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Inflammatory Vulvar Dermatoses (Part I)

Open AccessPublished:April 09, 2022DOI:https://doi.org/10.1016/j.urology.2022.03.025

      Abstract

      Vulvar disease is common, and urologists are often the first providers to see patients with a vulvar skin condition. Primary vulvar dermatoses can be localized to the anogenital area or a manifestation of more diffuse cutaneous disease. Additionally, secondary dermatoses can develop from exogenous agents and inflammatory vaginitis. Vulvar conditions are challenging to diagnose due to location and different types of skin and mucosal epithelium involved. Herein, we provide an overview of noninfectious inflammatory vulvar dermatoses (part I) and benign and malignant vulvar neoplasms (part II), grouped by morphologic findings. We include diagnostic evaluation, workup, and management of these conditions.

      INFLAMMATORY VULVAR DERMATOSES (PART I)

      In this review, we aim to provide a framework that will aid the practicing urologist in recognition, diagnosis, and even early treatment of various inflammatory vulvar dermatoses. Depending on the urologist's comfort level with skin conditions, scope of practice, and patient population, we hope this information may serve as a guide to aid in diagnosis and management of some of these conditions. A dermatology referral can be very helpful in cases where a urologist is less comfortable with treating skin conditions, the skin condition is outside the scope of their practice, the condition is not responsive to initial therapy, or the provider feels the patient would benefit from a more in-depth dermatologic evaluation.
      The vulva is made up of keratinized skin (hair-bearing labia minora and mons), partially keratinized modified mucous membranes (medial labia majora, labia minora), and nonkeratinized mucous membranes (introitus and vagina). While vulvar skin can be affected by skin conditions elsewhere on the body (psoriasis, eczema, vitiligo), some conditions have a predilection for vulvar skin (lichen sclerosus) and some involve both the vulva and vagina (erosive lichen planus). Thus, location of lesions can aid in diagnosis.
      Understanding the morphology of the primary lesion is helpful in diagnosis. Sections are separated into red and white dermatoses, erosions and ulcerations, and vesiculobullous (blisters), based on how lesions appear clinically. As vulvar skin is damp and consists of folds, scale can be hard to identify and dermatoses can appear white due to hydrated scale. Thus, it is important to be aware that many classic “red” dermatoses can mimic “white” dermatoses on the vulva. Please see Table for a summary of conditions discussed in this review, morphologic findings, and treatment modalities (Table 1).
      Table 1Inflammatory vulvar dermatoses: diagnoses, anatomic locations, clinical findings, and treatment modalities
      DiagnosisPredominant Anatomic Location
      Categories of vulvar epithelium: (1) Keratinized epithelium (KE) includes the lateral labia majora, mons pubis, and inner thighs; (2) Modified mucous membranes (MMM) include the medial labia majora and entire labia minora. These are partially keratinized, and transition between fully keratinized epithelium and mucous membranes (MM); (3) Mucous membranes (MM) include the medial labia minora (vestibule/introitus) and the vagina.
      Clinical FindingsTreatment Modality
      Treatment modalities: Examples of topical corticosteroid (TCS): (1) Low-potency TCS includes hydrocortisone 2.5% or desonide 0.05% ointment. Mid-potency TCS includes triamcinolone 0.1% ointment. High-potency TCS includes fluocinonide 0.1% ointment. Ultra-potent TCS includes clobetasol 0.05% or halobetasol 0.05% ointment. (2) Compounded TCS for intravaginal use usually consists of hydrocortisone 100 g (compounded), or for more mild disease can use hydrocortisone 25 g (available in most pharmacies as rectal suppositories). (3) Topical calcineurin inhibitors include pimecrolimus cream and tacrolimus ointment (both are non-steroidal). (4) Calcipotriene is a vitamin D analogue (non-steroidal). (5) Topical JAK inhibitors include ruxolitinib or tofacitinib ointment. (6) Surgical treatment includes wide local excision (WLE) and Mohs micrographic surgery (MMS).
      Red Dermatoses
      Lichen simplex chronicus
      • KE
      • MMM
      Perianal involvement often seen.
      Lichenified, erythematous, often hyper- and hypopigmented plaques, ± linear excoriations and erosions. Intensely pruritic.Eliminate irritants. Mid- to high-potency TCS. Calcineurin inhibitors or calcipotriene as second line. Barrier protection with petrolatum. Oral antihistamines.
      Contact dermatitis
      • KE
      • MMM
      Erythematous patches or plaques with subtle scale, ± fissures (located in areas where irritant or allergen is in contact with skin).Eliminate irritant/allergen. Mid-potency TCS. Barrier protection with petrolatum. Consider patch testing.
      Psoriasis
      • KE
      • MMM
      Red, well-demarcated plaques ± silver scale, ± fissures. Often have psoriasis elsewhere on body or nail changes consistent with psoriasis.Mid-potency TCS. Maintenance: Low-potency TCS, or topical calcineurin inhibitors, or calcipotriene. If disease is severe, consider systemic psoriasis therapies.
      Seborrheic dermatitis
      • KE
      MMM (less common).
      Erythematous patches, or thin papules and plaques. Can have an orange-hue, ± fine scale. Often involves inguinal folds.Low- to mid-potency TCS for flares. Consider topical calcineurin inhibitors for maintenance. Topical antifungals, such as azole (ketoconazole) solutions and ointments can also be used.
      Extramammary paget disease
      • KE
      • MMM
      MM (less common).Perianal involvement very common.
      Fixed red plaques and patches with white hydrated scale. Plaques are usually thick, with occasional erosions and hyperpigmentation.

