs on the face, especially on the cheeks and nose. However, the forehead, eyelids, and chin can also be involved (Figure 113-13).

Laboratory Studies

Not needed when the clinical picture is clear. If you are considering lupus or sarcoid, an antinuclear antibody (ANA), chest X-ray, or punch biopsy may be needed.

• Acne-The age of onset for rosacea tends to be 30 to 50 years, much later than the onset for acne vulgaris. Comedones are prominent in most cases of acne and generally absent in rosacea (see Chapter 112, Acne Vulgaris).
• Sarcoidosis on the face is much less common than rosacea, but the inflamed plaques can be red and resemble the inflammation of rosacea (see Chapter 175, Sarcoidosis).
• Seborrheic dermatitis tends to produce scale, whereas rosacea does not. Although both cause central facial erythema, papules and telangiectasias are present in rosacea and are not part of seborrheic dermatitis (see Chapter 151, Seborrheic Dermatitis).
• Systemic lupus erythematosus (SLE) can be scarring, does not usually produce papules or pustules, and it spares the nasolabial folds and nose (see Chapter 180, Lupus: Systemic and Cutaneous). The patient in Figure 113-13 has a butterfly distribution of her rosacea, but her right nasolabial fold is involved along with her chin.

The following three diagnoses were once considered variants of rosacea but a recent classification system identified these as separate entities:2

• Rosacea fulminans (known as pyoderma faciale) is characterized by the sudden appearance of papules, pustules, and nodules, along with fluctuating and draining sinuses that may be interconnecting. The condition appears primarily in women in their 20s, and intense redness and edema also may be prominent (Figure 113-14).2
• Steroid-induced acneiform eruption is not a variant of rosacea and can occur as an inflammatory response in any patient during or after chronic corticosteroid use. The same inflammatory response may also occur in patients with rosacea (Figure 113-15).
• Perioral dermatitis without rosacea symptoms should not be classified as a variant of rosacea. Perioral dermatitis is characterized by microvesicles, scaling, and peeling around the mouth (Figure 113-16).

• A Cochrane Database systematic review examined the efficacy of rosacea interventions.3 Oral doxycycline appeared to be significantly more effective than placebo and there was no statistically significant difference in effectiveness between the 100-mg and 40-mg doses.3 SOR A They found some evidence to support the effectiveness of topical metronidazole (0.75% or 1%), azelaic acid (15% or 20%) for the treatment of moderate to severe rosacea.3 SOR A Cyclosporine ophthalmic emulsion was significantly more effective than artificial tears for treating ocular rosacea (for all outcomes).3 SOR A
• When there are a limited number of papules and pustules, start with topical metronidazole (0.75% or 1%) or topical azelaic acid (15% or 20%).3
  • There are no substantial differences between topical metronidazole of 0.75% and 1%, or between once daily and twice daily regimens.4 Metronidazole cream, gel, and lotion have similar efficacies as well.4
• Azelaic acid in a 15% gel applied bid appeared to offer some modest benefits over 0.75% metronidazole gel in a manufacturer-sponsored study.4 Azelaic acid was not as well tolerated, so both medications are reasonable options with the choice depending on patient preference and tolerance.3 SOR B One study found that once-daily azelaic acid 15% gel was as effective as twice-daily application, which can translate into a significant cost saving.5
• If the skin lesions are more extensive, oral antibiotics, such as doxycycline (40 mg or 100 mg daily) is recommended.3 SOR A When attempting to avoid the photosensitivity side effects of doxycycline it is reasonable to prescribe oral tetracycline (250 mg to 500 mg daily) or oral metronidazole (250 mg to 500 mg daily). SOR C
• Patients who are started on oral antibiotics alone and improve may be switched to topical agents such as metronidazole or azelaic acid for maintenance.
• The Demodex mite may be one causative agent in rosacea. One study found permethrin 5% cream to be as effective as metronidazole 0.75% gel and superior to placebo in the treatment of rosacea.5 SOR B
• Severe papulopustular disease refractory to antibiotics and topical treatments can be treated with oral isotretinoin at a low dose of 0.3 mg/kg per day.6 SOR B
• Simple electrosurgery or laser without anesthesia can be used to treat the telangiectasias associated with rosacea. SOR C
• Rhinophyma can be excised with radiofrequency electrosurgery or laser. Isotretinoin is also used to treat rhinophyma.6 SOR B
• Traditional therapies for mild ocular rosacea include oral tetracyclines, lid hygiene, and warm compresses.1 SOR C Topical ophthalmic cyclosporine 0.05% (Restasis) is more effective than artificial tears for the treatment of rosacea-associated lid and corneal changes.7 SOR B Ocular rosacea that involves the cornea should be immediately referred to an ophthalmologist to prevent blindness (Figure 113-12).

