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The International Society for the Study of Vulvovaginal Disease (ISSVD) provides classification systems for vulvar abnormalities. Their 2006 Classification of Vulvar Dermatoses specifically focuses on dermatoses and divides these based on biopsy-obtained histology. Their more recent terminology and organization system does not supplant the 2006 system. Instead, it classifies a broader group of dermatologic disorders that includes dermatoses, infections, and neoplasias by their similar clinical presentations to aid identification and management (Lynch, 2007, 2012). The next sections describe the more frequently encountered conditions.
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Lichen Simplex Chronicus
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An itch-scratch cycle typically leads to chronic trauma from rubbing and scratching (Lynch, 2004). Early examination reveals excoriations within a background of erythema. With chronic trauma, the skin responds by thickening, termed lichenification. Thus, in long-standing cases, vulvar skin is thick with exaggerated skin markings causing a leathery, gray appearance. Skin changes are usually bilateral and symmetric and may extend beyond the labia majora. Intense vulvar pruritus causes functional and psychologic distress, and sleep is often disrupted. Potential pruritus triggers include irritation from clothing, heat, or sweating; chemicals contained within hygiene products and topical medications; laundry products; and even food sensitivities (Virgili, 2003). A detailed history typically leads to the diagnosis.
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Treatment involves halting the itch-scratch cycle. First, provocative stimuli are eliminated, and topical corticosteroid ointments help to reduce inflammation. In addition, lubricants, such as plain petrolatum or vegetable oil, and cool sitz baths help to restore the skin’s barrier function. Oral antihistamine use, trimmed fingernails, and cotton gloves worn at night can help decrease scratching during sleep. If symptoms fail to resolve within 1 to 3 weeks, biopsy is indicated to exclude other pathology. Histology classically shows thickening of both the epidermis (acanthosis) and the stratum corneum (hyperkeratosis). In these refractory cases, a trial of higher-potency corticosteroid may improve symptoms.
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This classically presents in postmenopausal women, although cases are less often found in premenopausal women, children, and men (Fig. 14-8). In a referral dermatologic clinic, lichen sclerosus was found in 1:300 to 1:1000 patients with a tendency toward whites (Wallace, 1971). Others estimate an incidence of childhood lichen sclerosus to be 1 in 900 (Powell, 2001).
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The cause of lichen sclerosus remains unknown, although infectious, hormonal, genetic, and autoimmune etiologies have been suggested. Approximately 20 to 30 percent of patients with lichen sclerosus have other autoimmune disorders, such as Graves disease, types 1 and 2 diabetes mellitus, systemic lupus erythematosus, and achlorhydria, with or without pernicious anemia (Bor, 1969; Kahana, 1985; Poskitt, 1993). Accordingly, concurrent testing for these is indicated if other suggestive findings are present.
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Although sometimes asymptomatic, most individuals with lichen sclerosus complain of anogenital symptoms that often worsen at night. Inflammation of local terminal nerve fibers is suspected. Pruritus-induced scratching creates a vicious cycle that may lead to excoriations and vulvar skin thickening. Late symptoms can include burning and dyspareunia due to vulvar skin fragility and structural changes.
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Perianal involvement is frequently seen. The typical white, atrophic papules may coalesce into porcelain-white plaques that distort normal vulvar anatomy. As a result, labia minora regression, clitoral concealment, urethral obstruction, and introital stenosis can develop. The skin generally appears thinned and crinkled. Over time, involvement may extend to the perineum and anus to form a “figure-eight” or “hourglass” shape (Fig. 4-3) (Clark, 1967). Thickened white plaques, areas of erythema, or nodularity should prompt biopsy to exclude preinvasive and malignant lesions. This characteristic clinical picture and histologic findings typically confirm the diagnosis. In long-standing cases, histologic findings may be nonspecific, and clinical suspicion will guide treatment.