      Often mistaken for eczema.

      Consider this in the differential for a vulvar disease that is chronic, fixed in location, and does not respond to TCS.

      Surgical treatment (WLE or MMS), systemic chemotherapy, or radiation. Topical immune modifying agents or chemotherapies can be considered in patients who are not surgical candidates.
      White Dermatoses
      Lichen sclerosus
      • KE
      • MMM
      Midline adhesions result in introital narrowing, but true vaginal involvement is very uncommon. Perianal involvement very common.
      White to hypopigmented patches or thin plaques with atrophic textural changes and wrinkling. Often starts with erythematous or pink changes that progress. Tends to involve perineal and perianal area.

      ± purpura/ecchymoses.

      ± fissures and erosions. Chronic lichen sclerosus leads to architectural changes (scarring).
      Ultrapotent TCS until skin texture normalizes (usually a few month).

      Maintenance: Ultrapotent TCS 3 ×/wk, or mid-potency TCS daily, or topical calcineurin inhibitors daily.

      Note: Even when asymptomatic, lichen sclerosus requires maintenance therapy to prevent squamous cell carcinoma.
      Vitiligo
      • KE
      MMM (less common).
      Well-demarcated, depigmented white macules and patches with lack of texture change. May have erythema in active inflammatory vitiligo. Generally do not see fissures, purpura, or architectural changes.

      Mid- to high-potency TCS initially, titrated to low-potency TCS, calcineurin inhibitors, or calcipotriene. Can also consider topical JAK inhibitors.
      Post-inflammatory hypopigmentation
      • KE
      • MMM
      Poorly-demarcated hypopigmentation that follows the distribution of preceding inflammation and lacks textural change. No scale or thickening.Identification and treatment of any inflammatory condition that remains. Reassurance that this will fade with time.
      Erosions and Ulcerations
      Erosive vulvovaginal lichen planus
      • KE
      • MMM
      • MM
      Non-specific erosions and erythematous plaques, thin patches ± lacy white reticulation. Often involves the vagina but can be localized to either vulva or vagina alone.

      Leads to architectural changes (scarring).
      Superpotent TCS to affected areas. If vaginal involvement compounded hydrocortisone suppositories (100 mg).

      Many patients require prednisone and bridge to systemic steroid-sparing immunosuppressive therapy. Some patients may require surgical lysis of adhesions.

      Plasma cell vulvitis
      • MMM
      • MM
      Well-demarcated, solitary, bright red or orange-brown macule or patch often associated with burning or pain. Can be pruritic.

      Note: While controversial, some consider this a less severe variant of lichen planus.
      Ultrapotent TCS, tacrolimus ointment, or compounded hydrocortisone combined with estrogen cream intravaginally.
      Vulvar aphthae
      • KE
      • MMM
      • MM
      Well-demarcated ulcers with a gray or necrotic base. Commonly appear as “punched out” lesions of various sizes. May have erythema, edema, and patients often have oral aphthae as well.Rule out infectious causes of ulcers (ie, HSV and syphilis).

      Topical anesthetics, chemical cauterization, intralesional triamcinolone, or systemic steroids can be used.

      Consider other systemic therapies such as colchicine or dapsone depending on severity and frequency.
      Crohn's disease
      • KE
      • MMM
      MM through fistula formation (less common).
      Characterized by perianal skin tags, genital edema, abscesses, fistulas, and linear “knife-cut” ulcers in skin folds.

      Patients with Crohn's may also have a non-specific “inflammatory vaginitis” that leads to vulvar irritation consistent with contact dermatitis from irritating secretions.
      Potent TCS, intralesional triamcinolone. Management of underlying gastrointestinal disease.

      For edema, consider compression and referral to lymphedema therapist with experience in treatment of vulvar lymphedema.
      Autoimmune Blistering Disease
      Bullous pemphigoid
      • KE
      Generally spares MMM and MM.
      Small fluid-filled vesicles and tense bullae that arise from inflamed erythematous urticarial or eczematous plaques. Genital involvement is more common in children than adults.

      High-potency TCS for mild disease. May require systemic steroids in conjunction with steroid-sparing agents.

      Mucous membrane pemphigoid
      • KE
      • MMM
      • MM (predominant)
      Blisters that rapidly erode and lead to architectural changes (scarring). Significant pain and pruritus.

