Vulval disease is common and although most of the conditions presenting in general practice are straightforward, a significant number of women pose rather more of a diagnostic and management problem.
The following are the important points to consider:
(1) What are the predominant symptoms: irritation, soreness or burning?
Is there an urge to scratch or is the skin too sore?
Is the whole vulva affected or are symptoms localized to one particular area?
(2) Is there a personal or family history of allergy?
(3) Any history of skin problems, for example dermatitis/eczema, psoriasis, lichen planus?
(4) Which soap is used for cleansing the genital area? Are bubble- bath, hygiene sprays, etc. used?
(5) Are symptoms related to the time in the menstrual cycle or brought on by coitus?
Although candidiasis is the commonest cause of vulval irritation, this diagnosis should be reconsidered if vaginal swabs fail to grow the fungus and there is no response to antifungal treatment. If there is doubt, consider using a longer course of an oral antifungal (e.g. itraconazole or fluconazole) as a diagnostic test. If there is no clinical response to antifungals in spite of Candida being isolated on culture, ask the laboratory to identify the Candida spp., as some of the more unusual strains (e.g. Candida glabrata) may be resistant to the commonly used imidazole and triazole preparations.
Although the vulva may be affected by a variety of skin conditions, the clinical features are often modified by secondary infection, scratching (causing lichenification or skin thickening), or by previous treatments. Examination of the scalp, nails, elbows, and mouth may provide useful clues to the diagnosis.
8.1 VULVAL IRRITATION
Conditions that may present with vulval irritation include the following:
(1) Candidiasis (see above, Chapter 5 and Figures 5.1 to 5.3).
(2) Human papillomavirus (HPV) infection (see also Chapter 18). Genital warts can cause slight irritation and when they first appear may be quite difficult to identify without some form of magnification, such as a colposcope. (Note: Anal warts may present as pruritis ani; beware the diagnosis of hemorrhoids without careful examination!) Vulval intraepithelial neoplasia (VIN, Figure 8.1) is strongly associated with HPV type 16 infection and often presents as white or off-white, flat or papular lesions, most commonly affecting the labia minora and perineum. Lesions are multifocal in 70% of women and cause irritation in just under two-thirds. Biopsy should be considered to confirm the diagnosis and stage the lesion (VIN I, VIN II, or VIN III). VIN has the potential to progress to squamous cell carcinoma, particularly in the more mature woman, and therefore careful follow-up is advisable. In addition, as VIN is associated with dysplasia elsewhere in the genital tract, it is important to ensure that cervical cytology, and if possible colposcopy, is performed on a regular basis, ideally annually.
Figure 8.1. Vulval intraepithelial neoplasia (VIN)
(3) Genital herpes (see also Chapter 17). Some women report vulval irritation before ulcers appear. With primary genital herpes, the irritation is soon superseded by increasing soreness and subsequently ulceration and vulval edema. The typical blisters are fragile and often missed. A history of a "flu-like" illness or sore throat prior to the onset of the vulval symptoms is often a helpful diagnostic clue. Women presenting with primary genital herpes often give a history of supposed "thrush" that has worsened whilst using antifungals.
In recurrent herpes, the vulval lesions may be tiny and easily overlooked unless the patient or examining clinician is alert to the possible diagnosis. Examination with a magnifying glass or colposcope can be helpful in these cases.
(4) Trichomoniasis. Trichomonas vaginalis usually causes a vulvovaginitis associated with an increased vaginal discharge. Diagnosis is by wet-mount microscopy or culture (see Chapter 6, Figure 6.1).
(5) Streptococcal infection. Although both Lancefield Group A and Group B streptococci may cause a vulvovaginitis, this is uncommon and vulval infection usually occurs secondarily to an already damaged vulval skin, for example from dermatitis. Vulval erysipelas is usually associated with Group A streptococci and presents as pronounced labial swelling and erythema which may progress to necrosis.
(6) Dermatoses. These are not uncommon and often involve the labia majora and perineum.
(a) Seborrheic dermatitis (Figure 8.2). Look for evidence elsewhere, such as on the face, chest, and scalp.
