History of Present Illness

A 55-year-old woman, gravida 2 para 2 presents to your office with itching and an intermittent painful burning sensation of the vulva. These symptoms have persisted for six months, and have gotten progressively worse. She tried an over-the-counter remedy containing benzocaine and aloe with limited improvement. She was seen by her internist on two occasions. She was treated with terconazole and later fluconazole for vaginal yeast; however, neither significantly improved her symptoms. She has noted that intercourse has become increasingly uncomfortable, and tried water-based lubricants with limited improvement. The symptoms are worst at night. She denies any vaginal bleeding, spotting, or abnormal discharge.

Her past medical history is significant for hypothyroidism, which is well controlled with medication. She has had two vaginal deliveries after uncomplicated pregnancies.

Physical Examination

General appearance

Well-developed, well-nourished Caucasian woman in mild discomfort

Temperature

37.0°C

Pulse

92 beats/min

Blood pressure

110/72 mmHg

Height

65 inches

Weight

130 lb

BMI

21.6 kg/m²

Heent

Oropharynx without lesions, thyroid non-enlarged and without masses

Abdomen

Thin, soft, non-tender, non-distended, no masses

Skin

No lesions

Vulva

Moderate atrophy with white, cigarette-paper plaques especially around the labia minora and coalescing around the anus. Thick 1.25-cm white plaque seen in the posterior fourchette. Absence of labia minora is noted. The clitoris is concealed.

Vagina

Stenotic and tender introitus with slightly flattened rugae. No lesions.

Cervix

Parous, no lesions, non-tender

Uterus

Anteverted, mobile, normal size, non-tender

Adnexa

No masses, non-tender

Anus

No lesions

How Would You Manage This Patient?

The most likely diagnosis is vulvar lichen sclerosus (LS). A careful analysis of history should consider the possibility of contact dermatitis, a common cause of similar symptoms. Common irritants, such as soaps, detergents, feminine hygiene products, and non-cotton underwear, should be eliminated. Vulvar hygiene should be emphasized. Many women with vulvar symptoms overuse products such as feminine hygiene wipes, which should be avoided. The examiner should inspect the oropharynx for aphthous ulcers. These, and other extragenital manifestations, are present in up to 50 percent of patients with lichen planus and may help in clarifying the differential diagnosis [1]. The thyroid should be palpated, as LS is believed to have an autoimmune origin. Up to 34 percent of women with LS will also have another autoimmune disorder, including thyroiditis, alopecia, pernicious anemia, or vitiligo [1]. The initial examination should include a vaginal pH, and microscopy utilizing potassium hydroxide and saline. Cultures for candida may be performed if indicated. A punch biopsy of the vulva, using a Keyes punch or a similar tool, should be performed under local anesthesia during this visit. This will confirm the diagnosis and exclude other benign as well as malignant skin conditions. Treatment with clobetasol ointment should be initiated once the diagnosis is confirmed. The patient should be re-examined at three and six months to assess response to treatment as well as progression of symptoms.

Lichen Sclerosus

LS is a chronic inflammatory skin condition, which is believed to have an autoimmune component [2]. Other proposed etiologies for this condition include infection with borrelia burgdorferei; however, this is controversial [1]. This disorder, first described in 1887, has also been called vulvar dystrophy and lichen sclerosus et atrophicus. Most commonly, LS is diagnosed in the fifth or sixth decades of life, but can also be seen in prepubertal girls. In prepubertal cases, it is best to avoid vulvar biopsy if possible, to reduce trauma. Otherwise the evaluation, management, and follow-up are similar to that recommended for older women.

Prepubertal girls account for approximately 7–15 percent of cases of LS [3].

