Common vulvar disorders
Dr Ross Pagano is a generalist obstetrician and gynaecologist who has a special interest in benign vulvar disorders. He is the gynaecologist in charge of the Vulvar Disorders Clinic and the DES Follow-up Clinic at The Royal Women’s Hospital, Melbourne, and he is president of the Australian and New Zealand Vulvovaginal Society. Dr Pagano also has a long-standing interest in colposcopy and laser treatment and is vice president of the Australian Society for Colposcopy and Cervical Pathology.
This article describes the common benign vulvar conditions presenting to a tertiary referral clinic at The Royal Women’s Hospital, Melbourne. An update on the aetiology and management of these disorders is presented. Herpes infection of the genital tract is not described, as patients with this problem are screened and managed so well by GPs that they very rarely present to a vulvar disorders clinic.
Terminology There have recently been significant changes in terminology adopted by the International Society for the Study of Vulvovaginal Disease (ISSVD). The term ‘dystrophy’ has been eliminated and the new classification lists specific disorders, e.g. lichen sclerosus, lichen planus, lichen simplex chronicus, etc. The term ‘vulvar dermatosis’ encompasses any non-malignant disorder of the skin or mucous membranes of the vulva and is not in itself meant to be a diagnosis. The terms hyper- or hypoplastic dystrophy and atypia are no longer used. The term ‘vulvodynia’ is described as vulvar discomfort, pain, irritation, burning or rawness in the absence of visible findings (except erythema) or a clinically identifiable neurological disorder. Thus the new classification of vulvar pain is divided into two main groups: A. Vulvar pain related to a specific disorder 1) Infectious, e.g. candidiasis, herpes 2) Inflammatory, e.g. lichen planus, Behcet’s syndrome 3) Neoplastic, e.g. Paget’s disease, carcinoma 4) Neurological, e.g. herpes neuralgia, spinal nerve compression, etc
B. Vulvodynia 1) Generalised a. Provoked b. Unprovoked c. Mixed (provoked and unprovoked) 2) Localised (vestibulodynia, clitorodynia, hemivulvo-dynia, etc) a. Provoked b. Unprovoked c. Mixed. Thus the condition formerly referred to as ‘vulvar vestibulitis syndrome’ causing entry dyspareunia would now be called ‘provoked vestibulodynia’. The extreme form of the condition, where vestibular pain is present continuously, would be called ‘unprovoked vestibulodynia’. Similarly, chronic unrelenting pain involving the whole of the vulva is ‘unprovoked generalised vulvodynia’, etc. Vulvar intraepithelial neoplasia (VIN) replaces such terms as Bowen’s disease, hyperplastic dystrophy with atypia, carcinoma in situ, etc. VIN is no longer classified into categories 1, 2 and 3 because VIN 1 is poorly reproducible and generally represents HPV effect only. Thus all VIN is high grade by definition. The new classification of VIN is: 1. VIN usual type – this refers to VIN of HPV origin (warty, basaloid or mixed type) 2. VIN differentiated – this refers to VIN of non-HPV origin and is usually associated with lichen sclerosus 3. VIN unclassified.
Symptoms The most common presenting symptoms of vulvar disorders are pruritus, burning, pain, and entry dyspareunia. Pruritus is either focal or diffuse; chronic or episodic; may or may not be related to the menstrual cycle; and can be confined mostly to the hair-bearing areas or mostly affecting the labia minora and introital areas. A careful history can be invaluable in determining the most likely diagnosis.