(b) Contact dermatitis. There is often a history of allergies or family history of atopy. Check whether any creams or lotions are being applied to the genital area. Latex allergy usually presents as vaginal soreness after using condoms. Seminal fluid, KY jelly, or spermicide allergy presents as postcoital vaginal discomfort sometimes associated with vulval edema. Scented soaps, bubble-baths, hygiene sprays, antimicrobial creams, and anesthetic hemorrhoid creams are potential sensitizers.
(c) Lichen simplex (Figure 8.3). Some degree of skin thickening or lichenification is common after chronic scratching. Treatment with a moderately potent topical steroid is often required.
(d) Lichen planus (Figure 8.4). Look for evidence elsewhere, particularly in the mouth. Erosive lichen planus is a variant that may present with severe vulvitis and vaginitis.
(e) Psoriasis (Figure 8.5). Look for evidence elsewhere, including nail pitting, and ask about family history. Lesions in the genital area may not appear typical as the scale is often lost leaving a red, glazed epithelium.
(f) Lichen sclerosus (Figure 8.6). Commonly affects the perianal and genital regions in children and adults. Often presents with irritation and less commonly soreness. Sexual intercourse can be painful either because of friction damaging the fragile vulval skin or secondary to tightening of the vaginal introitus resulting from postinflammatory scarring. in the early stages the skin appears white and slightly thinned sometimes with small, superficial erosions and "blood blisters." Untreated, the inflammatory process may lead to resorption of the labia minora and clitoris and narrowing of the introitus (Figure 8.7). Active disease should be treated initially with a potent topical steroid (e.g. clobetasol propionate). Long-term follow-up is recommended because of the small risk (up to 4%) of developing squamous cell carcinoma.
Figure 8.2. Seborrhoeic dermatitis
Figure 8.3. Lichen simplex
Figure 8.4. Lichen planus
Figure 8.5. Psoriasis
Figure 8.6. Lichen sclerosus - atrophic changes
8.1.1 A Short note about Topical Steroids
Patients are often concerned that topical steroids will damage the skin, particularly in the genital region, and may therefore fail to treat themselves adequately. it is worth reassuring patients that steroid creams and ointments are safe to use under clinical supervision and are required, sometimes in high strength and for long periods of time, to adequately treat skin problems. Not too much cream need be applied and suggesting to the patient that a tube should last a year or two may help to avoid over treatment. Creams sometimes sting a little more on application than ointments but may be easier to apply to mucosal surfaces. Combined steroid and anti-infective preparations may be required to treat genital dermatoses but be alert to hypersensitivity reactions to the topical antibiotic components (e.g. neomycin, tetracycline). Some conditions (e.g. lichen sclerosus) should be initially treated with a potent topical steroid and then a weaker preparation substituted after a few weeks when symptoms have improved.
Figure 8.7. Lichen sclerosus - showing adhesion formation between the labia
8.2 VULVAL SORENESS OR TENDERNESS
All of the above conditions may cause soreness in addition to or rather than irritation.
8.2.1 Vulvar Vestibulitis
This is an important, frequently misdiagnosed, or missed condition that causes pain on sexual intercourse, particularly penetration. Tampons may also be too uncomfortable to use. it would be reasonable to say that all women presenting with insertional dyspareunia should be considered to have vulvar vestibulitis until proven otherwise. The condition presents as small areas of localized erythema (Figure 8. 8) and tenderness at the introitus, classically over the vestibular gland openings at the 5 o'clock and 7 o'clock positions. Some form of magnification, such as a colposcope, will often be required to see the lesions adequately. The cause of vulvar vestibulitis is currently unknown. Some women experience pain from coitarche whilst others give a history of years of pain-free sexual intercourse. A variety of treatments have been used in this condition with, unfortunately, often poor response. These include topical steroids, topical estrogens, intralesional triamcinolone, cryotherapy, and laser ablation. Modified vestibulectomy has produced good results in some studies but patients need to be selected with care. Some women show marked introital sensitivity, with light touch with a cotton wool swab invoking marked tenderness (allodynia). Low dose amitriptyline (10 mg initially slowly increasing to 50 mg or 75 mg, if tolerated) or pregabilin can prove helpful in these cases.