      Systemic immunosuppressants. Compounded TCS for intravaginal use and dilators to prevent introital narrowing. Ultrapotent TCS for vulvar involvement. Some patients may require surgical therapy.

      Pemphigus vulgaris
      • KE
      • MMM
      • MM (predominant)
      Start as fragile blisters that give rise to well-demarcated erythematous erosions. Blisters and vesicles are rarely evident. Possible oral involvement.

      Systemic immunomodulators.

      Compounded TCS for intravaginal use and dilators to prevent introital narrowing. Ultrapotent TCS for vulvar involvement.
      Linear IgA bullous dermatosis
      • KE
      • MMM
      • MM
      Similar to lesions of bullous pemphigoid with tense vesicles/blisters. Non-scaling erosions often seen if blisters mechanically disrupted from friction in this area.Removal of inciting agent if medication-induced.

      Mid-potency TCS.

      Addition of oral dapsone, and other systemic immunomodulators
      Other Vesiculobullous Disease
      Hailey-Hailey disease
      • KE
      MMM (less common).
      Macerated red papules and plaques with thickened white areas. Fissures, erosions, crusts, and small vesicles can also be found. Pustules may be present. Common in skin folds.Mid-potency TCS and oral corticosteroids. Antifungals and antibiotics (topical clindamycin or oral doxycycline) may be used for superimposed infections. Systemic therapy including naltrexone and dapsone as well as medical and surgical therapies targeted at control of sweat.

      Fixed drug eruption
      • KE
      • MMM
      • MM
      Well-demarcated, red to violaceous patch, blister, or erosion. Occurs in the same location with repeated exposure to the culprit agent.Cessation of offending medication and local wound care.
      low asterisk Categories of vulvar epithelium: (1) Keratinized epithelium (KE) includes the lateral labia majora, mons pubis, and inner thighs; (2) Modified mucous membranes (MMM) include the medial labia majora and entire labia minora. These are partially keratinized, and transition between fully keratinized epithelium and mucous membranes (MM); (3) Mucous membranes (MM) include the medial labia minora (vestibule/introitus) and the vagina.
      Treatment modalities: Examples of topical corticosteroid (TCS): (1) Low-potency TCS includes hydrocortisone 2.5% or desonide 0.05% ointment. Mid-potency TCS includes triamcinolone 0.1% ointment. High-potency TCS includes fluocinonide 0.1% ointment. Ultra-potent TCS includes clobetasol 0.05% or halobetasol 0.05% ointment. (2) Compounded TCS for intravaginal use usually consists of hydrocortisone 100 g (compounded), or for more mild disease can use hydrocortisone 25 g (available in most pharmacies as rectal suppositories). (3) Topical calcineurin inhibitors include pimecrolimus cream and tacrolimus ointment (both are non-steroidal). (4) Calcipotriene is a vitamin D analogue (non-steroidal). (5) Topical JAK inhibitors include ruxolitinib or tofacitinib ointment. (6) Surgical treatment includes wide local excision (WLE) and Mohs micrographic surgery (MMS).
      For vulvar conditions, the preferred topical corticosteroid (TCS) preparation is an ointment. In general, application of ointments to the vulva is associated with less burning than with creams. While topical tacrolimus is a non-steroidal ointment that can be used on vulvar skin, it is important to counsel patients that this is an exception to the above in that tacrolimus may burn with application.

      RED DERMATOSES

      Lichen Simplex Chronicus

      Lichen simplex chronicus (LSC) occurs from chronic rubbing or scratching due to pruritus caused by various primary dermatologic conditions, including underlying atopic dermatitis (AD)/eczema or contact dermatitis.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Additionally, LSC can coexist with other inflammatory vulvar conditions due to chronic scratching/rubbing. Early vulvar LSC is characterized by erythema and eczematous patches and may have linear excoriations or erosions. Over time the skin becomes thickened or lichenified (Fig. 1A).
      • Lynch PJ
      Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region.
      Chronic LSC may be hypopigmented or hyperpigmented, particularly in darker skin types. Scale may be subtle or absent given increased moisture in this area.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      LSC is diagnosed clinically but may require biopsy if not responsive to treatment. Management of LSC focuses on breaking the itch scratch cycle, treating inflammation, and restoring skin barrier.
      • Lynch PJ
      Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region.
      Use of medium to high-potency TCS twice daily for short periods during flares and gentle skin care with soak and seal methods (application of petroleum-based emollients) are important. Lower potency TCS or topical calcineurin inhibitors (tacrolimus 0.1% ointment) can be considered if therapy is needed for extended durations.
      • Lynch PJ
      Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region.
      However, the calcineurin inhibitors (tacrolimus or pimecrolimus) can cause irritation and burning with application. Antihistamines such as hydroxyzine can also be helpful for symptom relief.
      • Lynch PJ
      Lichen simplex chronicus (atopic/neurodermatitis) of the anogenital region.
      Figure 1
      Figure 1(A) Lichen simplex chronicus (LSC) with prominent lichenification on left labium majus. (B). Acute contact dermatitis. (C). Psoriasis with well-demarcated patches and thin plaques with white scale. (D) Extramammary paget disease in the setting of scar and architectural changes from prior vulvectomy. (Color version available online.)

