Chronic vulvar lesions in a woman in her 30s

Figure 2

Figure 3

Presentation
A 34-year-old G3P2 is referred to your clinic for evaluation due to chronic vulvar lesions that intermittently become painful with purulent discharge despite antibiotics.

Your diagnosis is: 

Discussion

The most likely diagnosis in this patient is hidradenitis suppurativa (HS) (Hurley stage 2) and you could use all of the listed treatments to address this condition. HS is a chronic inflammatory skin condition that involves skin in areas where apocrine glands are found.^1,2 The lesions of HS can mimic other conditions such as acne, carbuncles or Crohn’s disease but they are distinct in their pattern of distribution. 

You should suspect HS if the following three criteria are met:

• Lesions are nodules, abscesses, tunnels or scars; 

• Location is axillae, inframammary folds, groin, perigenital, or perineal area; and 

• Lesions progress (two recurrences within 6 months or chronic or persistent lesions for ≥ 3 months).^1,3

It has been suggested that HS develops due to a defect in the folliculopilosebaceous unit.^3,4 This results in weak-walled follicles that when occluded ultimately rupture, resulting in release of multiple inflammatory factors.^3,4 In turn, an acute inflammatory response develops in the surrounding tissue. As the body attempts to heal, chronic inflammation occurs, creating chronic tissue damage and resultant scarring.^1,3-5 HS also is thought to have a genetic component, with multiple genes likely to be involved.⁶ Infection appears to be a secondary reaction.^1,3 Androgens also play a role likely secondary to an increased end-organ sensitivity of androgen receptors mediated by insulin and insulin-like growth factor.⁷

HS is seen primarily after puberty and before menopause, with women affected more commonly than men.^3,5 Estimated prevalence is 0.5% to 4%.^1,8,9 Mean time to diagnosis is 7.2 years, which is unfortunate as the disease can be progressive and debilitating.^1,10

HS is staged by Hurley’s criteria.¹ Stage 1 is characterized by transient abscess formation without scarring and sinus tracts.¹ Stage 2 is characterized by recurrent abscesses (single or multiple) with sinus tracts or scarring.¹ If multiple lesions are present, they are separated by normal skin.¹ Finally the most severe stage is stage 3, consisting of diffuse disease with multiple connected tracts, tunnels, and significant scarring.¹ (Figure 2)

The therapeutic goals of treatment are to treat active disease and prevent disease progression. Most therapies target suspected underlying mechanisms. For stage 1 disease, patients are treated with general care measures, including avoiding vulvar irritants and tight clothing.^1,3 Behavioral and dietary changes such as weight loss, smoking cessation, and elimination of dairy products are encouraged as well.^1,3

For the initial flare, amoxicillin/clavulanic acid (875 mg po every 12 hours for 7 to 10 days) helps to treat the inflammation and secondary infection. Other antibiotics that can be considered include doxycycline (100 mg po twice daily) or clindamycin (300 mg po twice daily) for 7 to 10 days. Patients are also advised to take zinc picolinate (30 mg) with copper gluconate (2 mg).¹¹ This is available in a single pill to be taken twice a day. Other therapies offered include topical clindamycin lotion 1% twice daily. Intralesional triamcinolone injections are used at times. Anti-androgens such as drospirenone-containing oral contraceptives, spironolactone (50-100 mg po daily), or finasteride (5 mg po daily) may be helpful to control disease.¹ For stage 2 disease, other oral agents can be considered (sulfamethoxazole-trimethoprim, dapsone, or a combination of rifampin, moxifloxacin, and metronidazole), with maintenance on doxycycline or minocycline for approximately 3 months. Local surgical treatments such as unroofing tracts, wide local excision of lesions, skin tissue–sparing excision with electrosurgical peeling (STEEP) surgery and carbon dioxide laser evaporation of diseased tissue may be used forstage 2 HS.

For stage 3 or severe HS, radical wide excision with skin grafting is recommended for definitive treatment (Figure 3). This is an extensive process requiring resection of HS and wound vac placement, wound vac changes, and skin grafting 1 week later, if the tissue is granulating well. A wound vac is applied over the skin graft for 5 days. 

For stage 3 HS, you can also consider medical therapy with clindamycin 300 mg BID with rifampin 300 mg BID and a TNF α inhibitor such as adalimumab or infliximab¹. This is typically done in consultation with a dermatologist. Unfortunately, medical management is generally not a cure for patients with stage 3 HS. Surgical management of stage 3 HS has been found to have good results.¹² Finally, psychosocial support is critical for patients with HS as the condition can be isolating due to its nature.¹
 

Acne vulgaris
Acne vulgaris is a chronic inflammatory skin disease that affects the pilosebaceous unit and is typically seen in adolescents and young adults.^12,13 The skin changes can be described as open or closed comedones or as inflammatory nodules, pustules or papules.^12,14 Inflammatory lesions look most similar to HS lesions but the topographical distribution differs. In general, acne tends to be found on the face, neck, and back.¹³ The two disorders also share some similarities with regards to treatment goals and minimizing comorbid conditions such as depression. 

As with HS, treatment for acne vulgaris is designed based on disease severity. First-line treatment for mild disease is primarily treated with topical medications (e.g. benzoyl peroxide, retinoids or combinations of these with or without antibiotics).¹⁵ Moderate disease is treated with topical combination therapy or topical therapy with oral antibiotics.¹⁵ Severe disease is treated with an oral antibiotic along with a topical combination regimen or with oral isotretinoin.¹⁵
 

Folliculitis
Folliculitis is a bacterial infection of the hair follicles.¹⁶ It can occur in any hair-bearing area and is a common skin condition.¹⁵ Tissue trauma (e.g. shaving), excess heat (tight clothing) and immunosuppression (chronic steroid use) can predispose to development of these lesions.¹⁵ On vulvar examination, one would see papules and pustules. Diagnosis of folliculitis can be confirmed with a culture, in contrast to HS.¹⁵ Typically, Staphylococcus aureus is the offending microbe.¹⁶ Therapy usually is short-term and can include topical cleansers or medications such as chlorhexidine, benzoyl peroxide, clindamycin, fusidic acid or mupirocin.¹⁶ Oral antibiotics such as dicloxacillin, clindamycin or a macrolide can be used for cases that do not respond to topical therapy.¹⁵ Good vulvar care hygiene is key to avoid recurrence.¹⁶

Disclosures:

The authors report no potential conflicts of interest with regard to this article.

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• Rhode JM, Burke WM, Cederna PS, Haefner HK.  Outcomes of surgical management of stage III vulvar hidradenitis suppurativa. J Reprod Med. 2008;53:420-428..Fox L, Csongradi C, Aucamp M, Plessis J, Gerber M. Treatment modalities for acne. Molecules. 2016;21:1-20.

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