CLINICAL OBSTETRICS AND GYNECOLOGY Volume 58, Number 3, 464–475 Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.
Inflammatory Vulvar Dermatoses ANGELA GUERRERO, BS,* and ARUNA VENKATESAN, MDw z *Stanford University School of Medicine; w Department of Dermatology, Stanford University, Redwood City, California; and z Department of Medicine, Division of Dermatology, Santa Clara Valley Medical Center, San Jose, California Abstract: Inflammatory vulvar dermatoses affect many women, but are likely underdiagnosed due to embarrassment and reluctance to visit a health care provider. Although itch and pain are common presenting symptoms, the physical examination can help distinguish between different disease entities. Because many women’s health providers have minimal training in the categorization and management of dermatologic disease, definitive diagnosis and management can be difficult. Herein, strategies for diagnosing vulvar lichen sclerosus, lichen planus, contact dermatitis, lichen simplex chronicus, and psoriasis are discussed along with basic management of these diseases, which commonly involves decreasing inflammation through behavioral change, gentle skin care, topical corticosteroids, and systemic therapies. Key words: vulva, lichen sclerosus, lichen planus, contact dermatitis, lichen simplex chronicus, psoriasis
Introduction When female patients experience vulvovaginal symptoms, such as pain, pruritus, and dyspareunia, they may present to a range of health care providers, including gynecologists, dermatologists, urologists, internists, and pediatricians. Women experiencing Correspondence: Aruna Venkatesan, MD, 751 South Bascom Avenue, Suite 510, San Jose, CA. E-mail: [email protected] The authors declare that they have nothing to disclose. CLINICAL OBSTETRICS AND GYNECOLOGY
464 | www.clinicalobgyn.com
/
these symptoms may later be diagnosed with inflammatory vulvar dermatologic conditions, including lichen sclerosus (LS), lichen planus (LP), lichen simplex chronicus (LSC), psoriasis, and contact dermatitis. The exact incidence and prevalence of each of these vulvar dermatoses is unknown since women may be initially reluctant to visit a health care provider for symptoms that can be associated with feelings of embarrassment. As each of these diseases can be aggravated by poor anogenital skin care, it is critical to become familiar with the basic principles of gentle skin care. Generally, all patients with vulvar dermatoses can benefit from gentle cleansing, use of topical emollients, and decreased contact with irritating or allergenic agents. An overview of vulvar dermatoses with principles for basic treatment and management will be discussed.
Basic Vulvar Skin Care Gentle skin care is the backbone of managing inflammatory vulvar dermatoses. Many of these conditions are worsened or even triggered by aggressive hygiene techniques that are often encouraged in US culture. All VOLUME 58
/
NUMBER 3
/
SEPTEMBER 2015
Inflammatory Vulvar Dermatoses chemical wipes, sprays, douches, and overthe-counter topical antibiotics and anesthetic preparations should be stopped. Washing of the anogenital skin when bathing should be done with plain water and hands only. If the patient desires, a mild cleanser for sensitive skin can be used but should be washed off with water. No scrubbing instruments such as loofah sponges, wash clothes, or fingernails should be used. If topical corticosteroid ointments are prescribed they should be applied directly to the involved skin, most effective when applied immediately after bathing, and a mirror should be used to show the patient the correct area to treat. If an emollient is desired, white petrolatum is recommended because of its lack of allergenicity and irritancy. White petrolatum can be applied several times during the day when a patient feel itch or irritation, or applied a few minutes after corticosteroid application. Sitz baths, cold compresses, and the use of a handheld shower head may be soothing. For some patients the use of tampons to collect menstrual blood may be less uncomfortable than using sanitary napkins, or vice versa. A good bowel regimen with increased dietary fiber can be helpful for patients with active perianal inflammation. The use of white cotton underwear and avoiding tight clothing has been recommended by some vulvologists. If a patient wants to attempt sexual intercourse once symptoms are improved, it is important that a slow, gently approach is used with ample time for arousal and natural lubrication. Silicone-based lubricants can significantly decrease friction and thus decrease chance of skin tearing. A patient should stop sexual intercourse if it is still painful despite taking these extra measures.
Scarring Dermatoses LS
Presentation LS is an inflammatory dermatosis that most commonly affects prepubertal girls
465
and postmenopausal women. The exact cause and pathogenesis of LS is unknown, but it is more prevalent in women with lower levels of estrogen, which correlates well with the typical age at presentation,1 and is also associated with other autoimmune diseases. It typically develops into a chronic skin disorder affecting the anogenital region, but may also have co-occurring or sole extragenital manifestations. Patients with LS typically present with intensely pruritic white plaques which can cause a patient to scratch herself and exacerbate her symptoms. Although the majority of patients with vulvar LS experience troublesome symptoms most notably pruritus, along with burning, dyspareunia, and dyschezia, a minority of them can be asymptomatic. Asymptomatic disease is likely even more underdiagnosed as these patients are usually only diagnosed by an astute clinician on routine well woman examination. Children with LS in contrast to adults most commonly present with constipation and dysuria, and are sometimes brought to medical attention for child abuse investigation because of often cooccurring petechiae, purpura, and fissures. These children may notice vulvovaginal irritation more prominently at nighttime and can experience night terrors along with other psychosocial issues.2 On physical examination, both acute and chronic manifestations of LS should be evaluated for and noted in clinic documentation. Genital LS classically occurs in a figure-of-eight or hourglass pattern around the vaginal and anal openings and is generally thought to spare the mucous membranes, although cases of oral and vaginal LS have been reported.1 Signs of active disease include white, atrophic plaques with cigarette-paper or tissue-paper wrinkling (a sign of epidermal atrophy), petechiae and purpura, fissures, and erosions (Figs. 1 and 2). Signs of chronic disease include clitoral hood phimosis and labia minora resorption or phimosis, which can be described as architectural www.clinicalobgyn.com
466
Guerrero and Venkatesan
FIGURE 2. Lichen sclerosus in a prepubertal child. Note the hypopigmentation in an hourglass distribution, the petechiae and purpura on the perineal body and labia minora, and the radially distributed fissures around the anus. FIGURE 1. Lichen sclerosus in a postmenopausal woman. Note the hypopigmentation and cigarette-paper wrinkling of epidermal atrophy in an hourglass distribution. You can also see subtle involvement of the inguinal or labiocrural folds where the labia majora and inner thighs meet.