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Treatment and Surveillance
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Curative therapies are not available for lichen sclerosus. Thus, treatment goals are symptom control and prevention of anatomic distortion. Despite its classification as a nonneoplastic dermatosis, patients with lichen sclerosus demonstrate an increased risk of vulvar malignancy. Malignant transformation within lichen sclerosus develops in 5 percent of patients. Histologic cellular atypia may precede a diagnosis of invasive squamous cell carcinoma (Scurry, 1997). Accordingly, lifetime surveillance of women with lichen sclerosus every 12 months is prudent (Neill, 2010). Persistently symptomatic, new, or changing lesions should be biopsied (American College of Obstetricians and Gynecologists, 2010).
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As with all vulvar disorders, hygiene recommendations focus on minimizing chemical and mechanical skin irritation (Table 4-2). The chronicity of lichen sclerosus and lack of cure elicits an array of emotions. Support groups dedicated to this condition, such as that found at www.lichensclerosus.org, offer needed psychologic support.
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First-line therapy for lichen sclerosus is an ultrapotent topical corticosteroid preparation such as 0.05-percent clobetasol propionate (Temovate) or 0.05-percent halobetasol propionate (Ultravate). Ointment formulations are preferred by some providers over creams due to their protective and less irritating properties (Table 4-3). Clobetasol propionate offers effective antiinflammatory, antipruritic, and vasoconstrictive properties. Theoretic adrenocorticosuppression and iatrogenic Cushing syndrome may be risks if it is used in large doses for extended periods.
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Treatment initiation within 2 years of symptom onset usually prevents significant scarring, but no treatment scheme is universally accepted for topical corticosteroid use. The currently recommended dosing schedule of the British Association of Dermatologists is 0.05-percent clobetasol propionate once nightly for 4 weeks, followed by alternating nights for 4 weeks, and finally tapering to twice weekly for 4 weeks (Neill, 2010). After this initial therapy, recommendations for maintenance therapy vary and range from tapering corticosteroids to “as needed” use to ongoing once- or twice-weekly applications. During initial treatment, some patients may also require oral antihistamines or topical 2-percent lidocaine jelly particularly at night to control itching.
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Corticosteroids can also be injected into affected areas, a treatment offered by specialty clinics familiar with techniques and potential complications. One study of eight patients evaluated the efficacy of once-monthly intralesional infiltration of 25 to 30 mg of triamcinolone hexacetonide for a total of 3 months. Severity scores decreased in all categories including symptoms, gross appearance, and histopathologic findings (Mazdisnian, 1999).
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Other Topical Treatments
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Estrogen cream is not a primary therapy for lichen sclerosus. However, its addition is indicated for menopausal atrophy, labial fusion, and dyspareunia. Testosterone ointment has failed to show efficacy in trials and is no longer recommended (Bornstein, 1998; Sideri, 1994).
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Retinoids are reserved for severe, nonresponsive cases of lichen sclerosus or for patients intolerant of ultrapotent corticosteroids. Topical tretinoin reduces hyperkeratosis, improves dysplastic changes, stimulates collagen and glycosaminoglycan synthesis, and induces local angiogenesis (Eichner, 1992; Kligman, 1986a,b; Varani, 1989). Virgili and colleagues (1995) evaluated the effects of topical 0.025-percent tretinoin (Retin-A, Renova) applied once daily, 5 days a week for 1 year. Complete remission of symptoms was seen in more than 75 percent of women. However, more than one quarter of patients experienced skin irritation, which is common with retinoids.
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Topical calcineurin inhibitors such as tacrolimus (Protopic) and pimecrolimus (Elidel) have antiinflammatory and immunomodulating effects. These are indicated for moderate to severe eczema and have been evaluated for lichen sclerosus (Goldstein, 2011; Hengge, 2006). Moreover, these agents, compared with topical corticosteroids, theoretically lower the risk of skin atrophy, since collagen synthesis is unaffected (Assmann, 2003; Kunstfeld, 2003). However, from a double-blind, randomized, prospective study, Funaro and associates (2014) concluded that topical clobetasol propionate was more effective in treating vulvar lichen sclerosus than topical tacrolimus. In the face of recent Food and Drug Administration (FDA) concerns regarding its link to various cancers, clinicians should exercise caution when prescribing tacrolimus for extended periods (Food and Drug Administration, 2010).