A complete physical examination should be performed when LS is suspected. Examination of the oropharynx will help exclude lichen planus, which is sometimes confused with LS. Careful inspection of the vulva may also help clarify the diagnosis. Patients with contact dermatitis often exhibit significant thickening (lichenification) of the affected skin, and evidence of scratching may be seen. Lichenification is the result of what is known as the “itch-scratch cycle.” Infectious etiologies should also be excluded. The complete differential diagnosis that should be considered in women with the presentation described above is seen in Table 45.1.

Whenever vulvar skin disorders are detected, patients should be counseled regarding proper vulvar hygiene. Box 45.1 contains information regarding key counseling pearls.

The typical appearance of the vulva in a patient with LS can be seen in Figure 45.1, which shows white vulvar plaques that are coalescing lesions. There may be areas of purpura, excoriation, or erosion. Architectural changes of the vulva are common, including partial or complete resorption of the labia minora, scarring of the clitoral hood, and narrowing of the introitus [4].

Figure 45.1 Typical presentation of lichen sclerosus. Note hypopigmentation, loss of architecture, and scarring of the introitus.

Reproduced from the website http://cvvd.org/conditions/lichen-sclerosus with permission of Dr Andrew Goldstein. Accessed on 5/1/2017

Vulvar biopsies should be sent to a pathologist with expertise in vulvar skin disorders. Characteristic histologic features include hyperkeratosis of the epidermis, epidermal atrophy, loss of rete ridges, homogenization of the collagen of the upper dermis [4], dense bands of lymphocytic infiltrates, and vasculitic processes [5]. Although many practitioners make the diagnosis of vulvar LS based on history and clinical presentation, it is imperative that a vulvar biopsy be performed to confirm the diagnosis, especially in cases where a discrete lesion is seen. It should be noted that up to 6 percent of women with vulvar LS develop squamous cell carcinoma of the vulva. In fact, in one study 36 percent of patients with vulvar squamous cell carcinoma had coexisting or synchronous LS at the time of diagnosis [6], suggesting that even more careful follow-up than the standard annual examination recommended by ACOG may be warranted.

Therapy for LS is lengthy, and sometimes indefinite. Although no cure for LS exists, the symptoms can be controlled if appropriate treatment is initiated before destructive or permanent scarring occurs. Since the etiology of LS is likely autoimmune, it is not surprising that the current gold-standard for initial treatment of LS is ultrapotent topical corticosteroid therapy, with agents such as clobetasol or halobetasol. Ointments are generally better than creams, as they are absorbed more readily, and are less irritating due to a base gentler to sensitive skin. It has been reported that the majority of patients (96 percent) will become either symptom-free or will have partial response to this treatment. Older patients are less likely to achieve complete remission [5]. The ideal treatment regimen has yet to be determined. Most experts recommend once or twice daily application of ultrapotent steroid ointment for at least four weeks, and up to three months [2]. Although somewhat controversial, it is generally believed that this therapy reduces the risk of malignant transformation. Long-term use of corticosteroids can have significant side effects and other complications. These include severe atrophy, infection, thickening of the skin, and even adrenal insufficiency [4]. For this reason, other therapies have also been studied.

A Cochrane review found no benefit from topical testosterone, dihydrotesterone, and progesterone, and these should not be utilized. The immune-modulator pimecrolimus was found to be as effective as clobetasol in relieving itching and burning pain, but may be less effective in achieving visible improvement [7]. Pimecrolimus and tacrolimus are nonsteroidal immunomodulators when applied topically. The mechanism of action involves blockage of calcineurin, inhibiting the production of interleukin-2, interferon, and other cytokines. This leads to the suppression of T-cells and other inflammatory cells. Tacrolimus is lipophilic and penetrates skin easily, but unlike corticosteroids will not cause thinning of the skin [8]. Calcineurin inhibitors are significantly more expensive than corticosteroids. A theoretical concern is the risk of malignant transformation due to immune suppression, and although there are case reports of rapid tumor progression this has not been borne out in the literature [9].

