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Vulvar Diseases in Primary Care The Visible Lesion: Is it Cancer?

D. Ashley Hill, M.D.
Associate Director Department of Obstetrics and Gynecology
Florida Hospital Family Practice Residency
Orlando, Florida

Although the vulva only comprises a small amount of the body's surface area, it is host to a surprisingly large number of diseases and ailments. Although many vulvar conditions cause symptoms such as burning, pruritus, other discomfort, or discharge, many do not. Thus, our patients may not seek the services of a health care provider until their condition is in a relatively advanced stage. Furthermore, many vulvar cancers are ignored by health care providers until they have reached an advanced stage, which often leads to radical and extremely disfiguring surgery. An understanding of the common nonneoplastic vulvar disorders that may mimic cancer, potentially precancerous lesions, and vulvar cancer itself will arm the primary care health care provider with the information needed to manage many of these conditions and to identify those needing biopsy or referral.

The participant should be able to:

  1. Identify common vulvar conditions, particularly those mimicking vulvar neoplasia.
  2. Understand the steps in evaluating vulvar conditions.
  3. Identify those lesions requiring biopsy.
  4. Identify vulvar conditions that require potential surgical management or referral.

Summary of Vulvar Conditions

Infectious

  • Candida (yeast)
  • Trichomonas
  • Bacterial vaginosis (Gardnerella)
  • Tinea cruris (i.e. Trichophyton rubrum)
  • Sexually transmitted infections (i.e. IAPV, herpes, syphilis, LGV, chancroid)
  • Parasites and Insect bites

Allergic

  • Contact dermatitis

Systemic

  • Psoriasis
  • Seborrheic dermatitis
  • Fox-Fordyce disease
  • Pemphigus
  • Stevens-Johnson Syndrome
    • Hepatitis
    • Severe renal disease
    • Sjogren's syndrome
    • Diabetes (i.e. pruritus)

    Epithelial Disorders (Nonneoplastic)

    • Lichen sclerosus
    • Squamous hyperplasia
    • Vulvar vestibulitis
    • Lichen simplex chronicus
    • Lichen planus

    Raised Lesions or Masses

    • Nevi
    • Bartholin gland cysts or abscesses
    • Inclusion cysts
    • Other various cysts

    Potentially Premalignant or Malignant


    • Vulvar intraepithelial neoplasia (VIN)
    • Paget's Disease
    • Melanoma
    • Squamous cell carcinoma

    Miscellaneous Vulvar Disorders


    • Factitial dermatitis
    • Genital atrophy
    • Trauma (i.e. hematoma, sexual assault)

    It is evident the list of possible vulvar abnormalities is exhausting. When confronted with a vulvar lesion, it is helpful to "categorize" these using a system that helps jog the memory and decreases the chance of missing a diagnosis. Therefore, this paper will focus on visible lesions of the vulva that may mimic cancer. Tips on office evaluation and biopsy techniques are discussed, as well as basic treatment strategies.

    Outline

    1. White Lesions
      • Lichen sclerosus
      • Squamous cell hyperplasia
      • Leukoplakia
      • HPV
      • VIN
      • Squamous cell carcinoma of the vulva

    2. Red or Dark Lesions
    • Seborrheid dermatitis
    • Psoriasis
    • Lichen planus
    • Lentigo
    • Paget's disease
    • Nevi
    • VIN or cancer
    • Malignant melanoma

    White Vulvar Lesions

    Lichen sclerosus:

    Lichen sclerosus is a benign disorder of the vulvar epithelium that can affect women of any age group. It causes thinning of the vulvar tissue with edema and fibrosis. If untreated, the labia may fuse together, the clitoris may shrink, and the introitus may become stenotic. The skin has a white, thin, shiny, "parchment paper" appearance. Fissures may be present. Bilateral symmetry is common. Some patients are asymptomatic, while others report pruritus, painful intercourse, and anorgasmia from clitoral shrinkage. There is a familial tendency. The diagnosis is by biopsy (see description of biopsy techniques below). Pathologists identify lichen sclerosus by hyperkeratosis, edema, loss of vascularity, and loss of rete ridges. Treatment has changed somewhat over the years. Formerly, testosterone cream was used, but most experts currently use high-potency steroid creams such as clobetasol propionate 0.05% twice daily for 3 weeks, then before bed each night for a few months. This disorder may require a lifetime of treatment, including either high-potency steroids once or twice weekly, or over the counter steroids every night. Long term use of high-potency steroids is associated with erosion, fissuring, and contractures, therefore lower-potency medications should be substituted if possible for chronic therapy. Patients need annual exams and biopsy of any worsening or new lesions, as progression to cancer can occur in about 3% of these cases.