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Last, phototherapy after pretreatment using 5-aminolevulinic acid was investigated in one small series of 12 postmenopausal women with advanced lichen sclerosus. Significant reductions in patient symptoms and short-term improvement for up to 9 months were noted (Hillemanns, 1999).
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Surgical intervention should be reserved for significant sequelae and not for primary treatment of uncomplicated lichen sclerosus. For introital stenosis, Rouzier and coworkers (2002) described marked improvements in dyspareunia and quality of sexual intercourse if perineoplasty was performed (Section 43-22). Vaginal dilation and corticosteroids are recommended following most surgical corrections of introital stenosis. For clitoral adhesions, surgical dissection can be used to free the hood from the glans. Reagglutination can be averted using initial nightly application of ultrapotent topical corticosteroid ointment (Goldstein, 2007).
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Inflammatory Dermatoses
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A primary irritant or allergen creates vulvar skin inflammation, termed contact dermatitis (Fig. 4-4). This condition is common, and in unexplained cases of vulvar pruritus and inflammation, irritant contact dermatitis is diagnosed in up to 54 percent of patients (Fischer, 1996).
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Irritant contact dermatitis classically presents as immediate burning and stinging upon exposure to an offending agent. In contrast, patients with allergic contact dermatitis experience a delayed onset and an intermittent course of pruritus and localized erythema, edema, and vesicles or bullae (Margesson, 2004). A detailed history will help distinguish between the two, and an inquiry for potential offending agents can help identify the irritant (see Table 4-1).
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With allergic contact dermatitis, patch testing may aid in identifying responsible allergen(s). Alternative conditions, such as candidiasis, psoriasis, seborrheic dermatitis, and squamous cell carcinoma, can be excluded through appropriate use of cultures and biopsy.
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Treatments for both entities involve elimination of the offending agent(s), restoration of the natural protective skin barrier, inflammation reduction, and scratch cessation (Table 4-4) (Farage, 2004; Margesson, 2004).
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Friction between moist skin surfaces produces this chronic condition. Found most often in genitocrural folds, intertrigo can also develop in the inguinal and intergluteal regions. Superimposed bacterial and fungal infections may complicate the condition.
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The initial erythematous phase, if untreated, can progress to intense inflammation with erosions, exudate, fissuring, maceration, and crusting (Mistiaen, 2004). Symptoms typically include burning and itching. With long-standing intertrigo, hyperpigmentation and verrucous changes can develop.
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Treatment entails the use of drying agents such as cornstarch and application of mild topical corticosteroids for inflammation. If skin changes do not respond, then seborrheic dermatitis, psoriasis, atopic dermatitis, pemphigus vegetans, or even scabies are considered. If the area is superinfected with bacteria or yeast, appropriate therapy is warranted.
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To prevent recurrent outbreaks, obese patients are encouraged to lose weight. Other preventions include light-weight, loose-fitting clothing made of natural fibers, improved ventilation, and thorough drying between skin folds after bathing (Janniger, 2005).
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Classically presenting in the first 5 years of life, atopic dermatitis is a severe pruritic dermatitis that follows a chronic, relapsing course. Scaly patches with fissuring are evident. Individuals with atopic eczema may later develop allergic rhinitis and asthma (Spergel, 2003).
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Topical corticosteroids and immunomodulators, such as tacrolimus, can control flares (Leung, 2004). For dry skin, moisturizing with emollients can offer relief.
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Approximately 1 to 2 percent of the United States’ population is affected by psoriasis (Gelfand, 2005). Psoriasis is a T-cell—mediated autoimmune process in which proinflammatory cytokines induce keratinocyte and endothelial cell proliferation. Thick, red plaques covered with silvery scales are generally found on extensor limb surfaces. Occasionally, lesions involve the mons pubis or labia (Fig. 4-5). Psoriasis can be exacerbated by nervous stress and menses, with remissions experienced during summer months and pregnancy. Pruritus may be minimal or absent, and this condition is often diagnosed by skin findings alone.