Another promising agent is Human Fibroblast Lysate cream, obtained from cultured human fetal fibroblasts. The proposed mechanism of action is anti-inflammatory cytokines, including interleukin 1 receptor agonists, and growth factors, such as VEGF. In a randomized, placebo-controlled trial this therapy was shown to reduce vulvar symptoms; however, the results were not statistically significantly different from placebo. Further study of this agent is warranted [4].

Photodynamic therapy is another promising option for treatment of LS. A combination of 8-methoxypsoralen cream and UVA light led to significant reduction in pruritus. In separate studies UVA1 has also been reported to decrease symptoms and improve physical findings, as has the combination of 5-aminolevulinic acid and the argon laser. Limited studies have been done and more studies are indicated before this type of therapy could be considered as an alternative to standard therapy [9].

Emollient therapy, with petrolatum, mineral oil, or lanolin, has been shown to help control symptoms after a course of steroids or immune modulators has been completed [9].

Surgical therapy is sometimes utilized to correct anatomic abnormalities such as labial adhesions. Surgical treatment of LS is reserved for cases with poor response to medical therapies, and in some cases to prevent the development of malignancy. Surgical options include vulvectomy, cryosurgery, and laser ablation. Empirical vulvectomy is not indicated, however, there is some evidence that skinning vulvectomy may help prevent cancer in patients with mixed dystrophy, since these patients are at higher risk of malignant transformation. Recurrence rates are high with this therapy as well [10].

Summary

The patient should be offered clobetasol therapy after a confirmatory biopsy, and follow-up examinations should be conducted at three and six months to assess for improvement. Topical estrogen may be offered as an adjunctive therapy for atrophy. Emollients can be initiated once symptoms have improved or resolved. New lesions should be biopsied to rule out malignancy.

Key Teaching Points

• 
  

  Any hypopigmented vulvar lesion should be biopsied, and LS suspected.

  
  
• 
  

  LS is associated with a 4–6 percent rate of progression to squamous cell carcinoma. Patients with LS should have a thorough examination of the vulva at least annually.

  
  
• 
  

  Topical treatment with an ultrapotent steroid ointment is the first-line therapy for LS.

  
  
• 
  

  Immune modulators are as effective in treating LS as steroids.

  
  
• 
  

  Hormonal therapies, such as testosterone or progesterone, have not been shown to be effective in treating LS.

History of Present Illness

A 55-year-old nulliparous woman presents to the office for her annual exam complaining of a new lesion on her vulva. She reports that she first noticed a bump over the last couple of weeks. She reports that the lesion is irregular and rough to the touch and is located near her urethra. She denies any pain or associated bleeding with the lesion. She is otherwise healthy.

Her last cervical cancer screening was reported as normal at age 48. Her past medical history includes menopause at age 52; she has no medical problems nor has she had any surgery. She is on no medications. She denies any medication allergies but does report a latex allergy. She is an automotive engineer, denies smoking, and drinks seven alcoholic beverages weekly. She divorced at age 53. She reports a new male sexual partner for the last six months.

Physical Examination

Temperature

36.7 C

Pulse

75 beats/min

Blood pressure

128/65 mmHg

BMI

32 kg/m²

Ht

5′ 6″

Weight

186 lb

• 
  

  General appearance Well-appearing Caucasian female in no apparent distress

  
  
• 
  

  Abdomen soft, non-tender, no rebound tenderness, guarding or masses

Pelvic Exam

• 
  

  Vulva/Vagina Right labium majus has an irregular appearing lesion (see Figure 46.1). Normal vaginal discharge. Postmenopausal atrophy noted.

Figure 46.1 High-grade squamous intraepithelial lesion of vulva.

Cervix

Normal appearing, pale

Uterus

Anteverted, small, regular

• 
  

  Adnexa No adnexal tenderness or mass

  
  
• 
  

  There are no pertinent laboratory findings.

How Would You Evaluate and Manage This Patient?