    Squamous cell hyperplasia

    Squamous cell hyperplasia is a common and benign vulvar condition that can look similar to other white vulvar lesions, including condyloma, VIN and carcinoma. The skin is typically white or off-white, with focal lesions. The cause is unknown, but may be due to a vulvar irritant callusing thickening of the skin. Pruritus is commonly found and may bring the patient to her health care provider. Diagnosis is by biopsy. Colposcopy with acetic acid application may reveal a thickened epithelium, with possible inflammation. Biopsy yield hyperkeratosis. Treatment consists of twice daily steroids for 3 weeks, then once daily thereafter until symptoms disappear. Chronic treatment with OTC steroids is sometimes necessary. Patients should also decrease exposure to vulvar irritants, for example perfumes, dyed toilet paper, etc. Biopsy of any new or persistent lesions is advocated as 4% of these patients may develop squamous cell carcinoma of the vulva.

    Leukoplakia:

    Vulvar leukoplakia is caused by vulvar irritation. It produces hyperkeratosis (excessive surface keratin) and creates a white, plaque-like area that is often strikingly white even without acetic acid application. While leukoplakia itself is a benign process, it may cover or be associated with an underlying VIN or carcinoma, therefore biopsy is prudent to rule out a more ominous disorder.

    HPV:

    A complete discussion of HPV is beyond the scope of this presentation. However, from the viewpoint of a health care provider examining a patient's vulva, HPV can mimic a number of other disorders, including VIN and even vulvar cancer. Some advocate biopsy of HPV lesions before starting treatment, while others suggest simply treating the warts with standard measures. Some studies have shown that 80% or more of VIN in younger women is associated with HPV. HPV lesions on older patients, or those with associated leukoplakia or other atypical appearances, should be evaluated by biopsy and histology to rule out VIN.

    VIN:

    Vulvar intraepithelial neoplasia is a potentially precancerous condition that can be either white, dark, or red. Excess keratin production leads to a white appearance, whereas excess melanin production leads to dark lesions. Formerly, many confusing terms existed to describe VIN, including Bowen's disease. Currently, VIN I refers to mild dysplasia, VIN II equals moderate dysplasia, and VIN III denotes severe dysplasia (also called carcinoma in situ). VIN may be focal or affect multiple sites on the vulva. Colposcopy with acetic acid may help delineate areas of VIN and make biopsy easier. As discussed previously, VIN in younger women is strongly associated with HPV (particularly types 16 and 18), and the epidemic of HPV infections has led to VIN in young women.

    VIN may be asymptomatic and can come to the attention of a patient's health care provider during an annual examination. Pruritus, however, may be present, as well as a burning sensation of the vulva. Biopsy of any white, red, or dark lesion is prudent, particularly in the older patient. Progression from VIN to cancer is uncommon in younger women, but more likely in older patients. Diagnosis is by biopsy, with colposcopy and acetic acid application. Multiple areas may need biopsy. The risk for progression to cancer is actually low for VIN, particularly in younger patients, but treatment is still necessary prevent patients from disease progression. Many treatment options exist, including topical 5-fluorouracil, laser vaporization (particularly useful for with low risk of subsequent cancer), wide local excision (the usual treatment of choice), and "skinning" vulvectomy for more advanced disease. Wide local excision is often adequate, as long as margins are free of dysplasia and vulvar colposcopy with acetic acid rules out other areas of VIN.

    Vulvar carcinoma:

    See discussion later in this presentation.

    Red Vulvar Lesions

    Seborrheic dermatitis:

    This vulvar disorder causes generalized erythema of the vulvar skin with red or yellow-brown plaques. These lesions may be covered by a nonadherent, oily scale. Pruritus and the finding of similar extraovular lesions increases suspicion, and biopsy make the diagnosis. Seborrheic dermatitis may mimic chronic candida, psoriasis, contact dermatitis, and, if focal, VIN, although this is usually a generalized disorder of the vulva. Treatment consists of topical steroids.

    Psoriasis:

    About 3% of adult women have psoriasis, with approximately 20% of these women having vulvar involvement. There is a familial tendency. Vulvar disease is manifested by red or red-yellow papules in intertriginous areas, with scales that produce pinpoint bleeding when removed. These lesions may enlarge to form focal, red plaques. This condition may mimic candidiasis. Diagnosis is clinical or preferably by biopsy, and treatment consists of topical 1% hydrocortisone cream, with referral to a gynecologist or dermatologist if treatment is unsuccessful.

    Lichen planus:

    Lichen planus can by an intensely pruritic and painful condition that may be an autoimmune phenomenon. Small, flat, violaceous papules develop on mucous membranes, including the vulva. If untreated, intense inflammation, ulceration and vulvar damage may occur. Biopsy will confirm the diagnosis, and treatment instituted with topical steroid creams. Oral steroids may be necessary for intense pruritus.

    Lentigo:

    Lentigo produces freckle-like, pigmented lesions that may be solitary or, more frequently, multifocal. This is a benign condition, but may be confused with melanoma (i.e. lentigo melanoma), therefore biopsy or excisional biopsy is necessary.

    Nevi:

    Nevi are not uncommon on the vulva, and can have a variety of color schemes, ranging from brown, brown-black, black, flesh-colored, or red. Since about 30% of melanomas arise from preexisting nevi, biopsy is mandatory for any nevus that has changed appearance, size, or color. Some advocate biopsy of all vulvar nevi, since patients are often reluctant to self-examine this area.

