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Vulval pain
GENITAL PAIN SYNDROMES
Glossary
Vulval pain
• Vulvodynia – vulval pain • Vulval dysaesthesia – chronic vulval pain with no obvious cause • Generalized vulval dysaesthesia – constant vulval pain; formerly termed ‘dysaesthetic vulvodynia’ • Localized vulval dysaesthesia – vulval pain usually triggered by contact; formerly termed ‘vestibulodynia’
Anne Edwards
1
Vulval pain syndromes are now well recognized in the developed world. The International Society for the Study of Vulvovaginal Disease has recently proposed changes to the classification of vulvodynia (vulval pain) (Figure 1).1
Course of the disease and prognosis Vulval pain syndromes typically have a relapsing and remitting course. However, it is likely that most patients improve and/or fully recover over time. In one study, 50% of patients recovered within 12 months of diagnosis.2 Data on the long-term outcome are not available, but most clinicians have experience of a few patients with intractable disease for whom no treatments are effective. Some women recover and then present with a recurrence. In the author’s experience, if they have previously responded to treatment they are likely to do so again.
Epidemiology There are no good data on the prevalence of vulval pain syndromes, but these are not rare disorders. In a GUM department with a total of 4000 new female attendances per year and a weekly specialist vulval clinic, there are about 20–30 new cases of vulval pain each year. Even more cases are seen in specialist vulval clinics taking referrals from primary care, and many gynaecology patients presenting with superficial (introital) dyspareunia have ‘localized vulval dysaesthesia’. Vulval pain syndromes can occur at any age throughout adult life. The localized vulval dysaesthesia variant is reported more commonly in premenopausal women, and ‘dysaesthetic vulvodynia’ is seen more commonly in perimenopausal and postmenopausal women. The possibility, and the potential challenges, of cases presenting in childhood has been raised, though not yet formally reported in the literature. The diagnosis of a vulval pain syndrome is seldom made in developing countries and is usually confined to white women.
History and examination Typically, the history is long, sometimes years. Patients may have tried treatments without success, and may see many doctors before a correct diagnosis is made (Figure 3). It is important to remember that, in vestibulodynia, pain is localized to the vestibule and commonly presents as dyspareunia.
Features and classification of vulval pain syndromes In all forms, the vulva appears normal. Some patients present with features of both generalized vulval dysaesthesia and localized vulval dysaesthesia.
Pathogenesis The aetiology remains unclear, but this group of disorders is best regarded as an example of a complex regional pain syndrome. The pathogenesis is similar to that of reflex sympathetic dystrophy, in which persistent pain occurs in the absence of tissue injury. This primary chronic pain syndrome subsequently impacts on the patient’s ability to function sexually.
Generalized vulval dysaesthesia • Tends to occur in older perimenopausal and post-menopausal women • Chronic vulval discomfort – burning, stinging, irritation or a feeling of rawness • May be worse at the end of the day • Intercourse does not usually aggravate symptoms
Diagnosis Localized vulval dysaesthesia Vestibulodynia (most common variant in this age group) • Young, sexually active women, usually premenopausal • Superficial dyspareunia • Pain on insertion of tampon/riding bicycle • Pain may persist after intercourse • Pain free at other times Clitorodynia Others
Vulval pain syndrome is a diagnosis of exclusion based on a typical history, absence of vulval pathology on examination and, in localized vulval dysaesthesia, the presence of touch tenderness. The symptoms are summarized in Figure 2. Other vulval disorders may coexist with vulval pain syndrome.
Anne Edwards is Consultant/Honorary Senior Lecturer in Genitourinary Medicine at the Radcliffe Infirmary, Oxford, UK. She is a Council Member of the British Society for the Study of Vulval Disease. Conflicts of interest: none declared.
MEDICINE 33:10
2
88
© 2005 The Medicine Publishing Company Ltd
GENITAL PAIN SYNDROMES
with a blunt instrument causes development of a red weal) and neurological disease.
When to consider a diagnosis of vulval pain • Long history/repeated consultations with a range of doctors and others • Chronic/recurrent symptoms • No response/partial response to a range of therapies • Vulva appears normal/ mild erythema • Microbiological tests repeatedly negative – notably for clinically diagnosed candidiasis
Management The approach to management is the same for the different vulval pain syndromes. Most specialists favour a combined medical and psychosexual/psychological approach. A definite diagnosis can be therapeutic in many patients with a long history of uncertainty, as can the knowledge that other women suffer the same problem. Reassurance about the lack of any sinister cause and the tendency to spontaneous improvement with time is essential. Written information, including details of support organizations, should be provided at the first consultation.
3
The vulva usually looks normal. In the original description of vestibulodynia, erythema was cited as one of the characteristics and part of the diagnostic criteria. It is now recognized that this is not an inflammatory process; the erythema reported in most cases reflects a subjective impression and is probably not real. • In generalized vulval dysaesthesia, touching the affected area does not exacerbate symptoms. • In localized vulval dysaesthesia, patients are symptom-free until the vulva is touched with, for example, a cotton bud. Commonly, the vestibule is affected, but in some women the whole introitus is involved. The distribution may be asymmetrical.