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Several treatments are available, and topical corticosteroids are widely used because of their rapid efficacy. High-potency corticosteroids are applied to affected areas twice daily for 2 to 4 weeks and then reduced to weekly applications. Diminishing response and skin atrophy are potential disadvantages of long-term corticosteroid use, and recalcitrant cases are best managed by a dermatologist. Vitamin D analogues, such as calcipotriene (Dovonex), although similar in efficacy to potent corticosteroids, are frequently associated with local irritation but avoid skin atrophy (Smith, 2006). Phototherapy offers short-term relief, but long-term treatment plans require a multidisciplinary team (Griffiths, 2000). For moderate to severe psoriasis, several FDA-approved immunomodulating biologic agents are available and include infliximab, adalimumab, etanercept, and ustekinumab (Smith, 2009).
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This uncommon disease involves both cutaneous and mucosal surfaces and affects genders equally between ages 30 and 60 years (Mann, 1991). Although not completely understood, T-cell autoimmunity directed against basal keratinocytes is thought to underlie its pathogenesis (Goldstein, 2005). Vulvar lichen planus can present as one of three variants: (1) erosive, (2) papulosquamous, or (3) hypertrophic. Of these, erosive lichen planus is the most common vulvovaginal form and the most difficult variant to treat. Lichen planus may be drug-induced, and nonsteroidal antiinflammatory drugs, β-blocking agents, methyldopa, penicillamine, and quinine drugs have been implicated.
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Table 4-5 summarizes the most common imitators of lichen planus. Women typically complain of chronic vaginal discharge with intense vulvovaginal pruritus, burning pain, dyspareunia, and postcoital bleeding. On inspection, papules classically are brightly erythematous or violaceous, flat-topped, shiny polygons most commonly found on the trunk, buccal mucosa, or flexor surfaces of the extremities (Goldstein, 2005; Zellis, 1996). Lacy, white striations (Wickham striae) are frequently found in conjunction with the papules and may also be present on the buccal mucosa (Fig. 4-6). Deep, painful erosions in the posterior vestibule can extend to the labia, resulting in agglutination. With speculum insertion, vulvar skin and vaginal mucosa bleed easily. Vaginal erosions can produce adhesions and synechiae, which may lead to vaginal obliteration.
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Women with suspected lichen planus require a thorough dermatologic survey looking for extragenital lesions. Nearly one quarter of women with oral lesions will have vulvovaginal involvement, and most with erosive vulvovaginal lichen planus will have oral involvement (Pelisse, 1989). Diagnosis is confirmed by biopsy.
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Vulvar Lichen Planus Treatment
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Pharmacotherapy remains the first-line treatment for this condition. Additionally, vulvar care measures, discontinuing any medications associated with lichenoid changes, and psychologic support should be instituted.
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Erosive vulvar lichen planus is treated initially with ultrapotent topical corticosteroid ointments, such as 0.05-percent clobetasol propionate applied daily for up to 3 months, and then slowly tapered. Refractory cases are common and may respond to a preparation containing 0.05-percent clobetasol butyrate, 3-percent oxytetracycline, and 100,000 U/g nystatin (Trimovate) (Cooper, 2006). Used in small case series, other beneficial agents include systemic corticosteroids, topical tacrolimus ointment, topical cyclosporine, and oral retinoids (Byrd, 2004; Eisen, 1990; Hersle, 1982; Morrison, 2002).
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Vaginal Lichen Planus Treatment
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Commonly prescribed to treat hemorrhoids, corticosteroid suppositories containing 25 mg of hydrocortisone used vaginally are helpful—specifically, if used twice daily and then tapered to maintain symptom remission (Anderson, 2002). For poorly responding patients, compounding pharmacies can provide a 100-mg hydrocortisone suppository. Potent corticosteroids are prescribed judiciously, as systemic absorption may lead to adrenocorticosuppression (Moyal-Barracco, 2004a). Combining local corticosteroid therapy with vaginal dilator use may help restore coital function in patients with moderate vaginal synechiae.