    Paget's disease:

    Paget's disease of the vulva can produce lesions that are white or red. However, an eczematoid, erythematous lesion is most common. Paget's disease produces pruritus. This condition is associated with malignancy of the vulva and other extraovular locations, such as the breasts and colon. Biopsy is mandatory, and treatment is wide local excision, although, unfortunately, local recurrence is not uncommon.

    Vulvar carcinoma and melanoma:

    Vulvar carcinoma is an uncommon gynecologic malignancy, representing 4-5% of all gyn tumors. Most vulvar carcinomas are squamous cell (90%) while about 5% are melanomas. Squamous carcinomas usually appear as vulvar masses of irregular and polypoid shape. Sometimes leukoplakia is present, and these lesions can be white, red, or pigmented. Vulvar cancer may appear as benign diseases such as nevi, dermatoses, or nonneoplastic conditions like squamous hyperplasia. Later-stage or advanced disease can produce a large, exophytic vulvar mass, but less advanced disease may arise as a smaller lesion. Sadly, many patients report a "sore" that has not healed for over a year before seeking medical attention. Any health care provider evaluating a vulvar lesion in a patient with a history of a non-healing sore or chronic pruritis should strongly consider biopsy, with colpgscony if necessary, or referral for biopsy. The vulva is rich in lymphatics so these cancers tend to spread to the groin (inguinal) nodes. Prognosis depends on the lesion size and extent of spread. Treatment depends on the extent of disease and anatomic location. Some lateral tumors are treated with radical wide excision, and possible ipsilateral inguinal-femoral lymphadenectomy. More extensive disease may require bilateral lymphadenectomy, and some cases require a modified radical vulvectomy, a disfiguring procedure. Thus, prevention of this disease by treating VIN, or diagnosis at an early stage, may allow the gynecologic-oncologist to treat this disease by less radical methods.

    Melanoma typically appear like melanoma in other areas. It occurs mainly on the labia minora and clitoris, in white women between ages 50-80. Biopsy proves the diagnosis, and surgical excision via radical wide local excision with large margins is acceptable treatment.

    Vulvar Biopsy

    Vulvar biopsy is a surprisingly easy office-based technique. Colposcopy of the vulva with acetic acid application, which can be challenging, will often help locate the optimum biopsy site. One should consider performing a biopsy for any of the following vulvar lesions:

    1. Any enlarging vulva lesion or one that has changed color or appearance.

    2. Lesions that are raised or pigmented.

    3. Presumed BPV unresponsive to office treatments.

    4. Vulvar dermatoses where the diagnosis is in doubt or there is no response to treatment.

    5. Any lesion that appears malignant.

    6. Any lesion that has associated white or thickened areas.

    Vulvar biopsy can be performed by shaving the lesion, excising with scissors or scalpel, or using a Keyes punch. I prefer the scissors or 4mm Keyes punch. After informed consent is given, the patient undergoes colposcopy if indicated. The vulva is cleansed with betadine or a similar agent, and local anesthesia is always applied, usually using a 25-gauge needle with lidocaine or a similar agent. Usually 1-3 cc is necessary to produce an adequate wheal, which will raise the lesion slightly, making biopsy easier.

    A representative area or the actual lesion is removed with the scissors, scalpel, or Keyes punch, and sent for permanent pathologic section. If a defect larger than a pencil eraser is produced there may be difficulty with hemostasis, requiring placement of an interrupted 4-0 or 3-0 delayed absorbable suture (for example vicryl). However, most of these excision sites respond well to silver nitrate application or direct pressure. The patient should use hydrogen peroxide and perhaps Neosporin or Polysporin ointment for about a week to facilitate healing. Infection and subsequent bleeding are rare, but if the patient reports increasing pain, she should be evaluated immediately. The results of her biopsy can be discussed at a 2-week follow up appointment.

    References

    1. Vulvar nonneoplastic epithelial disorders. ACOG educational bulletin number 241, 1997. American College of Obstetricians and Gynecologists, Washington, DC.

    2. Cattaneo A, Bracco GL, Maestrini G, Carli P, Taddei GL, Colafranceschi M, et al. Lichen sclerosus and squamous hyperplasia of the vulva: a clinical study of medical treatment. J Reprod Med 199 1;36:301-5.

    3. Apgar BS, Cox JT. Differentiating normal and abnormal findings of the vulva. Ain Fmn Physic 1996;53:1171-1180.

    4. Tovell HMM Young AW Jr. Diseases of the Vulva in Clinical Practice. 199 1, New York: Elsivier.

    5. Wilkinson EJ, Stone IK. Atlas of Vulvar Disease. 1995, Baltimore: Williams and Wilkins.

    6. Freidrich EG Jr., Wilkinson EJ, Fu YS. Carcinoma in situ of the vulva: a continuing challenge. Am J Obstet Gynecol 1980; 136:830-43.