Lichen sclerosus and lichen simplex chronicus
Lichen sclerosus Lichen sclerosus (LS) is the most common condition seen in a vulvar disorders clinic and usually presents with chronic pruritus vulvae. It usually affects older women (postmenopausal), although it is important to realise that it can occur at any age, even in children and infants. In young women, it is often misdiagnosed as candida infection, as the flare-ups of pruritus often resolve spontaneously and this may correspond in timing to anti-candida self-medication. Candidiasis is very rare in children and in postmenopausal women not on hormone replacement therapy, i.e. low oestrogenic states. Thus always suspect LS when pruritus vulvae occurs in these age groups and in young women labelled as suffering from chronic candida infection. Ignoring the condition in children and young women can lead to significant vulvar scarring and permanent introital stenosis. LS is now regarded as an autoimmune disorder that is familial and primarily affects the vulva, although it can affect other areas of the body. The epidermis is usually thin, hence the old term ‘et atrophicus’. This term is no longer used, as the epidermis is not atrophic and has a high cell turnover. However, the skin can look ‘atrophic’ and this should not stop you from using topical corticosteroids. The dermis is thickened and acellular due to deposition of collagen and this results in the white appearance of the skin and the resultant fibrosis and anatomical distortion, including labial resorption, burying of the clitoris and stricture of the introitus. Overall there is diffuse depigmentation of the vulva, however, in areas of chronic scratching, foci of pigmentation are often seen (dermal pigment incontinence). Management consists of avoidance of local irritants and the application of a high-potency corticosteroid preparation. This should be used frequently until the symptoms are brought under control then used on a prophylactic basis to prevent further flare-ups. Long-term treatment is required and prophylactic rather than prn use of corticosteroids will result in less scarring and anatomical destruction. Diprosone OV is currently the most potent topical corticosteroid preparation available in Australia, and once the initial flare-up is controlled, a less potent application such as Diprosone, Advantan, etc can be substituted. Ointments are preferred if there is skin splitting, as creams often cause stinging. If introital stenosis or chronic skin splitting of the fourchette occurs, this can only be relieved surgically. LS rarely affects the vagina, so any enlargement procedure to the introitus must involve advancement of the posterior vaginal wall to cover the vulvar defect created in order to achieve a permanent benefit. The overall risk of progression of LS to invasive cancer is 5%, although this may just be a result of long-term scratching and trauma. With aggressive use of topical corticosteroids to eliminate pruritus, this risk should be reduced. However, these patients require long-term follow-up for the development of VIN (differentiated). These lesions usually present as areas of unresponsive focal pruritus and hyper-keratosis, thus any such change requires biopsy. Occasionally, LS fails to respond to topical steroids, and systemic steroids have to be used. Topical pimecrolimus (Elidel) cream, a local immunosuppressant, is now being used with some success. Finally, most cases of ‘atrophic vulvitis’ represent vulvar irritation associated with atrophic vaginitis. If correction of the vaginitis by intravaginal oestrogen does not result in relief of the vulvar symptoms, topical oestrogen cream to the vulva will be of little benefit. Always suspect LS as the underlying cause of the vulvitis and ‘atrophic’ appearance.
Lichen simplex chronicus Lichen simplex chronicus (LSC) of the vulva is the second most common form of dermatitis seen in a vulvar disorders clinic. It usually occurs as a result of chronic irritation or stress and tends to affect the labia majora primarily. It is usually diffuse, although can be focal thus requiring biopsy. Lichenification (exaggeration of the skin creases due to dermal oedema) is usually evident clinically. The condition usually responds to removal of irritant factors (soaps, pads, etc) and responds well to short courses of topical high-potency corticosteroids. The main differential diagnosis is Paget’s disease, and biopsy is mandatory if there is failure to respond to topical steroids.
Recurrent vulvovaginal candidiasis (RVVC) is defined as four or more candida infections in a 12-month period. In practical terms, these patients usually feel they are never free of symptoms. With time, the vaginal discharge is minimal and symptoms are predominantly confined to the vulva. These are usually pruritus, burning, irritation and occasionally entry dyspareunia, particularly due to skin splitting. The symptoms are cyclical in nature and are often worse premenstrually and following intercourse, and frequently disappear during menses. RVVC usually occurs following prolonged courses of antibiotics and as a result of stress. Physical findings are minimal, with a brick-red appearance of the vulva and evidence of chronic skin splitting often seen. Diagnosis can be made on history, vulvar skin scrapings (on Sabouraud’s medium) and biopsy. Vaginal cultures are often negative. The most important differential diagnosis is vulvar dermatitis (atopic eczema, contact dermatitis, lichen sclerosus, lichen simplex chronicus, etc). Treatment of individual episodes with topical anti-candida preparations is usually ineffective long-term. Management usually involves oral antifungal agents (ketoconazole 200 mg daily or fluconazole 150 mg weekly) for up to six months, with regular monitoring of liver function with baseline LFTs taken prior to treatment, after the first two weeks of therapy and then monthly. Recurrence is common, and this can be reduced by careful attention to diet (low sugar, alcohol and yeast), as well as ceasing the oral contraceptive pill and using Depo-Provera or Implanon. Repeated courses of intra-vaginal boric acid capsules (600 mg daily for 14 days) have proven useful, especially in refractory cases due to Torulopsis glabrata.