      Contact Dermatitis

      Vulvar contact dermatitis (CD) is a common eczematous response to a substance applied to the skin.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      CD can occur as the primary skin condition or complicate other vulvar conditions. There are 2 types of CD, allergic contact dermatitis (ACD) which occurs through a delayed type IV hypersensitivity reaction where the skin is sensitized to an allergen, or irritant contact dermatitis (ICD) due to irritant exposure. ICD is more common than ACD but both can occur at the same time.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      The vulva has a higher prevalence of contact sensitization due to increased number of antigen-presenting cells, making ACD more likely on the vulva than on other areas of the skin.
      • Woodruff CM
      • Trivedi MK
      • Botto N
      • Kornik R
      Allergic contact dermatitis of the vulva.
      Acute CD presents with bright erythema (Fig. 1B), edema, and sometimes blistering.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      The skin can have a glazed appearance. Chronic CD has less edema and blistering but persistent erythema and fissures and lichenification can be seen due to longstanding irritation. Chronic CD is indistinguishable from LSC.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Common culprits of ACD include fragrances and botanical compounds in personal hygiene products, topical medicaments (including antibiotics, antifungals, analgesics, hemorrhoid creams), and preservatives in wipes and other cosmetics marketed for genital sites, spermicides, and menstrual pads.
      • Woodruff CM
      • Trivedi MK
      • Botto N
      • Kornik R
      Allergic contact dermatitis of the vulva.
      Causes of ICD include excessive cleansing, harsh soaps, detergents, irritants in products above, sweat, urine, and feces.
      • Woodruff CM
      • Trivedi MK
      • Botto N
      • Kornik R
      Allergic contact dermatitis of the vulva.
      Patients with an abnormal vaginal discharge can develop an ICD due to irritating secretions.
      Management requires identification of the irritant/allergen and removal from the environment. This requires a detailed history of use of wipes, topicals, douches, soaps, fragrance, laundry detergent, etc. If the allergen cannot be identified, a referral to dermatology or allergy should be considered for patch testing to identify culprit allergens. Treatment should be initiated with a mid-potency TCS (triamcinolone 0.1%) twice daily for 4-6 weeks.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Ultimately, identification and elimination of the allergen/irritant will be necessary.
      • Woodruff CM
      • Trivedi MK
      • Botto N
      • Kornik R
      Allergic contact dermatitis of the vulva.
      It is important to counsel that re-exposure to an allergen even once a month, could result in recurrence of CD.

      Psoriasis

      Vulvar psoriasis often presents in the setting of other cutaneous psoriasis, but the vulva can be the only area involved. Up to 46% of patients with psoriasis have genital involvement.
      • Meeuwis KAP
      • De Hullu JA
      • Inthout J
      • et al.
      Genital psoriasis awareness program: physical and psychological care for patients with genital psoriasis.
      Vulvar psoriasis is characterized by pruritus and often not as classic in appearance due to the location. For example, instead of the well-demarcated pink thick plaques with silver scale, that can be seen in “classic” psoriasis on other areas of the body, vulvar psoriasis can present with more subtle findings including non-specific erythema or thin pink papules or plaques.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Scale may or may not be seen but tends to be less thick than in other cutaneous sites (Fig. 1C). There may be fissuring and lichenification. There is no involvement of mucous membranes.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Initial treatment involves mid-potency TCS (triamcinolone 0.1%) as first-line agents and topical calcineurin inhibitors or calcipotriene for maintenance therapy.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      For severe disease, systemic therapies including oral agents and biologics are important.

      Seborrheic Dermatitis

      Seborrheic dermatitis is a very common inflammatory skin condition, involving intertriginous areas, related to overgrowth of yeast, such as Malassezia.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Seborrheic dermatitis (SD) affecting vulvar skin appears as erythematous patches or thin papules and plaques. Only keratinized skin is affected and there is no involvement of mucous membranes. Pruritus can be present but is usually less severe than in psoriasis or lichen sclerosus. SD is exacerbated by humidity and occlusive clothing. Treatment consists of low potency TCS (hydrocortisone 2.5%) or mid-potency TCS (triamcinolone 0.1%) for a short time with flares, with topical tacrolimus or pimecrolimus for maintenance. Topical azoles, such as ketoconazole can also be used to target yeast.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      It is important to counsel patients that there is no “cure” for SD, but it can be controlled and treated with maintenance therapy.