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If topical medications fail, systemic treatment with prednisone 40 to 60 mg daily for up to 4 weeks may modulate symptoms (Moyal-Barracco, 2004a). Although no alternative systemic medications have been fully studied, methotrexate, hydroxychloroquine, and mycophenolate mofetil administered by providers familiar with their use are effective within a multidisciplinary approach (Eisen, 1993; Frieling, 2003; Lundqvist, 2002). Surgical adhesiolysis is a last resort. In general, vulvovaginal lichen planus is a chronic, recurrent disease for which symptomatic improvement is possible, but complete control is unlikely.
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Hidradenitis Suppurativa
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This chronic disease is manifested by recurrent papular lesions that may lead to abscess, fistula formation, and scarring predominantly in apocrine gland-bearing skin (Fig. 4-7). In order of frequency, affected areas include the axillae; inguinal, perianal, and perineal skin; inframammary regions; and retroauricular skin. Chronic inflammation obstructs skin follicles, with subsequent subcutaneous abscess formation, skin thickening, and deformity. Abscesses typically form sinus tracts, and the resulting disfigurement and chronic purulent drainage can be devastating physically, emotionally, and sexually.
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The etiology of hidradenitis suppurativa is unknown. More than one quarter of patients will report a family history of the disease, and an autosomal dominant inheritance pattern has been hypothesized (der Werth, 2000). Although Mortimer and colleagues (1986) found higher plasma concentrations of androgens in women with hidradenitis suppurativa, others have been unable to replicate this finding (Barth, 1996).
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Treatment of early cases includes local hygiene and weight reduction in patients who are obese along with topical or oral antibiotics and warm compresses. Used individually, appropriate long-term oral antibiotics and their dosages include: tetracycline, 500 mg twice daily; erythromycin, 500 mg twice daily; doxycycline, 100 mg twice daily; or minocycline, 100 mg twice daily. Topical 1-percent clindamycin solution applied twice daily may also be effective (Jemec, 1998). Additionally, a 10-week course of oral clindamycin, 300 mg twice daily, plus rifampicin, 600 mg twice daily, has shown efficacy (Gener, 2009).
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As reviewed by Rhode and associates (2008), an arsenal of other treatment modalities has been reported with varying efficacies. These include cyproterone acetate (an antiandrogen available in Europe), corticosteroids, isotretinoin, cyclosporine, and infliximab. An evidence-based review of pharmacologic interventions provided by Alhusayen (2012) suggests antibacterials and anti-tumor necrosis factor therapy are effective for hidradenitis. In late 2015, the FDA approved Humira (adalimumab) for the treatment of moderate to severe hidradenitis. Nonmedical therapies include laser and phototherapy. Severe, refractory cases may require surgical excision that often involves extensive resection of the vulva and surrounding areas. Plastic surgery techniques are often needed to close these large defects. Unfortunately, postoperative local recurrences can develop.
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Nearly 25 percent of women in the second and third decade of life will experience these self-limited mucosal lesions. Classically found on nonkeratinized oral mucosa, aphthous ulcers may also develop on vulvovaginal surfaces. Lesions are painful and can recur every few months. Distinguishing an aphthous ulcer from genital herpes may require appropriate cultures, serologies, and/or biopsies. Histologically, aphthous ulcers are composed of a mononuclear infiltrate with a fibrin coating. Although the etiology is unknown, some theorize the origin to be immune-mediated epithelial cell damage (Rogers, 1997). Other described triggers include stress, trauma, infection, hormonal fluctuation, and nutritional deficiencies of vitamin B12, folate, iron, or zinc (Torgerson, 2006). Despite the normally self-limited nature of these ulcers, persistent lesions can lead to painful scarring (Rogers, 2003). Clinicians should consider human immunodeficiency virus testing when aphthae are large and slow to heal.
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High-potency topical corticosteroids can be used at the onset of ulceration. Oral corticosteroids may be used to decrease inflammation in cases resistant to topical corticosteroids. Finally, colchicine, dapsone, and thalidomide have been shown to be effective, although they are rarely used.