Vulvar intraepithelial neoplasia
Because of the significant risk of development of cancer, vulvar intraepithelial neoplasia (VIN) is the most important benign condition of the vulva to diagnose and manage correctly. It usually presents as areas of focal pruritus not responding to topical steroids or anti-candida medication. Lesions may be solitary or multifocal. Each lesion has three characteristics: it is usually raised, has a discreet margin and has an irregular surface contour. These features are easily recognised on colposcopy of the vulva, and a biopsy is required to confirm and classify the lesion. It is nearly always symptomatic, with pruritus being the most common symptom, and the lesion is pigmented in approximately 10% of cases. Note that in 20% of cases, a coexistent CIN lesion is present so colpo-scopy of the whole lower genital tract should be performed. Under the new classification, ‘VIN usual type’ refers to VIN associated with HPV infection and smoking, and the risk of progression to malignancy is low. This is particularly the case in women younger than 30 years. The warty and basaloid descriptions relate purely to the clinical appearance. On the other hand, ‘VIN differentiated’ is usually found in association with dermatitis (especially lichen sclerosus), is usually found in older women (although can occur at any age) and has a very high risk of progression to invasive cancer. ‘VIN unclassified’ refers to lesions that cannot be assigned pathologically to either category and includes VIN of the pagetoid type (Paget’s disease). Cure is best achieved by local excision. Laser (CO2) can be used if excision is difficult (e.g. periclitoral or perianal) or if lesions are multiple. Topical imiquimod (Aldara) cream has been used with some success in young women with multiple basaloid lesions. Whatever the treatment, long-term follow-up for at least 10 years is necessary as the risk of recurrence overall is 20 per cent.
Pruritus vulvae Thus, in summary, always beware of focal pruritus, especially if it hasn’t responded to topical cortisone, as it is often due to VIN. Generalised pruritus vulvae is not always due to candidiasis, and with anti-candida self-medication now available, many women are ignoring the possibility of other pathology. From our point of view, one should never prescribe medication for vulvar symptoms without looking first, as this is the reason why lichen sclerosus is often missed for so long.
Lichen planus (LP) is less common than lichen sclerosus and usually presents with vulvar pain and ulceration. It often affects the vagina (as well as the vulva) and can be a cause of chronic vaginal discharge not responding to antifungal and antibiotic treatment. It can be associated with LP of the mouth and often responds poorly to topical steroids. If the condition is confined to the vagina, the resultant discharge may be helped with the long-term application of hydrocortisone cream or prednisolone suppositories per vaginam (PV), although this condition usually requires systemic corticosteroids. Pimecrolimus cream PV can be helpful, although systemic azathioprine is required in refractory cases.
Vulvar ulceration and pigmentation
ulceration Apart from herpes infection, the most common ulcer to occur on the vulva is an aphthous ulcer. They are usually painful and respond well to high-potency topical steroid cream. Occasionally, they can become chronic and require biopsy. Usually, in this situation, surgical excision is required. Ulcers due to malignancy need to be excluded, as well as painless ulcers due to syphilis. Behcet’s syndrome presents with painful deep ulcers associated with mouth ulcers and iritis, often with a rash on the extremities, and represents a generalised vasculitis. Systemic prednisolone is required and management should be in conjunction with a physician, as cyclosporin is often required. In cases of refractory ulceration, thalidomide orally has been extremely effective. Pigmentation The most common cause of multiple areas of pigmentation of the vulva is melanosis vulvae. This is a benign condition, and provided the diagnosis has been confirmed on biopsy, the risk of developing melanoma is extremely small. Melanomas do occur on the vulva. The vulva represents only 1% of total body surface area, yet 7% of all melanomas in women occur on the vulva. Always ask about a family history of melanoma, and all pigmented lesions need to be followed for changes in pigmentation, internal borders, etc. Remember that 10% of VIN lesions are pigmented and pigmentation is commonly seen in chronic lichen sclerosus and is caused by chronic scratching.