change or scarring. Patients may describe these findings as ‘‘losing my clitoris or lips’’ or ‘‘my vagina is shrinking.’’ This architectural change does not reverse with treatment of LS in adults, but will often dramatically reverse with treatment in children. Conducting a physical examination for LS in children is usually easily managed by asking the child to lie supine on the table in a ‘‘butterfly’’ or ‘‘frogs legs’’ position, in which the knees are flexed and hips externally rotated with the soles resting in opposition. No stirrups are required for this positioning.2 For younger children, the patient may be held in a parent’s lap. It is important to address with the child why a genital examination by someone other than a guardian is acceptable in this case.2,3 A speculum www.clinicalobgyn.com
examiantion in a child is unnecessary when evaluating for LS. Diagnostic Studies Although a thorough history and physical examination is often sufficient for diagnosing LS, one or more skin punch biopsies of the vulva can be taken to confirm the diagnosis if there is doubt or if cooccurring differentiated vulvar intraepithelial neoplasia (VIN) or squamous cell carcinoma (SCC) is suspected, which can occur in patients with long-standing LS.4 It is best to take more than one biopsy if there are areas with different morphologies (eg, a white thin plaque and an eroded nodule, etc.). Previously, LS was called ‘‘lichen sclerosus et atrophicus’’; however, it has been shown histopathologically that not all LS biopsy samples have signs of atrophy.1 A biopsy may also be indicated for persistently affected areas that are refractory to treatment to rule out the development of dysplasia over time. In children, a biopsy is rarely indicated as examination findings in this age group are
Inflammatory Vulvar Dermatoses generally very classic. Because topical estrogens and corticosteroids used for treating this condition increase the risk of superinfection, cultures for microorganisms and/or empiric treatment for yeast and/or bacteria should be performed if there are clinical signs of infection. Management First-line treatment for LS involves the application of an ultra potent (class I) topical corticosteroid, most commonly clobetasol propionate 0.05%, which is very efficacious at controlling disease activity. Ointment is best as it is least irritating and a better barrier for inflamed skin. The frequency of its application should be slowly tapered at subsequent clinic visits to the lowest frequency of use that still controls symptoms and signs of active disease. Alternatively, management may consist of lowering the potency of corticosteroid while keeping the frequency of application relatively constant (eg, changing from a class I corticosteroid [eg, clobetasol propionate 0.05% ointment] to a class II [eg, fluocinonide 0.05% ointment] to a class IV [eg, triamcinolone 0.1% ointment], etc.). Although there are no clear guidelines on how to taper, most vulvologists typically begin with once to twice a day corticosteroid application and taper at each follow-up visit every 2 to 3 months if symptoms and clinical signs of active disease are minimal. Tapering generally will start from twice a day application to once a day application to 3 times a week application to 2 times a week application to once a week application to a trial-off of therapy. At any point, if symptoms or clinical signs of disease activity flare, the patient should resume more frequent use and then taper again as instructed by their clinician. Patients should be counseled that LS is generally a chronic disease without a true cure; however, activity can be very well managed with topical immunosuppressants. Alternatives to topical corticosteroids
467
include topical calcineurin inhibitors, most commonly topical tacrolimus 0.1% ointment, although its associated black box warning and possible stinging sensation with application should be discussed before prescribing.4 Adjunctive therapy in postmenopausal women usually consists of a local intravaginal topical estrogen, if there are no contraindications to its use. Lysis of adhesions may be indicated if scarring has led to functional limitations such as dyspareunia or caused urinary retention or increased frequency of urinary tract infections. This procedure should be done by an experienced physician and only after the disease is well controlled. A discussion of anogenital LS management is not complete without addressing the reported association with VIN/SCC. Sixty percent of cases of vulvar SCC appear to develop in the context of LS. Within western communities, the incidence of vulvar SCC has been reported to be 1.5 per 100,000 women.5 This increased risk of SCC should be discussed with patients upon diagnosis of LS and used as a means to encourage the patient to continue regular follow-up with a care provider familiar with LS and the clinical signs of VIN/SCC. There should be a low threshold to biopsy suspicious areas, such as nodules, bleeding lesions, hard lesions, red lesions, or areas of LS that are refractory to treatment. VIN associated with LS (the differentiated-type) has a higher risk of progression to SCC than HPV-associated VIN and treatment is usually surgical.6 LP
Presentation LP is a T-cell–mediated inflammatory disorder that affects mucous membranes, including the mouth and vagina, as well as the scalp, skin, and nails. Irreversible scarring can occur in the erosive form of the mucosa as well as with nail and scalp disease resulting in pain and significant psychosocial distress. Women with the www.clinicalobgyn.com
468
Guerrero and Venkatesan
FIGURE 4. Oral erosive lichen planus (LP) with involvement of the marginal gingiva, a classic site of involvement in LP but not a finding specific to LP. Autoimmune bullous diseases such as pemphigus vulgaris and mucous membrane pemphigus can also show this finding; however, if Wickham striae are also found on the buccal mucosa this is pathognomonic for LP.
FIGURE 3. Oral erosive lichen planus. Note the pathognomonic Wickham striae (a white reticulated or net-like plaque) located on the right upper buccal mucosa.
erosive form of vulvar LP commonly present with vulvar pain, dyspareunia, and dysuria, whereas women with the nonerosive vulvar form commonly present with severe itch.7,8 Patients with vaginal LP may present with copious yellow to yellowgreen vaginal discharge, due to a desquamative vaginitis. In contrast to LS, LP affects mostly perimenopausal and postmenopausal women but rarely affects children. LP similar to LS can be also associated with other autoimmune diseases, such as vitiligo, pernicious anemia, alopecia areata, and thyroid disease.8 It is clinically important to note that erosive LP of the vulvovaginal tissue is most commonly associated with oral erosive disease, termed the vulvovaginal gingival syndrome, thus necessitating an oropharyngeal examination in all patients with genital LP.7 www.clinicalobgyn.com
Oropharyngeal involvement may reveal Wickham striae on the buccal mucosa, erosions of the marginal gingiva, atrophic papillae on the tongue’s surface, or erosions on the tongue (Figs. 3 and 4). Vulvar LP can present with different morphologies, including classical (papulosquamous), erosive, hypertrophic, and mixed subtypes.9 Papulosquamous LP of the vulva presents as small, poorly demarcated pink-violaceous papules or plaques, which often demonstrate an overlying white reticulated or net-like plaque termed Wickham striae that is pathognomonic for LP. The most common form of vulvar LP is erosive, and erosions may be seen on the introitus, clitoris, and labia minora (Fig. 5).7 Diagnostic Studies Much like with LS, the diagnosis of LP is primarily made based on the history and physical examination; however, biopsies are more often needed in this entity because erosions can be seen in VIN/SCC as
Inflammatory Vulvar Dermatoses
FIGURE 5. Vulvar erosive lichen planus. Note the erosion medial to the left labium minus, the resorption of the right labium minus, and the adherent yellow-green discharge.
well as in autoimmune blistering diseases, such as pemphigus vulgaris and mucous membrane pemphigoid. A biopsy can be performed if there is doubt regarding the diagnosis of LP, especially if there are no Wickham striae or co-occurring classic findings of oral LP, or if there are hyperkeratotic or nodular lesions concerning for VIN or HPV-negative SCC, which has been associated with LP.8 A shave or punch biopsy may be performed as long as it adequately samples the dermis or submucosa. A biopsy at the edge of an erosion often gives the most diagnostic findings for the pathologist. Most commonly a specimen submitted in formalin for H&E analysis is sufficient, but when attempting to rule out an autoimmune bullous disease, a second sample should be submitted in Zeus or Michel’s medium for direct immunofluorescence. Histopathological review by both a surgical pathologist and a dermatopathologist is ideal.