      Extramammary Paget Disease

      Extramammary paget disease (EMPD) is a malignant condition discussed here because it clinically mimics a dermatitis and is often misdiagnosed as eczema, psoriasis, or another benign condition. EMPD is common on the vulvar and perianal skin, presenting with red plaques and patches with white hydrated scale, with an appearance sometimes referred to as “strawberries and cream” (Fig. 1D).
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Erosions can be seen. Patients may be symptomatic or have pruritus, burning or pain. Diagnosis is confirmed by biopsy. Patients must undergo extensive workup to rule out underlying adenocarcinoma which is associated with EMPD in 11% to 54% of cases.
      • Nitecki R
      • Davis M
      • Watkins JC
      • et al.
      Extramammary paget disease of the vulva: a case series examining treatment, recurrence, and malignant transformation.
      Treatment is usually wide local excision or Mohs micrographic surgery.
      • Shwe S
      • Elsensohn AN
      • Ortiz C
      • Kraus CN
      Mohs micrographic surgery for vulvar malignancies: a systematic review.
      Radiation, laser therapy, and topical therapies, such as imiquimod have also been used with variable success.
      • Dilmé-Carreras E
      • Iglesias-Sancho M
      • Márquez-Balbás G
      • Sola-Ortigosa J
      • Umbert-Millet P
      Radiotherapy for extramammary Paget disease of the anogenital region.
      ,
      • Nasioudis D
      • Bhadra M
      • Ko EM
      Extramammary Paget disease of the vulva: Management and prognosis.
      Systemic chemotherapy is utilized in advanced disease.
      • Nasioudis D
      • Bhadra M
      • Ko EM
      Extramammary Paget disease of the vulva: Management and prognosis.
      EMPD is challenging to treat due to its multifocal nature, and lesions tend to recur, so patients require close follow-up.
      • Nitecki R
      • Davis M
      • Watkins JC
      • et al.
      Extramammary paget disease of the vulva: a case series examining treatment, recurrence, and malignant transformation.

      WHITE DERMATOSES

      Lichen Sclerosus (LS)

      Lichen sclerosus (LS) is a chronic inflammatory disease that most commonly affects the vulva and perianal area but can involve any area of the skin.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      While etiology is unknown, autoimmune, genetic, and hormonal factors have been implicated.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      ,
      • Terlou A
      • Santegoets LAM
      • Van Der Meijden WI
      • et al.
      An autoimmune phenotype in vulvar lichen sclerosus and lichen planus: A Th1 response and high levels of microRNA-155.
      LS can occur at any age but is more common in prepubertal girls and post-menopausal women.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      As fissures can occur in the anal and perineal skin leading to painful bowel movements, it is important to consider LS in pediatric patients presenting with constipation
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      . In adults, LS is usually associated with pruritus and pain.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      LS presents with white to hypopigmented patches and thin plaques with textural changes that appear atrophic or “crinkled” (Fig. 2A). Erythema can be present as well as fissures and petechia or ecchymoses. Breaks in the skin and the presence of bruisin, result due to thinning of the skin from the chronic inflammation of LS itself. Perianal involvement which is common is sometimes referred to as a “figure-of-eight” pattern.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      In chronic cases, architectural changes develop, leading to resorption of labia minora, fusion of the clitoral hood to the clitoris, midline fusion and even narrowing of the introitus (Fig. 2B). Such changes can lead to genital pain and urinary symptoms. LS affects the keratinized epithelium and modified mucous membranes but only in very rare causes involves the vagina.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Diagnosis is clinical and aided by biopsy. SCC can develop in about 5% of patients with vulvar LS, so regular monitoring and maintenance therapy is important, even in the absence of symptoms.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      ,
      • Lee A
      • Bradford J
      • Fischer G
      Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women.
      Figure 2
      Figure 2(A) Lichen sclerosus of the vulva. (B) Chronic lichen sclerosus of the vulva with midline fusion and scarring. (C) Vulvar vitiligo. (D). Chronic changes of scarring and introital narrowing due to erosive vulvovaginal lichen planus. These chronic changes are indistinguishable from other scarring inflammatory dermatoses such as lichen sclerosus and mucous membrane pemphigoid. (E) Plasma cell vulvitis with subtle red/brown macules at right introitus. (F) Linear ulceration of inguinal fold in vulvar Crohn's disease. (Color version available online.)
      Initial treatment consists of ultrapotent TCS application once daily for 6-8 weeks with follow-up to assess for symptomatic and clinical improvement, usually requiring about 4 months duration.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      LS results in thinning of the skin due to the immune process itself. Topical steroids treat that inflammation, resulting in improvement in the wrinkled or shiny changes of the skin. Topical steroids can be then titrated to maintenance therapy which consists of either an ultrapotent TCS (clobetasol 0.05%) to the area three nights a week, or mid-potency TCS (triamcinolone 0.01%) to the area daily or tacrolimus ointment daily.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Lifetime maintenance therapy is needed to prevent inflammation that can lead to SCC in patients with uncontrolled LS. It is important to demonstrate (either with photographs or hand mirrors) where to apply the steroid ointment and amount to use.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Usually a lentil-size amount of TCS is enough to cover the entire involved area. Intralesional triamcinolone 10 mg/cc injections can be utilized for more recalcitrant areas.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Vitiligo