Excluding deep dyspareunia due to endometriosis, pelvic inflammatory disease, etc, gynaecologists are being confronted by more and more women presenting with entry dyspareunia. Often these women are rendered apareunic, and when examined, there is an area of extreme hypersensitivity confined to the vulvar vestibule. The vestibule is that part of the vulva extending from the hymen to the medial third of the labia laterally, the clitoris anteriorly and the fourchette posteriorly. Showing this focal hypersensitivity with Q tip pressure to the patient and her partner (e.g. by TV monitor) is helpful in explaining the condition to them and emphasising that the condition is ‘physical’ and not purely psychosexual in nature. Certainly these patients have significant secondary vaginismus and eventually they will develop psychosexual problems if the condition is not recognised and treated. Nevertheless, patients with primary psychosexual problems will also present with dyspareunia, and a careful history and examination are mandatory. Patients with primary psychosexual problems often have extreme vaginismus, and the vulvar tenderness is diffuse and not just confined to the vestibule. Where the tenderness is confined to the vestibule, there is often associated focal erythema, the cause of which is unknown. The condition was known as ‘vulvar vestibulitis syndrome’ (VVS) and is still called this in the media and on the Internet. The new term is ‘provokedvestibulodynia’. It represents a hyperaesthesia of the vestibule, and the current theory is that these women have a higher concentration of nerve endings (type C fibres) in the vestibule. This may explain the racial differences, as it is seen less often in darker-skinned races. It can follow sexual trauma to the vestibule and is often seen in association with chronic candidiasis. Management consists of careful explanation to the patient, excluding candidiasis and treating it effectively if present, encouraging the liberal use of an oil-based lubricant during attempted intercourse (e.g. olive oil) and addressing any psychosexual issues. Once candidiasis is eliminated, low-dose amitriptyline therapy is helpful in 60% of women, and the course is for at least six months. The starting dose is 10 mg nocte building up to a maximum of 100 mg. The next line of therapy is electromyographic biofeedback, a technique that teaches the woman to relax her pelvic floor muscles, which in turn results in a reduction in peripheral nerve sensitivity via a dorsal horn reflex. If conservative measures fail, a surgical vestibulectomy can be used effectively. Here the tender area of the vestibule (together with the hymen remnant) is excised, and the posterior wall of the vagina is advanced to cover the defect. In selected cases, long-term results are most encouraging.
REFERENCES Books Dennerstein GJ, Scurry J, Brenan J, Allen D, Marin G. The Vulva and Vaginal Manual. Melbourne: Gynederm Publishing; 2005. Kaufman R, Faro S, Brown D. Benign Diseases of the Vulva and Vagina. 5th ed. Elsevier Mosby; 2005. Neill SM, Ridley CM. The Vulva. 2nd ed. Blackwell Science; 1999. Edwards L (guest editor). Therapy of Genital Diseases. Dermatologic Therapy 2004 Mar: 17(1). Edwards L, editor. Genital Dermatology Atlas. Philadelphia, Pa: Lippincott Williams & Wilkins; 2004. Lessana-Leibowitch M. An Atlas of Vulvar Pathology. Janssen Pharmaceuticals.
Journal articles Turner ML, Marinoff SC. General principles in the diagnosis and treatment of vulvar diseases. Dermatol Clin 1992 Apr:10(2); 275-81. Smith Y, Haefner H. Vulvar lichen sclerosus: pathophysiology and treatment. Am J Clin Dermatol 2004; 5(2):105-25. Virgili A, Bacilieri S, Corraza M. Managing vulvar lichen simplex chronicus. J Reprod Med 2001 Apr;46(4):343-6. Jones R. Vulvar intraepithelial neoplasia: current perpectives. Eur J Gynaecol Oncol 2001;22(6):393-402. Edwards L. Subsets of vulvodynia: overlapping characteristics. J Reprod Med 2004; 49(11):883-7. Haefner HK, Collins ME, Davis GD, Edwards L, Foster DC, Hartmann ED, et al. The vulvodynia guideline. J Low Gen Tract Dis 2005;9(1):40-51. Pagano R. Vulvar Vestibulitis Syndrome: an often unrecognised cause of dyspareunia. Aust NZ J Obstet Gynaecol 1999;39(1):79-83.
- Generalised pruritus vulvae is not always due to candidiasis and with anti-candida self-medication available, many women are ignoring the possibility of other pathology.
- Candidiasis is very rare in children and in postmenopausal women not on hormone replacement therapy.
- Because of the significant risk of development of cancer, vulvar intraepithelial neoplasia (VIN) is the most important benign condition of the vulva to diagnose and manage correctly.
- Always beware of focal pruritus especially if it hasn't responded to topical cortisone as it is often due to VIN.
- Ignoring lichen sclerosus in children and young women can lead to significant vulvar scarring and permanent introital stenosis.
- Patients with lichen sclerosus require long-term follow-up for the development of VIN (differentiated).
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