469
Examination of the vulva, peri-anal skin, and vagina via a small speculum should be performed to look for signs of active disease (erosions, Wickham striae, purulent vaginal discharge) and chronic disease (scarring of the clitoral hood, labia minora, or vaginal canal). If resistance is felt or pain elicited on speculum examination, a lubricated single gloved digit can be gently placed inside to feel for strictures or tumors. Occasionally, an examination under anesthesia may be needed if a complete examination in clinic is limited by pain. For some patients, a vaginal examination may be deferred to the next follow-up visit if vulvar disease control is needed first to improve tolerance to speculum placement. On follow-up visits, patients with LP should be regularly assessed for signs of malignancy with a low threshold to biopsy lesions refractory to treatment similarly to that which was discussed for LS above.8 Hepatitis C virus (HCV) is most associated with the erosive subtype of LP; thus, checking HCV antibody titers and asking the patient about a history of hepatitis C is important. Of note, recent literature publications demonstrate that LP is truly a multiorgan disease that can cause symptoms of ocular tearing, hearing loss, pain with eating acidic, spicy, or caffeinated foods, odynophagia, dysphagia, painful defecation, and dysuria with decreased urinary stream. If a patient with suspected vulvovaginal LP reports any of these symptoms, it is fruitful to refer the patient to the appropriate specialist for evaluation. Management Gentle skin care is the foundation for managing genital LP. Medications that can trigger lichenoid drug eruptions, including NSAIDs, diuretics, and antihypertensives should be elicited on patient history and consideration for drug holiday with close involvement from a primary care provider can be considered, www.clinicalobgyn.com
470
Guerrero and Venkatesan
although this may or may not alter disease course. Treating erosive vulvar LP with class I topical corticosteroids such as clobetasol propionate 0.05% or betamethasone dipropionate 0.05% ointments should be attempted first. Particularly for hypertrophic lesions that are unresponsive to potent steroids, a biopsy must be taken to evaluate the lesion for malignancy. Additional therapies that can be used to treat LP lesions include topical calcineurin inhibitors, most commonly tacrolimus 0.1% ointment. Tapering protocols are similar to those mentioned above for LS. Intralesional triamcinolone acetonide injections can also be used especially in thicker lesions or to establish initial disease control in patients who may not tolerate topical preparations initially because of discomfort.1 Adjunctive therapy in postmenopausal women usually consists of an intravaginal local topical estrogen, if there are no contraindications to its use. For vaginal LP, a commercial hydrocortisone 25 mg rectal suppository may be used intravaginally on a nightly basis; however, this may not be sufficient to achieve disease control. Some vulvologists compound a hydrocortisone 300 mg suppository to be used intravaginally on a nightly basis. Alternatively, 0.5 gm or 1 gm of clobetasol propionate 0.05% ointment or fluocinonide 0.05% ointment may be inserted into the vagina with a vaginal applicator; however, this should only be done to achieve disease control on a short-term basis because of the risk of systemic absorption of the medication and subsequent hypothalamic-pituitary-adrenal axis suppression. Intravaginal corticosteroid use also increases the risk of candida, dermatophyte, and herpes infection; thus, the provider should monitor closely and have suspicion for superinfection if the patient calls or presents with a flare of symptoms after starting therapy. For severe disease refractory to treatment with topical corticosteroids, a short course of systemic steroids such as prednisone may www.clinicalobgyn.com
be given. Due to the side effects associated with long-term prednisone use, different agents are preferred for long-term control of severe LP. These steroid-sparing immunosuppressants include methotrexate, mycophenolate mofetil, azathioprine, cyclosporine, and hydroxychloroquine. Systemic immunomodulation should also be considered early in the disease course for patients with co-occurring LP on other body sites such as scarring alopecia of the scalp (lichen planopilaris) or scarring nail disease. Surgical management may be considered for patients with symptomatic labial adhesions who have adequate disease control. Lysis of adhesions may be helpful for decreasing dyspareunia, improving the flow of urine, and decreasing the risk of urinary tract infection.10 Due to the risk of developing VIN/SCC in association with vulvovaginal LP, patients should receive regular follow-up. Case reports and case series have demonstrated a 1% to 2.5% incidence of SCC with about 5 years of follow-up. Given the chronic nature of disease, multiple subsequent biopsies may be taken of hyperkeratotic areas and those refractory to treatment. Surveillance for cancer can be challenging and strictures may limit vaginal examination. In some cases an exam under anesthesia may be warranted, with appropriate imaging if warranted.
Nonscarring Dermatoses CONTACT DERMATITIS
Presentation Contact dermatitis may occur on any cutaneous surface and can be subdivided into irritant and allergic forms. Irritant contact dermatitis to a particular substance occurs in all people with sufficient exposure to that substance and is not immunologically mediated. It usually
Inflammatory Vulvar Dermatoses causes irritation and pain and occurs fairly quickly in response to exposure. In contrast allergic contact dermatitis to a particular allergen occurs in a subset of the population that is sensitized to the allergen, is immunologically mediated with a delayed reaction after exposure, and is associated with symptoms of itch and sometimes irritation or pain when very exuberant. Maceration decreases the threshold for allergen sensitization, so the anogenital region’s moist environment promotes the development of contact dermatitis. Heat, urine, and fecal incontinence, and excessive washing are common culprits for irritant contact dermatitis of the vulva. Topical benzocaine cream, topical antibiotics (such as neomycin, polymixin, bacitracin), preservatives, and lanolin are just a sampling of the allergens that can cause allergic contact dermatitis of the vulva.11 Some of these medications may have been used as a result of the patient trying to relieve her symptoms with over-the-counter products marketed to help vulvovaginal symptoms. Thus, when taking a thorough history of a patient with suspected contact dermatitis, it is necessary to ask about exposures to these triggering substances. If a woman’s regular hygiene routine includes douches, soaps, menstrual pads, and baby wipes, each of these should be evaluated as a potential cause for contact dermatitis. Wearing constrictive clothing, noncotton undergarments, and Lycra can generate excessive heat around the vulva, leading to sweating, which may promote chafing, the development of a contact dermatitis, and or superinfection with yeast. Other common vulvar irritants include condoms, lubricants, and spermicides.9 For acute contact dermatitis, physical examination can reveal erythema throughout the vulva, which can extend to the perianal skin and inner thighs (Fig. 6). Vesicles, bullae, and erosions, sometimes in a linear configuration, may be found if an exuberant reaction has occurred. Evidence
471
FIGURE 6. Vulvar contact dermatitis to a topical benzocaine cream.
of excoriation can be present along with fissures and scaling. Diagnostic Studies Contact dermatitis is diagnosed primarily based on the clinical examination and appropriate history. If a biopsy is performed, it will show spongiotic dermatitis with epidermal vesicles; eosinophils are commonly present in the allergic form. Sometimes patch testing can help identify a precise culprit allergen, thus referral to a dermatologist who conducts patch testing may be warranted if disease control is suboptimal. As contact dermatitis can develop after or during a vaginal infection, one can consider microscopy with pH testing of vaginal discharge and vaginal cultures to determine whether the patient concurrently has candidiasis, bacterial vaginosis, or trichomoniasis. These infections should not be overlooked as they will require additional management.9 Management Since contact dermatitis results from the skin being in contact with irritants or allergens, the first step in management is to ensure that the aggravating factors are removed. Women should be advised to stop douching or excessive cleansing of the vulva. Other measures such as the use of cold packs, oral antihistamines, and sitz baths will decrease pruritus and thus www.clinicalobgyn.com
472
Guerrero and Venkatesan
decrease scratching, which will help the epidermis to heal more quickly. A sitz bath twice daily with lukewarm water followed by a medium to ultrapotent topical steroid, followed by application of white petrolatum can restore the barrier function of the skin and calm the inflammation.12 For severe cases, oral prednisone may be needed to control symptoms with a 2- to 3-week taper to prevent a rebound flare. If there is evidence of secondary infection, appropriate antifungal, or antibiotic treatment must be started as guided by cultures and sensitivities. Secondary candida albicans infection can be controlled with oral fluconazole.12 LSC
Presentation LSC is generally a nonscarring process of epidermal thickening that develops as a result of repeated excoriation and rubbing from a myriad of triggers. LSC can occur anywhere on the body including genital skin such as the labia majora in women, the scrotum in men, and the perianal skin. Any disorder that causes itch can lead to the development of LSC including atopic dermatitis, contact dermatitis, and recurrent yeast infections. Additional triggers for LSC can include primary neurologic dysfunction or primary psychiatric illness leading to repetitive selfexcoriation.13 When a patient repetitively scratches or rubs a pruritic area of skin over time, lichenification (epidermal thickening and accentuation of skin markings) will develop and will be seen on physical examination as ‘‘leathery’’ or ‘‘bark-like’’ poorly demarcated, thick plaques with scale (Fig. 7).9,13 Other secondary changes associated with LSC include hyperpigmentation or hypopigmentation; depigmented scars can occur if the patient excoriates into the dermis.13 Diagnostic Studies LSC is usually a clinical diagnosis that can be achieved by eliciting a history of chronic www.clinicalobgyn.com
FIGURE 7. Lichen simplex chronicus with hyperpigmented, lichenified plaques over the labia majora and medial thighs.