Follow-up can be in 1 to 3 months as needed.

Sun protection, including use of a hat and daily application of sunscreen, should be emphasized. Choose a sunscreen that is nonirritating and protects against UVA and UVB rays. Advise patients to keep a diary to identify and avoid precipitating factors such as hot and humid weather, alcohol, hot beverages, spicy foods, and large hot meals.

Patient Resources

• National Rosacea Society. Its mission is to improve the lives of people with rosacea by raising awareness, providing public health information, and supporting medical research—http://www.rosacea.org/.

Provider Resources

• The National Rosacea Society also has an excellent set of materials that are geared for physicians—http://www.rosacea.org/.

A 17-year-old young African American man comes to the office because, for the past 2 years, he has been bothered by the uncomfortable bumps on the back of his neck (Figure 114-1). He is an athletic young man, more than 6 feet tall, and likes to keep his hair short for his sports. He notices the bumps get irritated and larger when he shaves his hair. He also has bumps on his face that get worse when he shaves his face (Figure 114-2). He is diagnosed with pseudofolliculitis barbae and acne keloidalis nuchae. His treatment consisted of patient education and twice-daily tretinoin cream and 0.1% triamcinolone cream to the neck area and nightly tretinoin cream to the beard area. He was told he could use 1% hydrocortisone cream on his face as needed. It was suggested that he minimize shaving, if possible.

Pseudofolliculitis is a common skin condition affecting the hair-bearing areas of the body that are shaved (Figures 114-2, 114-3, 114-4). Potential complications include postinflammatory hyperpigmentation, bacterial superinfection, and keloid formation.

• Pseudofolliculitis—Razor bumps, shave bumps.
• Acne keloidalis nuchae—Folliculitis keloidalis.

• Pseudofolliculitis is most common in black men, with at least 50% of black men who shave being prone to the condition.1 In the beard area it is called pseudofolliculitis barbae, and when it occurs after pubic hair is shaved, it is referred to as pseudofolliculitis pubis. It may also occur in the neck area.
• Acne keloidalis nuchae occurs most often in black men but can be seen in all ethnicities (Figures 114-3 and 114-5). The lesions are often painful and cosmetically disfiguring.
• Both conditions are seen in women but far less often than in men (Figure 114-6).

• Pseudofolliculitis develops when, after shaving, the free end of tightly coiled hair reenters the skin, causing a foreign-body-like inflammatory reaction. Shaving produces a sharp free end below the skin surface. Tightly curled hair has a greater tendency for the tip to pierce the surface of the skin and form ingrown hairs. This explains the relative predominance of this condition in patients of African ethnicity. The hair eventually forms a loop and if the embedded tip is pulled out there may be spontaneous resolution of symptoms.
• The exact cause of acne keloidalis is uncertain. It often develops in areas of pseudofolliculitis or folliculitis. It may be associated with haircuts where the posterior hairline is shaved with a razor and with tightly curved hair shafts. Other possible etiologies include irritation from shirt collars, chronic bacterial infections, and an autoimmune process. It is a form of primary scarring alopecia.2 As such, multiple hairs can be seen growing from single follicle (hair tufts) in the midst of the keloidal scarring (Figure 114-7).