      Vitiligo is an autoimmune skin condition characterized by depigmentation (complete loss of pigment) resulting from destruction of melanocytes in the epidermis. It may be isolated to the vulva or occur with vitiligo in other locations.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      For patients who are predisposed, trauma or inflammation can precipitate vulvar vitiligo. This presents as well-demarcated depigmented white macules and patches (Fig. 2C). Vitiligo is usually asymptomatic. While clinically it can be mistaken for LS, vitiligo is more well-demarcated, does not have ecchymoses or fissures or any textural change.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Additionally, vitiligo is less likely to affect the mucous membranes and more localized to keratinized skin. First-line therapy includes mid- to high-potency TCS daily for 6-8 weeks, subsequently tapering to a low-potency TCS or tacrolimus or calcipotriene.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      While phototherapy can be used for vitiligo on the body, it is not practical or safe for the vulva.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Post-Inflammatory Hypopigmentation

      Inflammation of the skin can lead to hyperpigmentation or hypopigmentation, which resolves over time once the underlying inflammation is treated.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Diagnosis can be made based on history of prior inflammation and pattern of pigmentary changes. Textural changes and scarring are not present with post-inflammatory hypopigmentation, which should help distinguish hypopigmentation from LS. Vitiligo is due to depigmentation and can be distinguished from hypopigmentation (decreased pigment, but not complete loss of pigment) clinically with use of a Wood's lamp (bright blue-white on exam indicates vitiligo) or by biopsy if the diagnosis is in question.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      EROSIONS AND ULCERATIONS

      Erosive Vulvovaginal Lichen Planus

      Lichen planus (LP) is a mucocutaneous condition that can have various presentations on the skin. The most common form of LP affecting the vulvovaginal area is the erosive variant (erosive vulvovaginal lichen planus [EVVLP]).
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      This is characterized by non-specific erosions and erythema leading to scarring and introital narrowing (Fig. 2D). Erosions may be bordered by lacy white reticulation (Wickham's striae) though this is less common on genital mucosa than oral mucosa.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Patients usually present with pain, burning, and pruritus. With vaginal involvement, a sterile inflammatory vaginal discharge is present. Patient often have oral involvement as well. Diagnosis is made by biopsy, which is usually required as these lesions can clinically appear like other inflammatory dermatoses, including LS. Topical therapy involves superpotent TCS (clobetasol 0.05%) to the vulva, and compounded hydrocortisone 100-300 mg suppositories for vaginal involvement, tapering with symptom control.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Intravaginal TCS predisposes patients to candidiasis so weekly fluconazole 200 mg or an azole cream should be applied twice weekly for prophylaxis.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Dilators should be used to maintain introital patency. EVVLP usually require aggressive management and various systemic agents have been utilized including hydroxychloroquine, methotrexate, mycophenolate mofetil, azathioprine, cyclosporine and tacrolimus.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      ,
      • Dunaway S
      • Tyler K
      • Kaffenberger J
      Update on treatments for erosive vulvovaginal lichen planus.
      It is important to closely follow these patients as there is an increased risk of vulvovaginal SCC developing in chronic EVVLP, estimated to be around 2%.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.

      Plasma Cell Vulvitis

      Plasma cell vulvitis (PCV) is an uncommon, chronic, inflammatory disease of the vulva, also known as Zoon's vulvitis.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      It commonly presents in menopausal or post-menopausal women.
      • Sattler S
      • Elsensohn AN
      • Mauskar MM
      • Kraus CN
      Plasma cell vulvitis: a systematic review.
      PCV usually presents as a solitary bright red or orange-brown well-demarcated macule or patch, often mimicking an erosion, however on inspection the epidermis is intact (Fig. 2E).
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Sattler S
      • Elsensohn AN
      • Mauskar MM
      • Kraus CN
      Plasma cell vulvitis: a systematic review.
      PCV tends to occur more on the labia minora and introitus than keratinized skin.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Sattler S
      • Elsensohn AN
      • Mauskar MM
      • Kraus CN
      Plasma cell vulvitis: a systematic review.
      Symptoms include burning, stinging, and pain.
      • Sattler S
      • Elsensohn AN
      • Mauskar MM
      • Kraus CN
      Plasma cell vulvitis: a systematic review.
      Diagnosis is facilitated by biopsy. Potent TCS or tacrolimus are first-line agents and alternating 100 mg hydrocortisone suppositories with estradiol 0.01% vaginal cream nightly for 4-8 weeks has shown success.
      • Sattler S
      • Elsensohn AN
      • Mauskar MM
      • Kraus CN
      Plasma cell vulvitis: a systematic review.
      ,
      • Prestwood CA
      • Granberry R
      • Rutherford A
      • Mauskar MM
      Successful treatment of plasma cell vulvitis: a case series.