Figure 8.8. Vulvar vestibulitis - area of erythema at introital 7 o'clock position
8.2.2 Posterior Fourchette Tear
Posterior fourchette tears cause pain during sexual intercourse, sometimes associated with bleeding. Examination with a colposcope may be required to make the diagnosis as the tears are often very small. Although a mild strength combined steroid/ antibacterial cream may prove helpful, some women are prone to recurrences. Tearing is sometimes associated with a bridge of skin at this site, in which case surgical removal (e.g. modified Fenton's procedure) should be considered (Figure 8.9).
Figure 8.9. Posterior fourchette tear
8.3 VULVAL BURNING
"Essential vulvodynia" or "dysesthetic vulvodynia" is the term used to describe symptoms of vulval burning with a normal appearing epithelium. Pudendal neuralgia is an important cause with some patients demonstrating diminished sensation in the sacral sensory distribution. Benign sacral meningeal cysts have been reported to cause genital pain and burning in both men and women; the diagnosis being made by magnetic resonance imaging of the lumbosacral spine. i would however suggest a neurological referral or alternative specialist opinion before requesting MRI scans on your patients with genital pain.
In the majority of patients, however, no obvious physical cause can be found for their symptoms in which case psychological issues should be considered and addressed.
Management may include the use of ‘pain modifiers', such as low to medium dose amitriptyline, prothiaden or fluoxetine, pregabalin, gabapentin, hypnosis, acupuncture, transcutaneous electrical nerve simulation (TENS) or caudal injection. Amitriptyline or pregabalin are useful first line options.
8.4 OTHER VULVAL CONDITIONS
8.4.1 Vulval Edema
The lax vulval skin is prone to edema and is particularly associated with infections such as herpes, candidiasis, and syphilis, although the latter is uncommon in women in the UK nowadays. Edema is an occasional feature of contact dermatitis and has been reported following intercourse in women with semen allergy. Vulval edema may be a presenting sign of Crohn's disease and intrapelvic pathology.
8.4.2 Angiokeratomata
These small lesions usually appear on the labia majora as tiny, often multiple, bright red vascular spots (Figure 8.10). They may increase in number and size with age and are harmless.
8.4.3 Melanocytic Naevi
These may appear anywhere on the vulva or perineum and have the same characteristics as naevi elsewhere on the body.
See also Chapters 17 and 18.
Figure 8.10. Angiokeratomata
8.5 IMPORTANT MANAGEMENT POINTS FOR VULVAL DISEASE
• Vulval moistness may increase the risk of secondary infection with yeasts or bacteria. Advise patients to dry the skin thoroughly after washing, if possible with a hair dryer on cool setting. Avoid tight clothing and try to ventilate the area as much as sociably possible.
• Even with careful attention, secondary infection of genital dermatoses may occur. Treatment with a combined anti-infective and steroidal preparation should be considered.
• Although creams are often easier to apply to the genital epithelium, they may sting a little more than ointments.
• Soap, bubble-bath, shower gel, and feminine washes should be avoided. Many women find aqueous cream or emulsifying ointment useful as soap substitutes for cleansing. Applying cold cream from the refrigerator can be particularly soothing.
• Vulval biopsy may be required to accurately diagnose skin dermatoses. The application of lignocaine/prilocaine cream prior to injecting local anesthetic makes this a painless and generally well-tolerated procedure.
• All painful vulval conditions have the potential to cause a secondary vaginismus that can often persist after the original complaint has settled. This will require appropriate treatment and follow-up. (see also Chapter 22)
• Vulval disease is often chronic and inevitably affects relationships and leads to a degree of psychological morbidity. Psychological support is therefore an important part of the management of these patients and should be considered along with treatment aimed at the physical component of the condition.
• The diagnosis and management of vulval disease can be difficult and may, in some cases, require the assistance of a clinician with a specific interest in the vulva. Many hospitals now run "vulva clinics" where specialists in GU medicine, dermatology and gynecology offer a combined opinion. This is the ideal approach to managing vulval disease.