This irregular appearing lesion requires biopsy. In addition, colposcopy of the vulva was performed to better visualize and document the extent of the lesion and to rule out additional pathology. Punch biopsy was performed after injecting 1 percent lidocaine with epinephrine. The biopsy returns as high-grade squamous intraepithelial lesion (HSIL) (previously called vulvar intraepithelial neoplasia-usual type). Treatment of HSIL of the vulva for this patient would include additional wide local excision if there was risk of occult invasion by punch biopsy or ablative therapy if no risk of occult invasion was noted on biopsy. If sensitive or important structures lie within the scope of the lesion, consultation with a vulvar specialist would be recommended.

Pap smear and high-risk HPV screening of the cervix is also performed during the exam consistent with current ASCCP screening guidelines and to rule out concomitant cervical disease. Testing for other sexually transmitted infections was performed.

Vulvar HSIL and Condyloma Accuminata

Vulvar HSIL is associated with high-risk HPV types (i.e., 16, 18, 31, 33, 45) and is considered a precancerous lesion. Vulvar HSIL related to HPV has recently been reported to be more common in women under 50 years. Common symptoms include itching, burning, soreness of the vulva, or painful intercourse. Discoloration of the vulvar skin can also occur.

The International Society for the Study of Vulvovaginal Disease (ISSVD) updated the terminology for vulvar intraepithelial neoplasia in 2015 (Box 46.1)[1] [2]. The ISSVD adopted the 2014 WHO (World Health Organization) Classification of Tumors of the Vulva that divides lesions into squamous cell tumors and precursors that include squamous intraepithelial lesions (SILs), subdivided into LSIL (low-grade SIL), HSIL (high-grade SIL), and differentiated-type VIN (DVIN). What previously was called usual or undifferentiated VIN is now included in HSIL [3].

Treatment of HSIL of the vulva varies depending on the location of the disease. Wide local excision (margins of 0.5 to 1 cm) is preferred in hair-bearing areas or in cases where occult invasion is of concern as may be suggested from the punch biopsy [4]. CO₂ laser is ideal for non–hair-bearing areas, which requires a 2 mm depth. Laser should not be performed on the hair-bearing areas as the depth required to ensure eradication around the follicles is 3–4 mm in depth and would be painful and disfiguring. An alternative treatment for HSIL of the vulva is the use of topical imquimod (off label use). Topical imiquimod is particularly useful when HSIL is found on the clitoris or urethral meatus.

Recurrence rates for HSIL of the vulva can range from 9 to 50 percent, thus ongoing surveillance is important. Initially, patients should be examined at 6 and 12 months after initial diagnosis and excision/ablation. If no lesions are seen, then annual examination is appropriate [5].

Condyloma accuminata would be classified as benign squamous lesions in the aforementioned classification of the ISVVD [1]. If a vulvar biopsy is performed and returns as condyloma accuminata or anogenital warts (AGWs), the clinical suspicion of a low-risk HPV lesion is confirmed. Ninety percent of condyloma accuminata are caused by HPV 6 and 11 [6,7]. HPV genital infections are the most common STD in the world. Over 50 percent of the sexual population is infected with HPV at some point in time. Recurrence rates for condyloma accuminata have not been clearly delineated but can be common. AGWs are highly contagious – an HPV negative sexual partner has a 65 percent risk of developing an AGW three weeks to eight months after contact with an infected partner. If there is a suspicion of immunosuppression in the patient, further evaluation may be warranted. In patients where condyloma is newly diagnosed, other STI screening tests, including HIV, should be considered.

For lesions with the appearance of typical condyloma accuminata in premenopausal women, biopsy is not warranted (see Figure 46.2). When treatment is indicated, in-office treatments can be administered or self-application treatments at home can be prescribed.

Figure 46.2 White condyloma on vulva.