rubbing and scratching and physical examination demonstrating lichenified hairbearing skin. If a biopsy is performed it should demonstrate irregular acanthosis (thickening of the epidermis). Parakeratosis, a superficial perivascular infiltrate, and superinfection with yeast or bacteria can also be seen on histopathologic analysis.9 Management LSC is a chronic disease that often does not have a true cure. The goal of management is to decrease the severity of symptoms by decreasing the rubbing and scratching. Counseling the patient about the itch-scratch cycle is paramount. Oral antipruritics are often the cornerstone of management, such as hydroxyzine or doxepin at night, being mindful of the side effects of sedation of both medications and the medication interactions of doxepin with some psychotropic medications, as doxepin is both an antihistamine and a tricyclic antidepressant.14 Management of LSC must involve eliminating triggers by using gentle skin care and avoiding culprit irritants and allergens. Again, patients can be advised to soak the affected areas in a lukewarm bath before applying a medium to ultrapotent topical corticosteroid followed by a gentle emollient, such as white petrolatum. The goal over time is to decrease the potency of corticosteroid prescribed once the LSC plaques
Inflammatory Vulvar Dermatoses
473
become thinner and return closer to normal epidermal thickness. Eventually patients may be able to control symptoms using only a low to medium potency corticosteroid during flares of pruritus.14 Patients with neurologic or psychiatric triggers for LSC should be referred to the appropriate specialists. PSORIASIS
Presentation Psoriasis is a relatively common chronic inflammatory skin disease of epidermal keratinocyte hyperproliferation and Tcell activation. About 15% of patients with cutaneous psoriasis also develop an inflammatory, sometimes destructive arthritis and enthesitis. In the general population, the prevalence of psoriasis is estimated at 1% to 2%, and isolated genital psoriasis is estimated to occur in 2% to 5% of individuals affected with the disease.15 All forms of cutaneous psoriasis can affect the genital skin including plaque-type, pustular, and inverse psoriasis. Psoriasis can be itchy, but itch is not a hallmark of disease.16 Physical examination of the vulva reveals well-demarcated pink plaques that can extend to the perianal skin (Figs. 8 and 9). Although psoriasis on the body
FIGURE 8. Vulvar psoriasis in a child. Note the well-demarcated nature of the plaque.
FIGURE 9. Vulvar and perianal psoriasis in a child.
often demonstrates a classic silvery micaceous scale, genital psoriasis often is scalefree because of the external genitalia’s naturally moist environment.
Diagnostic Studies Psoriasis is generally a clinical diagnosis. Looking for plaques elsewhere on the body can help confirm diagnosis; classic sites of involvement include the scalp, elbows, knees, gluteal cleft, and nail plates. Nail involvement can manifest clinically as nail pitting, oil spots, or elevation of the distal nail plate from the bed called distal onycholysis. A skin biopsy can be performed if the diagnosis is in question and usually shows regular acanthosis (epidermal thickening) with parakeratosis, hypogranulosis, thinned suprapapillary plates, dilated capillaries in the dermal papillae, and neutrophils in the corneal layer. Pustular psoriasis shows subcorneal pustules on biopsy.16 www.clinicalobgyn.com
474
Guerrero and Venkatesan
Management Like most inflammatory vulvar dermatoses psoriasis has no cure, but disease activity and associated symptoms can be controlled with a combination of medications. Patients with psoriasis are advised to establish a gentle skin care regimen. Psoriasis is typically treated with low to medium potency topical corticosteroids, such as hydrocortisone 2.5%, desonide, or triamcinolone 0.1% ointment. Chronic topical corticosteroid use especially in the body folds, where their effects are potentiated by occlusion, can lead to skin atrophy, easy bruising, striae, and telangiectasias. To decrease the risk of these side effects, medications such as topical vitamin D analogues (eg, calcipotriene 0.005%) or topical calcineurin inhibitors (eg, tacrolimus 0.1% ointment) may be used along with or as an alternative to topical corticosteroids. If vulvar plaques do not respond to these medications, a biopsy should be taken to evaluate for malignancy.17 In addition, for patients experiencing severe pruritus, an oral antihistamine can bring relief. The Koebner phenomenon describes how lesions can develop at sites of trauma, and this occurs not only in psoriasis, but in LS and LP as well.17 To prevent further development of these lesions, minimizing trauma to the skin is necessary. For instance, long distance cyclers or horseback riders may develop plaques over time in areas with repeated trauma. Since patients with cutaneous psoriasis may also develop psoriatic arthritis, providers must inquire about joint symptoms and refer these patients to a rheumatologist if appropriate.
Summary Inflammatory vulvar dermatoses can severely impact the quality of a woman’s life, and it is critical to accurately diagnose these entities so that tailored management can be recommended. Providers must www.clinicalobgyn.com
hone in on key findings in the history and physical examination to guide diagnosis, with skin biopsy serving as a useful tool when the diagnosis is in question. In general, the presence of scarring can be used to divide these diseases into 2 groups: scarring dermatoses (LS and LP) and nonscarring dermatoses (contact dermatitis, LSC, and psoriasis). For scarring dermatoses, notable differences between LS and LP include the affected age groups (LP is rare in prepubertal children) and physical examination findings (thin, hypopigmented cigarette-paper skin in LS vs. eroded plaques in LP). For nonscarring dermatoses, the clinical history can be telling, as patients with contact dermatitis or LSC may have experienced intense pruritus and may have a history of atopic disease. Psoriasis is classically diagnosed based on its presentation as well-demarcated plaques, with often involvement of the elbows, knees, and scalp with overlying silver scale. For all of these inflammatory vulvar dermatoses, gentle skin care must be taught. Even once a diagnosis has been established, consideration for skin biopsy and/or microbial cultures for refractory disease may be warranted to identify iatrogenic infections or the development of VIN or SCC. Topical corticosteroids of varying potency or systemic therapies are often prescribed. Arranging regular follow-up visits, particularly for diseases with an increased malignant potential, is necessary to ensure optimal response to treatment and to perform appropriate surveillance for malignancy.
References 1. Val I, Gutemberg A. An overview of lichen sclerosus. Clin Obstet Gynecol. 2005;48:808–817. 2. Epstein AG. Gynecological examination of a prepubertal child [Armenian medical Network web site]. Available at: http://www.health.am/ gyneco/more/gynecological-examination-of-aprepubertal-child/. Accessed November 2, 2014. 3. Davis AJ, Katz VL. Pediatric and Adolescent Gynecology. In: Lentz GM, ed. Comprehensive
Inflammatory Vulvar Dermatoses
4.
5. 6.
7. 8.
9. 10.
Gynecology, 6th ed. Philadelphia: Elsevier Mosby; 2012:199–213. Van de Nieuwenhof H, Bulten J, Hollema H, et al. Differentiated vulvar intraepithelial neoplasia is often found in lesions, previously diagnosed as lichen sclerosus, which have progressed to vulvar squamous cell carcinoma. Mod Pathol. 2011;24: 297–305. Murphy R. Lichen sclerosus. Dermatol Clin. 2010; 28:707–715. Brodrick B, Belkin ZR, Goldstein AT. Influence of treatments on prognosis for vulvar lichen sclerosus: facts and controversies. Clin Dermatol. 2013;13:780–786. Goldstein AT, Metz A. Vulvar lichen planus. Clin Obstet Gynecol. 2005;48:818–823. Regauer S, Reich O, Eberz B. Vulvar cancers in women with vulvar lichen planus: a clinicopathological study. J Am Acad Dermatol. 2014;71: 698–707. Rodriguez MI, Leclair CM. Benign vulvar dermatoses. Obstet Gynecol Surv. 2012;67:55–63. Suzuki V, Haefner HK, Piper CK, et al. Postoperative sexual concerns and functioning in patients who underwent lysis of vulvovaginal
11. 12. 13.
14.
15.
16.
17.