• Pseudofolliculitis:
  • African ethnicity.
  • Curly hair.
• Acne keloidalis nuchae:
  • Shaving the hair on the neck.
  • Pseudofolliculitis.

Clinical Features

• The diagnosis of pseudofolliculitis is based upon clinical appearance. A piece of hair often may be identified protruding from a lesion. Inflammation results in the formation of firm, skin-colored, erythematous or hyperpigmented papules that occur after shaving (Figures 114-2, 114-3, 114-4). Pustules may develop secondarily. The severity varies from a few papules or pustules to hundreds of lesions.
• Patients with acne keloidalis initially develop a folliculitis or pseudofolliculitis, which heals with keloid-like lesions, sometimes with discharging sinuses. It starts after puberty as 2- to 4-mm firm, follicular papules (Figure 114-1). More papules appear and enlarge over time (Figure 114-5). Papules may coalesce to form keloid-like plaques, which are usually arranged in a band-like distribution along the posterior part of the hairline (Figures 114-7 and 114-8).

Typical Distribution

• Pseudofolliculitis affects the hair-bearing areas of the body that are shaved, especially the face, neck, and pubic area (Figures 114-2, 114-3, 114-4).
• Acne keloidalis occurs on the occipital scalp and the posterior part of the neck (Figures 114-1, 114-5, and 114-7).

Biopsy

• Histologic evaluation of a biopsy may confirm either diagnosis but is usually not necessary.

• True folliculitis, which is an acute pustular infection of a hair follicle with more localized inflammation (see Chapter 117, Folliculitis).
• Impetigo, which presents with yellowish pustules or bullae that rupture and develop honey crusts, sometimes with adenopathy (see Chapter 116, Impetigo).
• Acne vulgaris, which presents with comedones and pustules usually including the forehead (see Chapter 112, Acne Vulgaris).

Nonpharmacologic

• Avoid close shaving, avoid all shaving, or permanently remove hair.3 Some occupations, however, such as the military and law enforcement, require facial shaving. Occasionally, a doctor's note will allow these men to go without shaving. In mild cases, shaving should be discontinued for a month. The beard can be coarsely trimmed with scissors or electric clippers during this time. Shaving should not resume until all inflammatory lesions have resolved. Warm Burow solution compresses may be applied to the lesions for 10 minutes, 2 times per day. Instruct the patient to search for ingrown hairs each day using a magnifying mirror and release them gently using a sterilized needle or tweezers. The hairs should not be plucked as this may cause recurrence of symptoms with hair regrowth (Figure 114-6). SOR C
• Chemical depilatories (Ali, Royal Crown, Magic Shave, and others) cause fewer symptoms than shaving.4 SOR B However, these creams can cause severe irritation, so testing a small amount on the forearm is important. They work by breaking the disulfide bonds in hair, which results in the hair being bluntly broken at the follicular opening instead of sharply cut below the surface. They should be used every second or third day to avoid skin irritation, although this can be controlled with hydrocortisone cream. Barium sulfide 2% powder depilatories can be made into a paste with water, applied to the beard, and removed after 3 to 5 minutes. Calcium thioglycolate preparations are left on 10 to 15 minutes, but the fragrances can cause an allergic reaction and chemical burns can result if it is left for too long.
• People who have acne keloidalis nuchae should avoid anything that causes folliculitis or pseudofolliculitis, such as getting their neck or hairline shaved with a razor.