      Vulvar Aphthae

      Aphthous ulcers of the vulva (Lipschutz ulcers) must be distinguished from erosions affecting the vulva.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Erosions like those of EVVLP are more superficial while ulcers are deeper, with a white fibrin base. The main diagnoses to exclude when working up a patient with vulvar ulcers are infections such as herpes simplex virus. The typical presentation of vulvar aphthae is a solitary well-demarcated ulcer on the vestibule but can affect any vulvar site.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Ulcers can appear bright red or gray and necrotic and may be accompanied by edema. Typically girls aged 9-18 are most likely to develop vulvar apthae.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      A prodrome with fatigue, malaise, low-grade fever may be reported.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      This is generally a diagnosis of exclusion after ruling out infection and other etiologies. Management of vulvar aphthae includes topical anesthetics such as lidocaine 5% ointment. Chemical cauterization with silver nitrate sticks can be considered. Cases may require intralesional triamcinolone or systemic steroids and recurrent disease may require colchicine or dapsone or tumor necrosis factor antagonists.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Of note, Behcet disease is an uncommon condition which consists of genital and oral aphthae in the setting of multisystem inflammation including other cutaneous manifestations, ocular involvement, gastrointestinal, neurologic, or vascular involvement. The International Criteria for Behcet disease can be used to support the diagnosis of Behcet disease.
      • Davatchi F
      • Assaad-Khalil S
      • Calamia KT
      • et al.
      The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria.
      Vulvar and oral aphthae are not sufficient for a diagnosis of Behcet disease.

      Crohn's Disease

      Crohn's disease (CD) is an inflammatory granulomatous disorder of the gastrointestinal tract that can result in anogenital lesions.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Rarely, vulvar involvement may precede gastrointestinal symptoms. Vulvar edema and perianal skin tags can be clues to a diagnosis of CD. Linear ulcerations described as “knife-cut,” can be seen in skin folds (Fig. 2F).
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Anogenital fistulas may be present. In children, vulvar edema is the most common presentation. The diagnosis can be made clinically in the setting of a known diagnosis of CD or in a patient with consistent GI symptoms, and is facilitated by biopsy. Fecal calprotectin levels can be useful as a screening test for intestinal inflammation.
      • Mauskar MM
      • Marathe K
      • Venkatesan A
      • Schlosser BJ
      • Edwards L
      Vulvar diseases: conditions in adults and children.
      Therapies should be directed toward management of the underlying gastrointestinal disease. Vulvar-directed therapies involve potent TCS and intralesional triamcinolone.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Vesiculobullous (Blisters): Autoimmune Blistering Disease

      Diagnosis of autoimmune blistering disease (ABD) relies on tissue biopsy for routine histology and direct immunofluorescence to evaluate antibody deposition, as well as serologies for autoantibodies. These tests also help distinguish type of ABD to determine optimal treatment modalities. For most cases of ABD, systemic immunomodulators are required.

      Bullous Pemphigoid

      Bullous pemphigoid (BP) is an ABD characterized by development of pruritic tense bullae.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Due to friction in the vulvar area, lesions progress from blisters to erosions. BP affects extracutaneous sites more commonly but can involve the vulva and cases of localized vulvar BP have been reported.
      • Mounsey SJ
      • Heelan K
      • Hughes S
      • Fawcett H
      • Bunker CB
      Localized genital bullous pemphigoid.
      It is important to distinguish BP from MMP. BP involves the keratinized vulvar epithelium and unlike MMP, does not involve the mucous membranes or lead to scarring. For localized mild disease, high potency TCS may be sufficient. However, BP usually requires use of systemic steroids in conjunction with steroid-sparing agents depending on severity of disease and patient co-morbidities: doxycycline, niacinamide, mycophenolate mofetil, azathioprine, dupilumab, rituximab, intravenous immunoglobulin.
      • Montagnon CM
      • Lehman JS
      • Murrell DF
      • Camilleri MJ
      • Tolkachjov SN
      Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.

      Mucous Membrane Pemphigoid

      Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is much less common than BP but more likely to affect the vulva and mucous membranes and difficult to diagnose and treat.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Montagnon CM
      • Lehman JS
      • Murrell DF
      • Camilleri MJ
      • Tolkachjov SN
      Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
      ,
      • Rashid H
      • Oldhoff JM
      • Esajas M
      • et al.
      Juvenile and adult vulvar pemphigoid, an under recognized entity: case series of fourteen patients.
      Early MMP is characterized by pain and irritation of the oral, ocular, and genital mucosa.
      • Montagnon CM
      • Lehman JS
      • Murrell DF
      • Camilleri MJ
      • Tolkachjov SN
      Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
      Blisters are less frequently seen in MMP as they rapidly progress to painful erosions. Chronic inflammation progresses to scarring and strictures. Late stage MMP is indistinguishable from chronic LS or EVVLP due to scarring and introital narrowing.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      MMP requires systemic immunosuppressants and is often recalcitrant to therapy.
      • Montagnon CM
      • Lehman JS
      • Murrell DF
      • Camilleri MJ
      • Tolkachjov SN
      Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
      TCS can be compounded for intravaginal use and dilators should be utilized to prevent introital narrowing.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Adhesions may require surgical intervention.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Pemphigus Vulgaris