475
adhesions. J Low Genit Tract Dis. 2013;17: 33–37. Schlosser BJ. Contact dermatitis of the vulva. Dermatol Clin. 2010;28:697–706. Margesson LJ. Contact dermatitis of the vulva. Dermatol Ther. 2004;17:20–27. Lynch PJ. Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region. Dermatol Ther. 2004;17:8–19. Thorstensen KA, Birenbaum DL. Recognition and management of vulvar dermatologic conditions: lichen sclerosus, lichen planus, and lichen simplex chronicus. J Midwifery Womens Health. 2012;57:260–275. Meeuwis KAP, De Hullu JA, Massuger LFAG, et al. Genital psoriasis: a systematic literature review on this hidden skin disease. Acta Derm Venereol. 2011;91:5–11. Murphy M, Kerr P, Grant-Kels JM. The histopathologic spectrum of psoriasis. Clin Dermatol. 2007;25:524–528. Guglielmetti A, Conlledo R, Bedoya J, et al. Inverse psoriasis involving genital skin folds: successful therapy with dapsone. Dermatol Ther. 2012;2:15–23.
www.clinicalobgyn.com
Inflammatory Vulvar Dermatoses ANGELA GUERRERO, BS,* and ARUNA VENKATESAN, MDw z *Stanford University School of Medicine; w Department of Dermatology, Stanford University, Redwood City, California; and z Department of Medicine, Division of Dermatology, Santa Clara Valley Medical Center, San Jose, California Abstract: Inflammatory vulvar dermatoses affect many women, but are likely underdiagnosed due to embarrassment and reluctance to visit a health care provider. Although itch and pain are common presenting symptoms, the physical examination can help distinguish between different disease entities. Because many women’s health providers have minimal training in the categorization and management of dermatologic disease, definitive diagnosis and management can be difficult. Herein, strategies for diagnosing vulvar lichen sclerosus, lichen planus, contact dermatitis, lichen simplex chronicus, and psoriasis are discussed along with basic management of these diseases, which commonly involves decreasing inflammation through behavioral change, gentle skin care, topical corticosteroids, and systemic therapies. Key words: vulva, lichen sclerosus, lichen planus, contact dermatitis, lichen simplex chronicus, psoriasis
Introduction When female patients experience vulvovaginal symptoms, such as pain, pruritus, and dyspareunia, they may present to a range of health care providers, including gynecologists, dermatologists, urologists, internists, and pediatricians. Women experiencing Correspondence: Aruna Venkatesan, MD, 751 South Bascom Avenue, Suite 510, San Jose, CA. E-mail: [email protected] The authors declare that they have nothing to disclose. CLINICAL OBSTETRICS AND GYNECOLOGY
464 | www.clinicalobgyn.com
/
these symptoms may later be diagnosed with inflammatory vulvar dermatologic conditions, including lichen sclerosus (LS), lichen planus (LP), lichen simplex chronicus (LSC), psoriasis, and contact dermatitis. The exact incidence and prevalence of each of these vulvar dermatoses is unknown since women may be initially reluctant to visit a health care provider for symptoms that can be associated with feelings of embarrassment. As each of these diseases can be aggravated by poor anogenital skin care, it is critical to become familiar with the basic principles of gentle skin care. Generally, all patients with vulvar dermatoses can benefit from gentle cleansing, use of topical emollients, and decreased contact with irritating or allergenic agents. An overview of vulvar dermatoses with principles for basic treatment and management will be discussed.
Basic Vulvar Skin Care Gentle skin care is the backbone of managing inflammatory vulvar dermatoses. Many of these conditions are worsened or even triggered by aggressive hygiene techniques that are often encouraged in US culture. All VOLUME 58
/
NUMBER 3
/
SEPTEMBER 2015
Inflammatory Vulvar Dermatoses chemical wipes, sprays, douches, and overthe-counter topical antibiotics and anesthetic preparations should be stopped. Washing of the anogenital skin when bathing should be done with plain water and hands only. If the patient desires, a mild cleanser for sensitive skin can be used but should be washed off with water. No scrubbing instruments such as loofah sponges, wash clothes, or fingernails should be used. If topical corticosteroid ointments are prescribed they should be applied directly to the involved skin, most effective when applied immediately after bathing, and a mirror should be used to show the patient the correct area to treat. If an emollient is desired, white petrolatum is recommended because of its lack of allergenicity and irritancy. White petrolatum can be applied several times during the day when a patient feel itch or irritation, or applied a few minutes after corticosteroid application. Sitz baths, cold compresses, and the use of a handheld shower head may be soothing. For some patients the use of tampons to collect menstrual blood may be less uncomfortable than using sanitary napkins, or vice versa. A good bowel regimen with increased dietary fiber can be helpful for patients with active perianal inflammation. The use of white cotton underwear and avoiding tight clothing has been recommended by some vulvologists. If a patient wants to attempt sexual intercourse once symptoms are improved, it is important that a slow, gently approach is used with ample time for arousal and natural lubrication. Silicone-based lubricants can significantly decrease friction and thus decrease chance of skin tearing. A patient should stop sexual intercourse if it is still painful despite taking these extra measures.
Scarring Dermatoses LS
Presentation LS is an inflammatory dermatosis that most commonly affects prepubertal girls
465
and postmenopausal women. The exact cause and pathogenesis of LS is unknown, but it is more prevalent in women with lower levels of estrogen, which correlates well with the typical age at presentation,1 and is also associated with other autoimmune diseases. It typically develops into a chronic skin disorder affecting the anogenital region, but may also have co-occurring or sole extragenital manifestations. Patients with LS typically present with intensely pruritic white plaques which can cause a patient to scratch herself and exacerbate her symptoms. Although the majority of patients with vulvar LS experience troublesome symptoms most notably pruritus, along with burning, dyspareunia, and dyschezia, a minority of them can be asymptomatic. Asymptomatic disease is likely even more underdiagnosed as these patients are usually only diagnosed by an astute clinician on routine well woman examination. Children with LS in contrast to adults most commonly present with constipation and dysuria, and are sometimes brought to medical attention for child abuse investigation because of often cooccurring petechiae, purpura, and fissures. These children may notice vulvovaginal irritation more prominently at nighttime and can experience night terrors along with other psychosocial issues.2 On physical examination, both acute and chronic manifestations of LS should be evaluated for and noted in clinic documentation. Genital LS classically occurs in a figure-of-eight or hourglass pattern around the vaginal and anal openings and is generally thought to spare the mucous membranes, although cases of oral and vaginal LS have been reported.1 Signs of active disease include white, atrophic plaques with cigarette-paper or tissue-paper wrinkling (a sign of epidermal atrophy), petechiae and purpura, fissures, and erosions (Figs. 1 and 2). Signs of chronic disease include clitoral hood phimosis and labia minora resorption or phimosis, which can be described as architectural www.clinicalobgyn.com
466
Guerrero and Venkatesan
FIGURE 2. Lichen sclerosus in a prepubertal child. Note the hypopigmentation in an hourglass distribution, the petechiae and purpura on the perineal body and labia minora, and the radially distributed fissures around the anus. FIGURE 1. Lichen sclerosus in a postmenopausal woman. Note the hypopigmentation and cigarette-paper wrinkling of epidermal atrophy in an hourglass distribution. You can also see subtle involvement of the inguinal or labiocrural folds where the labia majora and inner thighs meet.