Medications

• Topical eflornithine HCL 13.9% cream (Vaniqa; by prescription only) may be used to inhibit hair growth. It decreases the rate of hair growth and may make the hair finer and lighter. Unfortunately, this medication is expensive and requires daily application for continued efficacy. SOR C
• Twice-daily treatment with a class 2 or 3 corticosteroid may be sufficient to shrink pseudofolliculitis lesions and relieve symptoms (see Appendix B: Topical and Intralesional Corticosteroids). SOR C
• When pustules, crust formation, or drainage is present, use topical clindamycin or erythromycin. Unresponsive patients may be changed to a systemic antibiotic. SOR C
• Topical erythromycin, clindamycin, and combination clindamycin-benzoyl peroxide (BenzaClin, Duac) and erythromycin-benzoyl peroxide (Benzamycin) may be used once or twice daily.5 SOR B
• Oral doxycycline 100 mg bid, tetracycline 500 mg bid, or erythromycin 500 mg bid may be used for patients with more severe secondary inflammation. SOR C
• Tretinoin cream, 0.025%, may be useful in patients with mild disease, but is rarely helpful in moderate to severe cases.6 It is applied nightly for a week then reduced to every second or third night. Tretinoin may be used in conjunction with a mid-potency topical corticosteroid applied each morning. The mechanism of action is thought to be by relieving hyperkeratosis and “toughening” the skin. Topical combination cream (tretinoin 0.05%, fluocinolone acetonide 0.01%, and hydroquinone 4%) (Tri-Luma) adds an additional postinflammatory hyperpigmentation treatment. SOR C
• Intralesional steroid injections (10 to 20 mg/mL) may be used to soften and shrink keloids. Warn patients that this therapy may cause hypopigmentation (Figure 114-8). SOR C

Surgical

• The only definitive cure for pseudofolliculitis is permanent hair removal. Electrolysis is expensive, painful, and sometimes unsuccessful. Laser hair removal is fairly successful for treating pseudofolliculitis.7 SOR B Diode laser (810 nm) treatments have been proven safe and effective in patients with skin phototypes I to IV.8
• Excision of acne keloidalis lesions may be attempted. Recalcitrant keloidal lesions may be treated with removing individual papules with a small punch, or large keloids (Figure 114-9) with an elliptical excision closed with sutures. After removal, the wound edges should be injected with a mixture of equal amount of triamcinolone acetonide 40 mg/mL and sterile saline. Remove the sutures in 1 to 2 weeks and inject the edges every month with the above mixture for 3 to 4 times. SOR C Excision should extend into the subcutaneous tissue and the wound edges can be injected with 10 to 40 mg/mL of triamcinolone acetonide and be reapproximated. SOR C Recurrence is common, especially with shallow excisions or not treating with steroids.
• Other therapies that may be considered are laser therapy (carbon dioxide or Nd:YAG [neodymium:yttrium-aluminum-garnet]) followed by intralesional triamcinolone injections or cryotherapy for two 20-second bursts that are allowed to thaw and are then applied again a minute later. These methods may produce more pain and hypopigmentation. SOR C

• Termination of shaving prevents the development of pseudofolliculitis.

• No specific cure exists. If the patient is able to stop shaving, the problem usually disappears (except for any scar formation).

• Instruct patients to return if any complications occur. Otherwise have them return for possible initiation of intralesional steroid injections or topical steroid/retinoic acid therapy once the area has healed.

• For those who must shave, have the patient clip hairs no shorter than needed for maintenance. Use fine scissors or facial hair clippers if possible. When shaving, have the patient rinse with warm tap water for several minutes, use generous amounts of a highly lubricating shaving gel, and allow it to soften the skin for 5 to 10 minutes before shaving. The patient should always use sharp razors and shave in the direction of hair growth. Specialized guarded razors (e.g., PFB Bump Fighter) are available in pharmacies and by mail order. After shaving rinse the face with tap water, and then apply cold water compresses.
• With acne keloidalis, instruct males who play football to make sure their helmets fit properly and do not cause irritation on the posterior part of the scalp. They should avoid having the posterior part of the hairline shaved with a razor as part of a haircut, and discontinue wearing garments that rub or irritate the posterior parts of the scalp and the neck.