      Pemphigus vulgaris (PV) affects the skin and mucous membranes and is characterized by painful, flaccid blisters and erosions.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Because of its predilection for the mucosa, vaginal involvement is likely more common than reported and pap smears in these patients exhibit acantholysis and inflammation consistent with PV.
      • Fairbanks Barbosa ND
      • De Aguiar LM
      • Maruta CW
      • et al.
      Vulvo-cervico-vaginal manifestations and evaluation of Papanicolaou smears in pemphigus vulgaris and pemphigus foliaceus.
      Similar to MMP, PV requires systemic immunomodulators and is often refractory to treatment.
      • Murrell DF
      • Peña S
      • Joly P
      • et al.
      Diagnosis and management of pemphigus: recommendations of an international panel of experts.
      TCS can be compounded for intravaginal use and dilators should be utilized to prevent introital narrowing.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Adhesions may require surgical intervention.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Linear IgA Bullous Dermatosis

      Linear IgA (Immunoglobulin A) bullous dermatosis is an ABD that involves the mucosa in up to 80% of adults and 64% of children, which can result in scarring.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Montagnon CM
      • Tolkachjov SN
      • Murrell DF
      • Camilleri MJ
      • Lehman JS
      Subepithelial autoimmune blistering dermatoses: clinical features and diagnosis.
      It is often associated with underlying conditions such as ulcerative colitis. There are drug-induced variants, the most common associated with vancomycin.
      • Montagnon CM
      • Tolkachjov SN
      • Murrell DF
      • Camilleri MJ
      • Lehman JS
      Subepithelial autoimmune blistering dermatoses: clinical features and diagnosis.
      Clinical findings are variable but vulvar mucosal erosions and perianal involvement can be seen.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      ,
      • Montagnon CM
      • Tolkachjov SN
      • Murrell DF
      • Camilleri MJ
      • Lehman JS
      Subepithelial autoimmune blistering dermatoses: clinical features and diagnosis.
      Treatment consists of removing any inciting agent if medication-induced, dapsone, and systemic corticosteroids as well as other immunomodulators.
      • Montagnon CM
      • Lehman JS
      • Murrell DF
      • Camilleri MJ
      • Tolkachjov SN
      Subepithelial autoimmune bullous dermatoses disease activity assessment and therapy.
      Vulvar directed therapies consist of high potency TCS and wound care.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.

      Vesiculobullous (Blisters): Other Vesiculobullous Disease

      Hailey-Hailey Disease

      Hailey-Hailey disease (benign familial Pemphigus) is an autosomal dominant vesiculobullous disorder of intertriginous areas that often presents as red papules and plaques with thickened white areas due to maceration.
      • Farahnik B
      • Blattner CM
      • Mortazie MB
      • Perry BM
      • Lear W
      • Elston DM
      Interventional treatments for Hailey–Hailey disease.
      Fissures, erosions, and small vesicles can be seen. The onset of Hailey-Hailey disease is typically in adolescence. Vulvar involvement includes pain and pruritus. Vegetative, malodorous papules and plaques may be seen.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Treatment is challenging and involves topical and oral corticosteroids, as well as antifungals and antibiotics because superimposed infections are common. Other therapies include methotrexate, cyclosporine, oral retinoids, dapsone, and naltrexone, as well as therapies focused on minimizing sweat including laser therapies, oral glycopyrrolate, and botulinum toxin injections.
      • Farahnik B
      • Blattner CM
      • Mortazie MB
      • Perry BM
      • Lear W
      • Elston DM
      Interventional treatments for Hailey–Hailey disease.
      ,
      • Lemieux A
      • Funaro D
      Recalcitrant vulvar Hailey-Hailey disease treated with alitretinoin and onabotulinumtoxinA: a case report.

      Fixed Drug Eruption

      Fixed drug eruption is a recurrent skin finding that occurs at the same location each time the patient is exposed to a culprit medication.
      • Drummond C
      • Fischer G
      Vulval fixed drug eruption due to paracetamol.
      Fixed drug eruption is usually a solitary lesion and the vulvar mucosa is commonly involved.
      • Drummond C
      • Fischer G
      Vulval fixed drug eruption due to paracetamol.
      A well-demarcated red to violaceous patch, blister, or erosion can be seen.
      • Edwards L
      • Lynch PJ
      Genital Dermatology Atlas and Manual.
      Numerous medications have been implicated including non-steroidal anti-inflammatory drugs, antibiotics, antiepileptics, and biologic agents. Management involves cessation of the offending medication and any local wound care if blisters or erosions are present.
      • Drummond C
      • Fischer G
      Vulval fixed drug eruption due to paracetamol.

      CONFLICTS OF INTEREST

      None.

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        Genital Dermatology Atlas and Manual.
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