change or scarring. Patients may describe these findings as ‘‘losing my clitoris or lips’’ or ‘‘my vagina is shrinking.’’ This architectural change does not reverse with treatment of LS in adults, but will often dramatically reverse with treatment in children. Conducting a physical examination for LS in children is usually easily managed by asking the child to lie supine on the table in a ‘‘butterfly’’ or ‘‘frogs legs’’ position, in which the knees are flexed and hips externally rotated with the soles resting in opposition. No stirrups are required for this positioning.2 For younger children, the patient may be held in a parent’s lap. It is important to address with the child why a genital examination by someone other than a guardian is acceptable in this case.2,3 A speculum www.clinicalobgyn.com
examiantion in a child is unnecessary when evaluating for LS. Diagnostic Studies Although a thorough history and physical examination is often sufficient for diagnosing LS, one or more skin punch biopsies of the vulva can be taken to confirm the diagnosis if there is doubt or if cooccurring differentiated vulvar intraepithelial neoplasia (VIN) or squamous cell carcinoma (SCC) is suspected, which can occur in patients with long-standing LS.4 It is best to take more than one biopsy if there are areas with different morphologies (eg, a white thin plaque and an eroded nodule, etc.). Previously, LS was called ‘‘lichen sclerosus et atrophicus’’; however, it has been shown histopathologically that not all LS biopsy samples have signs of atrophy.1 A biopsy may also be indicated for persistently affected areas that are refractory to treatment to rule out the development of dysplasia over time. In children, a biopsy is rarely indicated as examination findings in this age group are
Inflammatory Vulvar Dermatoses generally very classic. Because topical estrogens and corticosteroids used for treating this condition increase the risk of superinfection, cultures for microorganisms and/or empiric treatment for yeast and/or bacteria should be performed if there are clinical signs of infection. Management First-line treatment for LS involves the application of an ultra potent (class I) topical corticosteroid, most commonly clobetasol propionate 0.05%, which is very efficacious at controlling disease activity. Ointment is best as it is least irritating and a better barrier for inflamed skin. The frequency of its application should be slowly tapered at subsequent clinic visits to the lowest frequency of use that still controls symptoms and signs of active disease. Alternatively, management may consist of lowering the potency of corticosteroid while keeping the frequency of application relatively constant (eg, changing from a class I corticosteroid [eg, clobetasol propionate 0.05% ointment] to a class II [eg, fluocinonide 0.05% ointment] to a class IV [eg, triamcinolone 0.1% ointment], etc.). Although there are no clear guidelines on how to taper, most vulvologists typically begin with once to twice a day corticosteroid application and taper at each follow-up visit every 2 to 3 months if symptoms and clinical signs of active disease are minimal. Tapering generally will start from twice a day application to once a day application to 3 times a week application to 2 times a week application to once a week application to a trial-off of therapy. At any point, if symptoms or clinical signs of disease activity flare, the patient should resume more frequent use and then taper again as instructed by their clinician. Patients should be counseled that LS is generally a chronic disease without a true cure; however, activity can be very well managed with topical immunosuppressants. Alternatives to topical corticosteroids
467
include topical calcineurin inhibitors, most commonly topical tacrolimus 0.1% ointment, although its associated black box warning and possible stinging sensation with application should be discussed before prescribing.4 Adjunctive therapy in postmenopausal women usually consists of a local intravaginal topical estrogen, if there are no contraindications to its use. Lysis of adhesions may be indicated if scarring has led to functional limitations such as dyspareunia or caused urinary retention or increased frequency of urinary tract infections. This procedure should be done by an experienced physician and only after the disease is well controlled. A discussion of anogenital LS management is not complete without addressing the reported association with VIN/SCC. Sixty percent of cases of vulvar SCC appear to develop in the context of LS. Within western communities, the incidence of vulvar SCC has been reported to be 1.5 per 100,000 women.5 This increased risk of SCC should be discussed with patients upon diagnosis of LS and used as a means to encourage the patient to continue regular follow-up with a care provider familiar with LS and the clinical signs of VIN/SCC. There should be a low threshold to biopsy suspicious areas, such as nodules, bleeding lesions, hard lesions, red lesions, or areas of LS that are refractory to treatment. VIN associated with LS (the differentiated-type) has a higher risk of progression to SCC than HPV-associated VIN and treatment is usually surgical.6 LP
Presentation LP is a T-cell–mediated inflammatory disorder that affects mucous membranes, including the mouth and vagina, as well as the scalp, skin, and nails. Irreversible scarring can occur in the erosive form of the mucosa as well as with nail and scalp disease resulting in pain and significant psychosocial distress. Women with the www.clinicalobgyn.com
468
Guerrero and Venkatesan
FIGURE 4. Oral erosive lichen planus (LP) with involvement of the marginal gingiva, a classic site of involvement in LP but not a finding specific to LP. Autoimmune bullous diseases such as pemphigus vulgaris and mucous membrane pemphigus can also show this finding; however, if Wickham striae are also found on the buccal mucosa this is pathognomonic for LP.
FIGURE 3. Oral erosive lichen planus. Note the pathognomonic Wickham striae (a white reticulated or net-like plaque) located on the right upper buccal mucosa.
erosive form of vulvar LP commonly present with vulvar pain, dyspareunia, and dysuria, whereas women with the nonerosive vulvar form commonly present with severe itch.7,8 Patients with vaginal LP may present with copious yellow to yellowgreen vaginal discharge, due to a desquamative vaginitis. In contrast to LS, LP affects mostly perimenopausal and postmenopausal women but rarely affects children. LP similar to LS can be also associated with other autoimmune diseases, such as vitiligo, pernicious anemia, alopecia areata, and thyroid disease.8 It is clinically important to note that erosive LP of the vulvovaginal tissue is most commonly associated with oral erosive disease, termed the vulvovaginal gingival syndrome, thus necessitating an oropharyngeal examination in all patients with genital LP.7 www.clinicalobgyn.com
Oropharyngeal involvement may reveal Wickham striae on the buccal mucosa, erosions of the marginal gingiva, atrophic papillae on the tongue’s surface, or erosions on the tongue (Figs. 3 and 4). Vulvar LP can present with different morphologies, including classical (papulosquamous), erosive, hypertrophic, and mixed subtypes.9 Papulosquamous LP of the vulva presents as small, poorly demarcated pink-violaceous papules or plaques, which often demonstrate an overlying white reticulated or net-like plaque termed Wickham striae that is pathognomonic for LP. The most common form of vulvar LP is erosive, and erosions may be seen on the introitus, clitoris, and labia minora (Fig. 5).7 Diagnostic Studies Much like with LS, the diagnosis of LP is primarily made based on the history and physical examination; however, biopsies are more often needed in this entity because erosions can be seen in VIN/SCC as
Inflammatory Vulvar Dermatoses
FIGURE 5. Vulvar erosive lichen planus. Note the erosion medial to the left labium minus, the resorption of the right labium minus, and the adherent yellow-green discharge.
well as in autoimmune blistering diseases, such as pemphigus vulgaris and mucous membrane pemphigoid. A biopsy can be performed if there is doubt regarding the diagnosis of LP, especially if there are no Wickham striae or co-occurring classic findings of oral LP, or if there are hyperkeratotic or nodular lesions concerning for VIN or HPV-negative SCC, which has been associated with LP.8 A shave or punch biopsy may be performed as long as it adequately samples the dermis or submucosa. A biopsy at the edge of an erosion often gives the most diagnostic findings for the pathologist. Most commonly a specimen submitted in formalin for H&E analysis is sufficient, but when attempting to rule out an autoimmune bullous disease, a second sample should be submitted in Zeus or Michel’s medium for direct immunofluorescence. Histopathological review by both a surgical pathologist and a dermatopathologist is ideal.