Patient Resources

• American Osteopathic College of Dermatology. Pseudofolliculitis— http://www.aocd.org/skin/dermatologic_diseases/pseudofolliculitis.html.
• Skin Channel. Acne Keloidalis Nuchae—http://skinchannel.com/acne/acne-keloidalis-nuchae/.

Provider Resources

• eMedicine. Pseudofolliculitis—http://emedicine.medscape.com/article/1071251.
• eMedicine. Acne Keloidalis—http://emedicine.medscape.com/article/1072149.
• DermNet NZ. Acne Keloidalis—http://dermnetnz.org/acne/keloid-acne.html.

A 25-year-old woman presents with new tender lesions in her axilla (Figure 115-1). She admits to years of similar outbreaks in both axilla and occasional painful bumps in the groin. She states that it is painful to have them opened and just wants to get some relief without surgery. We elected to inject the nodules with triamcinolone and start the patient on doxycycline 100 mg twice daily. Smoking cessation was emphasized and the patient agreed to start on a nicotine patch that evening. She had relief within 24 hours from the steroid injection.

Hidradenitis suppurativa (HS) is an inflammatory disease of the pilosebaceous unit in the apocrine gland-bearing skin. HS is most common in the axilla and inguinal area, but may be found in the inframammary area as well. It produces painful inflammatory nodules, cysts, and sinus tracks with mucopurulent discharge and progressive scarring.

It is called acne inversa because it involves intertriginous areas and not the regions affected by acne (similar to inverse psoriasis).

• Occurs after puberty in approximately 1% of the population.1
• Incidence is higher in females, in the range of 4:1 to 5:1. Flare-ups may be associated with menses.1

• Disorder of the terminal follicular epithelium in the apocrine gland-bearing skin.1
• Starts with occlusion of hair follicles that lead to occlusion of surrounding apocrine glands.
• Chronic relapsing inflammation with mucopurulent discharge (Figures 115-2, 115-3, 115-4, 115-5, 115-6, 115-7).
• Can lead to sinus tracts, draining fistulas and progressive scarring (Figures 115-2, 115-3, 115-4, 115-5, 115-6, 115-7).

Obesity, smoking, and tight-fitting clothing.

Clinical Features

• Most common presentation is painful, tender, firm, nodular lesions in axillae (Figures 115-1, 115-2, 115-3).
• Nodules may open and drain pus spontaneously and heal slowly, with or without drainage, over 10 to 30 days.1
• Nodules may recur several times yearly, or in severe cases new lesions form as old ones heal.
• Surrounding cellulitis may be present and require systemic antibiotic treatment.
• Chronic recurrences result in thickened sinus tracts, which may become draining fistulas (Figures 115-3, 115-4, 115-5, 115-6, 115-7).
• HS can cause disabling pain, diminished range of motion, and social isolation (Figure 115-5).

Typical Distribution

• Axillary, inguinal, periareolar, intermammary zones, pubic area, infraumbilical midline, gluteal folds, top of the anterior thighs, and the perianal region.1

Laboratory Studies

Culture of purulence is likely to yield staphylococci and streptococci and is usually unnecessary to determine treatment. Culture may be useful if you suspect methicillin-resistant Staphylococcus aureus.

• Bacterial infections, including folliculitis, carbuncles, furuncles, abscess, and cellulitis, may resemble HS but are less likely to be recurrent in the intertriginous areas.
• Epidermal cysts in the intertriginous regions may resemble HS. Theses cysts contain malodorous keratin contents.
• Granuloma inguinale and lymphogranuloma venereum are sexually transmitted infections that can produce inguinal ulcers and adenopathy that could be mistaken for HS.

• Lifestyle changes are recommended, including weight loss if obesity is present. SOR C
• Smoking is a risk factor for HS and cessation is highly recommended for many reasons.1 See SOR B for HS and SOR A for other health reasons.
• Frequent bathing and wearing loose-fitting clothing may help.