Trichloroacetic acid (TCA) can be applied immediately in the office. Other in-office treatments include cryotherapy, podophyllin, interferon, and sinecatechins (green tea extract) [6]. Alternatively, if the patient does not wish to return for multiple applications of topicals, excision or CO₂ laser with local anesthesia can be performed. At home self-application treatments include Podofilox and imiquimod cream, which are applied topically [7]. Indications for biopsy in the premenopausal state include lesions that do not respond to therapy, the disease worsens during therapy, the lesion becomes atypical, or the patient has compromised immunity.

Extensive lesions may require surgical excision in addition to ablation and can be co-treated with topical medications to decrease the volume of excision. Cotreatment with topical agents, such as imiquimod, can also be used to decrease recurrence rates [8].

Key Teaching Points

• 
  

  Vulvar lesions in postmenopausal women and atypical vulvar lesions in women under 50 require biopsy.

  
  
• 
  

  HSIL diagnosed on vulvar biopsy with concern for occult invasion requires wide local excision or ablative therapy depending on concern for occult invasion.

  
  
• 
  

  Recurrence of HSIL is common; thus, ongoing disease requires surveillance, initially every six months and then annually thereafter.

  
  
• 
  

  Benign condylomas are amenable to multiple treatments. Imiquimod and podofilox may be successfully administered at home.

  
  
• 
  

  Extensive condylomas can be treated with a combination of topical medication and excision or laser.

History of Present Illness

A 25-year-old G0 female is referred for evaluation and management of pain with intercourse. She reports a one-year history of severe dyspareunia that began when she became sexually active with her husband, who has been her only partner. She has never been able to use tampons because of pain with attempted insertion. She rates her initial pain as 10 out of 10 and describes it as most prominent with penile insertion and dissipating somewhat with continued coitus. She also reports burning pain following intercourse that may last for a few hours. Her symptoms were significantly interfering with her marriage, so her primary gynecologist offered a trial of topical lidocaine 5 percent cream and topical estrogen cream. The lidocaine has improved the insertion pain to a 6 out of 10, but she still has significant discomfort with intercourse and postcoital burning.

The patient’s past medical history is positive only for irritable bowel syndrome. She recently stopped her oral contraceptive pills. Her current medications include prenatal vitamins, Colace, hyoscyamine, and topical lidocaine and estrogen creams.

Physical Examination

General appearance Patient is a well-developed, well-nourished female in no acute distress, appearing her stated age.

Height

155 cm

Weight

62 kg

Blood pressure

100/60 mmHg

Abdomen

Soft, thin, mildly diffusely tender without masses, guarding, or rebound

External genitalia

Anatomically normal female external genitalia, no suspicious lesions, cotton swab testing confirmed point tenderness of vaginal vestibule lateral to the hymen at 1, 11, 4, and 8 o’clock with slight erythema

Vagina

Normal, non-tender, no discharge

Bladder

Normal

Cervix

Normal

Uterus

Anteverted, small, mobile, non-tender

Adnexa

No masses or tenderness; pelvic floor non-tender

How Would You Manage This Patient?

This patient has primary, localized, provoked vestibulodynia, previously referred to as vulvar vestibulitis, which was confirmed on cotton swab testing. Fungal cultures were obtained and were negative. According to Freidrich’s criteria [1], initially described in 1987, this patient’s symptoms and physical exam are pathognomonic for this condition. These criteria include (1) severe pain on vestibular touch or attempted vaginal entry; (2) tenderness to pressure localized within the vulvar vestibule; and (3) no evidence of physical findings except for varying degrees of erythema.

The patient was instructed on vulvar care measures and was thoroughly counseled about the treatment options. Although she had a partial response to topical lidocaine, she found the precoital application to be intrusive and non-spontaneous. She elected a trial of oral lamotrigine 25 mg daily, which was titrated up by 25 mg each week to a dose of 100 mg daily. She reported some improvement at her three-month follow-up and is considering the addition of physical therapy with biofeedback.