469
Examination of the vulva, peri-anal skin, and vagina via a small speculum should be performed to look for signs of active disease (erosions, Wickham striae, purulent vaginal discharge) and chronic disease (scarring of the clitoral hood, labia minora, or vaginal canal). If resistance is felt or pain elicited on speculum examination, a lubricated single gloved digit can be gently placed inside to feel for strictures or tumors. Occasionally, an examination under anesthesia may be needed if a complete examination in clinic is limited by pain. For some patients, a vaginal examination may be deferred to the next follow-up visit if vulvar disease control is needed first to improve tolerance to speculum placement. On follow-up visits, patients with LP should be regularly assessed for signs of malignancy with a low threshold to biopsy lesions refractory to treatment similarly to that which was discussed for LS above.8 Hepatitis C virus (HCV) is most associated with the erosive subtype of LP; thus, checking HCV antibody titers and asking the patient about a history of hepatitis C is important. Of note, recent literature publications demonstrate that LP is truly a multiorgan disease that can cause symptoms of ocular tearing, hearing loss, pain with eating acidic, spicy, or caffeinated foods, odynophagia, dysphagia, painful defecation, and dysuria with decreased urinary stream. If a patient with suspected vulvovaginal LP reports any of these symptoms, it is fruitful to refer the patient to the appropriate specialist for evaluation. Management Gentle skin care is the foundation for managing genital LP. Medications that can trigger lichenoid drug eruptions, including NSAIDs, diuretics, and antihypertensives should be elicited on patient history and consideration for drug holiday with close involvement from a primary care provider can be considered, www.clinicalobgyn.com
470
Guerrero and Venkatesan
although this may or may not alter disease course. Treating erosive vulvar LP with class I topical corticosteroids such as clobetasol propionate 0.05% or betamethasone dipropionate 0.05% ointments should be attempted first. Particularly for hypertrophic lesions that are unresponsive to potent steroids, a biopsy must be taken to evaluate the lesion for malignancy. Additional therapies that can be used to treat LP lesions include topical calcineurin inhibitors, most commonly tacrolimus 0.1% ointment. Tapering protocols are similar to those mentioned above for LS. Intralesional triamcinolone acetonide injections can also be used especially in thicker lesions or to establish initial disease control in patients who may not tolerate topical preparations initially because of discomfort.1 Adjunctive therapy in postmenopausal women usually consists of an intravaginal local topical estrogen, if there are no contraindications to its use. For vaginal LP, a commercial hydrocortisone 25 mg rectal suppository may be used intravaginally on a nightly basis; however, this may not be sufficient to achieve disease control. Some vulvologists compound a hydrocortisone 300 mg suppository to be used intravaginally on a nightly basis. Alternatively, 0.5 gm or 1 gm of clobetasol propionate 0.05% ointment or fluocinonide 0.05% ointment may be inserted into the vagina with a vaginal applicator; however, this should only be done to achieve disease control on a short-term basis because of the risk of systemic absorption of the medication and subsequent hypothalamic-pituitary-adrenal axis suppression. Intravaginal corticosteroid use also increases the risk of candida, dermatophyte, and herpes infection; thus, the provider should monitor closely and have suspicion for superinfection if the patient calls or presents with a flare of symptoms after starting therapy. For severe disease refractory to treatment with topical corticosteroids, a short course of systemic steroids such as prednisone may www.clinicalobgyn.com
be given. Due to the side effects associated with long-term prednisone use, different agents are preferred for long-term control of severe LP. These steroid-sparing immunosuppressants include methotrexate, mycophenolate mofetil, azathioprine, cyclosporine, and hydroxychloroquine. Systemic immunomodulation should also be considered early in the disease course for patients with co-occurring LP on other body sites such as scarring alopecia of the scalp (lichen planopilaris) or scarring nail disease. Surgical management may be considered for patients with symptomatic labial adhesions who have adequate disease control. Lysis of adhesions may be helpful for decreasing dyspareunia, improving the flow of urine, and decreasing the risk of urinary tract infection.10 Due to the risk of developing VIN/SCC in association with vulvovaginal LP, patients should receive regular follow-up. Case reports and case series have demonstrated a 1% to 2.5% incidence of SCC with about 5 years of follow-up. Given the chronic nature of disease, multiple subsequent biopsies may be taken of hyperkeratotic areas and those refractory to treatment. Surveillance for cancer can be challenging and strictures may limit vaginal examination. In some cases an exam under anesthesia may be warranted, with appropriate imaging if warranted.
Nonscarring Dermatoses CONTACT DERMATITIS
Presentation Contact dermatitis may occur on any cutaneous surface and can be subdivided into irritant and allergic forms. Irritant contact dermatitis to a particular substance occurs in all people with sufficient exposure to that substance and is not immunologically mediated. It usually
Inflammatory Vulvar Dermatoses causes irritation and pain and occurs fairly quickly in response to exposure. In contrast allergic contact dermatitis to a particular allergen occurs in a subset of the population that is sensitized to the allergen, is immunologically mediated with a delayed reaction after exposure, and is associated with symptoms of itch and sometimes irritation or pain when very exuberant. Maceration decreases the threshold for allergen sensitization, so the anogenital region’s moist environment promotes the development of contact dermatitis. Heat, urine, and fecal incontinence, and excessive washing are common culprits for irritant contact dermatitis of the vulva. Topical benzocaine cream, topical antibiotics (such as neomycin, polymixin, bacitracin), preservatives, and lanolin are just a sampling of the allergens that can cause allergic contact dermatitis of the vulva.11 Some of these medications may have been used as a result of the patient trying to relieve her symptoms with over-the-counter products marketed to help vulvovaginal symptoms. Thus, when taking a thorough history of a patient with suspected contact dermatitis, it is necessary to ask about exposures to these triggering substances. If a woman’s regular hygiene routine includes douches, soaps, menstrual pads, and baby wipes, each of these should be evaluated as a potential cause for contact dermatitis. Wearing constrictive clothing, noncotton undergarments, and Lycra can generate excessive heat around the vulva, leading to sweating, which may promote chafing, the development of a contact dermatitis, and or superinfection with yeast. Other common vulvar irritants include condoms, lubricants, and spermicides.9 For acute contact dermatitis, physical examination can reveal erythema throughout the vulva, which can extend to the perianal skin and inner thighs (Fig. 6). Vesicles, bullae, and erosions, sometimes in a linear configuration, may be found if an exuberant reaction has occurred. Evidence
471
FIGURE 6. Vulvar contact dermatitis to a topical benzocaine cream.
of excoriation can be present along with fissures and scaling. Diagnostic Studies Contact dermatitis is diagnosed primarily based on the clinical examination and appropriate history. If a biopsy is performed, it will show spongiotic dermatitis with epidermal vesicles; eosinophils are commonly present in the allergic form. Sometimes patch testing can help identify a precise culprit allergen, thus referral to a dermatologist who conducts patch testing may be warranted if disease control is suboptimal. As contact dermatitis can develop after or during a vaginal infection, one can consider microscopy with pH testing of vaginal discharge and vaginal cultures to determine whether the patient concurrently has candidiasis, bacterial vaginosis, or trichomoniasis. These infections should not be overlooked as they will require additional management.9 Management Since contact dermatitis results from the skin being in contact with irritants or allergens, the first step in management is to ensure that the aggravating factors are removed. Women should be advised to stop douching or excessive cleansing of the vulva. Other measures such as the use of cold packs, oral antihistamines, and sitz baths will decrease pruritus and thus www.clinicalobgyn.com
472
Guerrero and Venkatesan
decrease scratching, which will help the epidermis to heal more quickly. A sitz bath twice daily with lukewarm water followed by a medium to ultrapotent topical steroid, followed by application of white petrolatum can restore the barrier function of the skin and calm the inflammation.12 For severe cases, oral prednisone may be needed to control symptoms with a 2- to 3-week taper to prevent a rebound flare. If there is evidence of secondary infection, appropriate antifungal, or antibiotic treatment must be started as guided by cultures and sensitivities. Secondary candida albicans infection can be controlled with oral fluconazole.12 LSC
Presentation LSC is generally a nonscarring process of epidermal thickening that develops as a result of repeated excoriation and rubbing from a myriad of triggers. LSC can occur anywhere on the body including genital skin such as the labia majora in women, the scrotum in men, and the perianal skin. Any disorder that causes itch can lead to the development of LSC including atopic dermatitis, contact dermatitis, and recurrent yeast infections. Additional triggers for LSC can include primary neurologic dysfunction or primary psychiatric illness leading to repetitive selfexcoriation.13 When a patient repetitively scratches or rubs a pruritic area of skin over time, lichenification (epidermal thickening and accentuation of skin markings) will develop and will be seen on physical examination as ‘‘leathery’’ or ‘‘bark-like’’ poorly demarcated, thick plaques with scale (Fig. 7).9,13 Other secondary changes associated with LSC include hyperpigmentation or hypopigmentation; depigmented scars can occur if the patient excoriates into the dermis.13 Diagnostic Studies LSC is usually a clinical diagnosis that can be achieved by eliciting a history of chronic www.clinicalobgyn.com
FIGURE 7. Lichen simplex chronicus with hyperpigmented, lichenified plaques over the labia majora and medial thighs.