Medical treatment is similar to acne treatment:

• Oral antibiotics are used in acute and chronic treatment. Oral tetracyclines, clindamycin, rifampin, and dapsone have been touted as beneficial. If there is methicillin-resistant S. aureus present, trimethoprim/sulfamethoxazole or clindamycin should be used.
• Tetracycline 500 mg bid and doxycycline 100 mg bid can be used acutely and to prevent new lesions in the mildest of cases. Many patients do not find these antibiotics to be of great help. SOR C
• Topical clindamycin bid may be used in the mildest of cases. In one randomized controlled trial (RCT), systemic therapy with tetracyclines did not show better results than topical therapy with clindamycin.1 SOR B
  • Combination of systemic clindamycin (300 mg twice daily) and rifampin (600 mg daily) is recommended for patients with more severe HS.2,3 In a series of 116 patients, parameters of severity improved, as did the quality-of-life score.2 In another study, 28 of 34 patients (82%) experienced at least partial improvement, and 16 (47%) showed a total remission.3 The maximum effect of treatment appeared within 10 weeks. Following total remission, 8 of 13 (61.5%) patients experienced a relapse after a mean period of 5 months. Nonresponders were predominantly patients with severe disease. The most frequent side effect is diarrhea.2,3
  • Oral dapsone may be considered in milder cases. In one study, only 38% of patients experienced improvement.4 SOR B Rapid recurrence after stopping treatment suggests that antiinflammatory effects may predominate over antimicrobial effects. The total effect appears to be smaller than that reported with combination therapy using clindamycin and rifampicin.
• Isotretinoin can reduce the severity of attacks in some patients but is not a reliable cure for HS.5 SOR C
• Acitretin can be an effective treatment for refractory HS. In one study, all 12 patients achieved remission and experienced a significant decrease in pain. Long-lasting improvement was observed in 9 patients, with no recurrence of lesions after 6 months (n = 1), 1 year (n = 3), more than 2 years (n = 2), more than 3 years (n = 2), and more than 4 years (n = 1). 5 SOR B
• Antitumor necrosis factor (TNF) agents are being studied for severe, recalcitrant HS (Figure 115-8). In one series, infliximab therapy (weight based) was shown to be effective and well tolerated in 6 of 7 patients with HS who were resistant to previous therapy.6 This was in agreement with preexisting literature showing that 52 of 60 patients (87%) were improved after infliximab therapy.6 SOR B Adalimumab helps in the short-term, but no long-term curative effect was uniformly seen.7
• Intense pulsed light (IPL) with laser may be worth considering for patients who can afford the cost and time for treatment. In one study of 18 patients who were randomized to treatment of one axilla, groin, or inframammary area with IPL 2 times per week for 4 weeks, there was a significant improvement in the mean examination score, which was maintained at 12 months. Patients reported high levels of satisfaction with the IPL treatment.8 SOR B

Surgical treatments include the following:

• Intralesional steroids with 5 to 10 mg/mL of triamcinolone may help to decrease inflammation and pain within 24 to 48 hours. SOR C
• Incision and drainage of acute lesions are suggested for the large fluctuant abscesses that can occur in HS. Although this may give some relief of the pressure, the surgical treatment and repacking of the wound is painful, and there is no evidence that it speeds healing. SOR C
• Lancing small nodules is more painful than helpful and is not recommended.
• Surgical excision of affected area with or without skin grafting is used for recalcitrant disabling disease and should be individualized based on the stage and location of the disease.9 SOR B One surgical group has been using a medial thigh lift for immediate defect closure after radical excision of localized inguinal hidradenitis.10

If there is cellulitis or a large abscess was drained, follow-up should be within days. Chronic relapsing disease can ultimately be managed with appointments every 3 to 6 months depending upon the treatment and its success.

Smoking cessation, weight loss if overweight, and avoidance of tight-fitting clothes.

Patient Resources

• Patient education materials at Medline Plus—http://www.nlm.nih.gov/medlineplus/hidradenitissuppurativa.html.

Provider Resources

• http://emedicine.medscape.com/article/1073117-overview.