Vulvodynia and Vestibulodynia

Vestibulodynia is a subset of idiopathic vulvodynia, which is now considered a chronic pain disorder. To make the diagnosis of idiopathic vulvodynia, a patient must have vulvar pain of at least three months’ duration in the absence of other identifiable pathology. The International Society for the Study of Vulvovaginal Disease (ISSVD) released updated terminology guidelines in April 2016 [2]. These were reiterated in a subsequent combined ACOG/ASSCP Committee Opinion from 2016 [3].

According to the ISSVD, vulvodynia classification can be broken down into four major areas:

1. 
   

   1. Onset – primary, as in this patient, or secondary, occurring after the onset of sexual activity;

   
   
2. 
   

   2. Location – localized, generalized, or mixed;

   
   
3. 
   

   3. Timing – provoked, spontaneous, or mixed; and

   
   
4. 
   

   4. Temporal pattern of pain (constant, intermittent, etc.).

The prevalence of vulvodynia is about 8–16 percent of the female population and affects women of all age and ethnic groups [4]. Localized, provoked vestibulodynia refers to a subset of vulvodynia in which the pain is localized to the vaginal opening or vestibule when the area is provoked by pressure. Provoked vestibulodynia accounts for about 80 percent of idiopathic vulvodynia cases and is more common in reproductive-age women.

The cause of vulvodynia is not known and likely heterogeneous. Studies examining pathophysiology and histopathology have shown results such as peripheral nerve proliferation, alterations in local immune factors, and chronic inflammatory changes [5]. Some women, like the patient described above, will have pain from their first attempt at tampon insertion or penile penetration. For women with secondary vestibulodynia, the onset of pain may be linked temporally to a specific trigger or event, such as a severe candida vulvovaginitis. For most women, however, the onset is more insidious. Vulvodynia can cause significant distress and relationship difficulties because of the impact on sexual function. Many studies have documented a significant association between vulvodynia and other chronic pain conditions, such as interstitial cystitis, fibromyalgia, and irritable bowel syndrome [6]. Depression and anxiety are also common comorbid conditions.

The evaluation of a woman with complaints of vulvar pain should include a thorough medical, obstetrical, and gynecologic history. The American College of Obstetricians and Gynecologists’ 2016 Committee Opinion outlines an assessment and treatment algorithm that is helpful in the work-up of persistent vulvar pain [3]. The history should include assessment of urinary tract symptoms, a sexual history, validated sexual distress scale, review of musculoskeletal symptoms, and history of physical or sexual abuse. The external genital exam should be approached systematically, first inspecting the vulva for any lesions, discolorations, or other abnormalities that could be contributing to the patient’s symptoms. The standard for assessing tenderness is the use of a cotton swab test. This involves lightly touching the vulva in a consistent pattern, starting with the most lateral aspects and working medially, quantifying patient’s pain response at each area on a ten-point Likert scale. A yeast culture should be obtained in women who report pain and/or burning on cotton swab testing, and those who test positive should be treated with an antifungal. The vagina should be assessed for signs of infection or inflammation as well as for vaginal wall and pelvic floor tenderness. In addition, it is important to assess for involuntary contraction of the vaginal and pelvic floor muscles, as the presence of vaginismus would modify the management approach. In the absence of abnormal physical exam findings, vulvar biopsy is not useful.

Evaluation and Treatment Algorithm

Figure 47.1 shows the distribution of pain in women with generalized vulvodynia and Figure 47.2 shows the distribution in women with provoked vestibulodynia. With provoked, localized vestibulodynia, tenderness is usually concentrated around the openings of the minor vestibular glands, which are located medial to Hart’s line and lateral to the hymenal ring (Figure 47.3), as well as the posterior fourchette.

Figure 47.1 Generalized, unprovoked vulvodynia

Figure 47.2 Localized, provoked vestibulodynia

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1. Section I – Pelvic Pain
2. Section VI – Cancer Screening and Prevention
3. Section XI – Pelvic Masses and Cysts
4. Section XII – Incontinence and Prolapse

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