rubbing and scratching and physical examination demonstrating lichenified hairbearing skin. If a biopsy is performed it should demonstrate irregular acanthosis (thickening of the epidermis). Parakeratosis, a superficial perivascular infiltrate, and superinfection with yeast or bacteria can also be seen on histopathologic analysis.9 Management LSC is a chronic disease that often does not have a true cure. The goal of management is to decrease the severity of symptoms by decreasing the rubbing and scratching. Counseling the patient about the itch-scratch cycle is paramount. Oral antipruritics are often the cornerstone of management, such as hydroxyzine or doxepin at night, being mindful of the side effects of sedation of both medications and the medication interactions of doxepin with some psychotropic medications, as doxepin is both an antihistamine and a tricyclic antidepressant.14 Management of LSC must involve eliminating triggers by using gentle skin care and avoiding culprit irritants and allergens. Again, patients can be advised to soak the affected areas in a lukewarm bath before applying a medium to ultrapotent topical corticosteroid followed by a gentle emollient, such as white petrolatum. The goal over time is to decrease the potency of corticosteroid prescribed once the LSC plaques
Inflammatory Vulvar Dermatoses
473
become thinner and return closer to normal epidermal thickness. Eventually patients may be able to control symptoms using only a low to medium potency corticosteroid during flares of pruritus.14 Patients with neurologic or psychiatric triggers for LSC should be referred to the appropriate specialists. PSORIASIS
Presentation Psoriasis is a relatively common chronic inflammatory skin disease of epidermal keratinocyte hyperproliferation and Tcell activation. About 15% of patients with cutaneous psoriasis also develop an inflammatory, sometimes destructive arthritis and enthesitis. In the general population, the prevalence of psoriasis is estimated at 1% to 2%, and isolated genital psoriasis is estimated to occur in 2% to 5% of individuals affected with the disease.15 All forms of cutaneous psoriasis can affect the genital skin including plaque-type, pustular, and inverse psoriasis. Psoriasis can be itchy, but itch is not a hallmark of disease.16 Physical examination of the vulva reveals well-demarcated pink plaques that can extend to the perianal skin (Figs. 8 and 9). Although psoriasis on the body
FIGURE 8. Vulvar psoriasis in a child. Note the well-demarcated nature of the plaque.
FIGURE 9. Vulvar and perianal psoriasis in a child.
often demonstrates a classic silvery micaceous scale, genital psoriasis often is scalefree because of the external genitalia’s naturally moist environment.
Diagnostic Studies Psoriasis is generally a clinical diagnosis. Looking for plaques elsewhere on the body can help confirm diagnosis; classic sites of involvement include the scalp, elbows, knees, gluteal cleft, and nail plates. Nail involvement can manifest clinically as nail pitting, oil spots, or elevation of the distal nail plate from the bed called distal onycholysis. A skin biopsy can be performed if the diagnosis is in question and usually shows regular acanthosis (epidermal thickening) with parakeratosis, hypogranulosis, thinned suprapapillary plates, dilated capillaries in the dermal papillae, and neutrophils in the corneal layer. Pustular psoriasis shows subcorneal pustules on biopsy.16 www.clinicalobgyn.com
474
Guerrero and Venkatesan
Management Like most inflammatory vulvar dermatoses psoriasis has no cure, but disease activity and associated symptoms can be controlled with a combination of medications. Patients with psoriasis are advised to establish a gentle skin care regimen. Psoriasis is typically treated with low to medium potency topical corticosteroids, such as hydrocortisone 2.5%, desonide, or triamcinolone 0.1% ointment. Chronic topical corticosteroid use especially in the body folds, where their effects are potentiated by occlusion, can lead to skin atrophy, easy bruising, striae, and telangiectasias. To decrease the risk of these side effects, medications such as topical vitamin D analogues (eg, calcipotriene 0.005%) or topical calcineurin inhibitors (eg, tacrolimus 0.1% ointment) may be used along with or as an alternative to topical corticosteroids. If vulvar plaques do not respond to these medications, a biopsy should be taken to evaluate for malignancy.17 In addition, for patients experiencing severe pruritus, an oral antihistamine can bring relief. The Koebner phenomenon describes how lesions can develop at sites of trauma, and this occurs not only in psoriasis, but in LS and LP as well.17 To prevent further development of these lesions, minimizing trauma to the skin is necessary. For instance, long distance cyclers or horseback riders may develop plaques over time in areas with repeated trauma. Since patients with cutaneous psoriasis may also develop psoriatic arthritis, providers must inquire about joint symptoms and refer these patients to a rheumatologist if appropriate.
Summary Inflammatory vulvar dermatoses can severely impact the quality of a woman’s life, and it is critical to accurately diagnose these entities so that tailored management can be recommended. Providers must www.clinicalobgyn.com
hone in on key findings in the history and physical examination to guide diagnosis, with skin biopsy serving as a useful tool when the diagnosis is in question. In general, the presence of scarring can be used to divide these diseases into 2 groups: scarring dermatoses (LS and LP) and nonscarring dermatoses (contact dermatitis, LSC, and psoriasis). For scarring dermatoses, notable differences between LS and LP include the affected age groups (LP is rare in prepubertal children) and physical examination findings (thin, hypopigmented cigarette-paper skin in LS vs. eroded plaques in LP). For nonscarring dermatoses, the clinical history can be telling, as patients with contact dermatitis or LSC may have experienced intense pruritus and may have a history of atopic disease. Psoriasis is classically diagnosed based on its presentation as well-demarcated plaques, with often involvement of the elbows, knees, and scalp with overlying silver scale. For all of these inflammatory vulvar dermatoses, gentle skin care must be taught. Even once a diagnosis has been established, consideration for skin biopsy and/or microbial cultures for refractory disease may be warranted to identify iatrogenic infections or the development of VIN or SCC. Topical corticosteroids of varying potency or systemic therapies are often prescribed. Arranging regular follow-up visits, particularly for diseases with an increased malignant potential, is necessary to ensure optimal response to treatment and to perform appropriate surveillance for malignancy.
References 1. Val I, Gutemberg A. An overview of lichen sclerosus. Clin Obstet Gynecol. 2005;48:808–817. 2. Epstein AG. Gynecological examination of a prepubertal child [Armenian medical Network web site]. Available at: http://www.health.am/ gyneco/more/gynecological-examination-of-aprepubertal-child/. Accessed November 2, 2014. 3. Davis AJ, Katz VL. Pediatric and Adolescent Gynecology. In: Lentz GM, ed. Comprehensive
Inflammatory Vulvar Dermatoses
4.
5. 6.
7. 8.
9. 10.
Gynecology, 6th ed. Philadelphia: Elsevier Mosby; 2012:199–213. Van de Nieuwenhof H, Bulten J, Hollema H, et al. Differentiated vulvar intraepithelial neoplasia is often found in lesions, previously diagnosed as lichen sclerosus, which have progressed to vulvar squamous cell carcinoma. Mod Pathol. 2011;24: 297–305. Murphy R. Lichen sclerosus. Dermatol Clin. 2010; 28:707–715. Brodrick B, Belkin ZR, Goldstein AT. Influence of treatments on prognosis for vulvar lichen sclerosus: facts and controversies. Clin Dermatol. 2013;13:780–786. Goldstein AT, Metz A. Vulvar lichen planus. Clin Obstet Gynecol. 2005;48:818–823. Regauer S, Reich O, Eberz B. Vulvar cancers in women with vulvar lichen planus: a clinicopathological study. J Am Acad Dermatol. 2014;71: 698–707. Rodriguez MI, Leclair CM. Benign vulvar dermatoses. Obstet Gynecol Surv. 2012;67:55–63. Suzuki V, Haefner HK, Piper CK, et al. Postoperative sexual concerns and functioning in patients who underwent lysis of vulvovaginal
11. 12. 13.
14.
15.
16.
17.
475
adhesions. J Low Genit Tract Dis. 2013;17: 33–37. Schlosser BJ. Contact dermatitis of the vulva. Dermatol Clin. 2010;28:697–706. Margesson LJ. Contact dermatitis of the vulva. Dermatol Ther. 2004;17:20–27. Lynch PJ. Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region. Dermatol Ther. 2004;17:8–19. Thorstensen KA, Birenbaum DL. Recognition and management of vulvar dermatologic conditions: lichen sclerosus, lichen planus, and lichen simplex chronicus. J Midwifery Womens Health. 2012;57:260–275. Meeuwis KAP, De Hullu JA, Massuger LFAG, et al. Genital psoriasis: a systematic literature review on this hidden skin disease. Acta Derm Venereol. 2011;91:5–11. Murphy M, Kerr P, Grant-Kels JM. The histopathologic spectrum of psoriasis. Clin Dermatol. 2007;25:524–528. Guglielmetti A, Conlledo R, Bedoya J, et al. Inverse psoriasis involving genital skin folds: successful therapy with dapsone. Dermatol Ther. 2012;2:15–23.
www.clinicalobgyn.com