Drug therapy Topical agents (e.g. moisturizing creams, corticosteroids) may be soothing, but seldom produce any long-term, sustained improvements. Some patients achieve temporary relief with topical local anaesthetic agents, which can be a useful adjunct. Low-dose tricyclic agents used for their analgesic, and possibly anxiolytic, effects may accelerate recovery and are the mainstay of treatment. Treatment is usually started with low-dose amitryptiline or impramine, 10 mg nocte increasing after 1 month to 25 mg nocte. Patients who are likely to respond do so at doses of 40–75 mg, though doses of up to 125 mg may be tried with occasional benefit. Those who respond should remain on a maintenance regimen for 3–6 months or longer, depending on individual wishes. After a period of stability, dose reduction can be tried. When a trial of therapy with a tricyclic agent is undertaken, compliance is improved if the patient is given a detailed explanation of what to expect (Figure 4). In patients who are intolerant of amitryptiline, other agents from the same family could be used. The fact that tricyclic drugs are not acting as antidepressants in vulval pain syndromes is highlighted by the observation that serotonin re-uptake inhibitors (SSRIs) appear to be ineffective in these conditions. Patients who are already taking SSRIs for depression can take low-dose tricyclics in addition. Some patients may prefer to gradually increase to an antidepressant dose of the tricyclic and discontinue the SSRI. Others can be maintained on both a low-dose tricyclic and an SSRI. In complicated cases, psychiatric advice should be sought.
Investigations There are no specific investigations to confirm vulval pain syndrome. Biopsy and/or screening for infection is indicated when the vulva looks abnormal. However, vulval pain and a specific skin disease may coexist. Formal neurological assessment may be useful in women with symptoms or signs supporting a neurological diagnosis.
Differential diagnosis A full history and thorough examination of the mouth, genitalia and skin should exclude other diagnoses, most of which are uncommon. These include recurrent hymenal/posterior fourchette tears (examine patients within 48 hours of intercourse if this is suspected – a magnifying instrument such as a colposcope may be useful), symptomatic dermographism (writing on the skin
Psychosexual and psychological management Most patients benefit from a combined approach in addition to the medical management described above. Psychosexual approaches – although pain is the primary symptom, most patients (particularly those with localized vulval dysaesthesia) experience impairment of sexual function. Commonly, this results in loss of desire and/or avoidance of sex. Vaginismus can occur in localized vulval dysaesthesia, which compounds the pain associated with sexual intercourse. Psychosexual approaches may help, including an explanation of how pain can be provoked during intercourse, and the effects of muscle contraction, which may worsen the pain. Some practical hints include: • use of lubrication (e.g. baby oil) • Kegel’s exercises • positional advice, showing the patient how penetration can be achieved with minimal discomfort.
Counselling for patients with vulval pain starting tricyclic therapy for analgesia • These drugs are not acting as antidepressants in vulval pain syndromes • Side-effects tend to resolve after 2–3 weeks of use (or an alternative tricyclic can be tried) • A therapeutic benefit may not be noticed for 3–4 weeks or even longer (most commonly, not until doses of 40 mg are reached) • These are not drugs of dependency
4
MEDICINE 33:10
89
© 2005 The Medicine Publishing Company Ltd
GENITAL PAIN SYNDROMES
Exercises for relaxing the pelvic floor musculature may speed recovery. In the USA and Europe, this latter approach has been formalized with the development of biofeedback and surface electromyography.3 If available, a physiotherapist with an interest in biofeedback may provide additional help. In chronic cases, involvement of the partner can be helpful. Partners may avoid initiating sexual contact, and this can reinforce the patient’s self-perceived feeling of unattractiveness, thereby reducing her libido. Psychological approaches – in some cases, particularly those with generalized vulval dysaesthesia, psychological assessment may help to provide an overview of the patient and assist her in developing strategies to cope with chronic pain.
In a few women, vulval pain remains an intractable problem for which there is no effective treatment.
Non-responders Management of non-responders is difficult. There are anecdotal reports suggesting that gabapentin (as an analgesic) may be beneficial. Some authorities have advocated surgical resection of the vestibule in intractable localized vulval dysaesthesia, but this is an invasive procedure, there is no good evidence to support its efficacy, and many patients subsequently relapse. In the late 1980s, superficial laser vulvectomy was tried for vulval dysesthesia, but this has since been abandoned because it was ineffective and associated with complications. Further options are limited; they include: • referral for advice from a chronic pain clinic • alternative therapies such as acupuncture • strong reassurance – the limited data available suggest that most patients improve over time.
REFERENCES 1 International Society for the Study of Vulvovaginal Disease. The terminology and classification of vulvodynia: past, present and future. www.vulvodynia.com.au 2 Peckham B M, Maki D G, Patterson J J et al. Focal vulvitis: a characteristic syndrome and cause of dyspareunia. Am J Obstet Gynecol 1986; 154: 855–64. 3 Jantos M. The role of biofeedback in the management of vulvodynia. Application of surface electromyography in chronic vulvar pain. www.vulvodynia.com.au/articles/001.html FURTHER READING Edward L, ed. Genital dermatology atlas. London: Lippincot, Williams & Wilkins, 2004. Edwards A, Wojnarowska F. The vulval pain syndromes. Int J STD AIDS 1998; 9: 74–7.
MEDICINE 33:10
Follow-up Patients probably benefit from follow-up in a specialist unit. Some start treatment immediately; others prefer to consider the diagnosis, obtain information and access the Internet. An initial follow-up appointment at 4–6 weeks allows discussion of questions that may have arisen. In patients who have started therapy, it is useful to review possible side-effects and consider further dose increases. When a tricyclic agent has not been tolerated, it is usually possible to find an alternative. Once patients are stabilized on therapy, follow-up may be extended to every 3–6 months.
90
© 2005 The Medicine Publishing Company Ltd
Glossary
Vulval pain
• Vulvodynia – vulval pain • Vulval dysaesthesia – chronic vulval pain with no obvious cause • Generalized vulval dysaesthesia – constant vulval pain; formerly termed ‘dysaesthetic vulvodynia’ • Localized vulval dysaesthesia – vulval pain usually triggered by contact; formerly termed ‘vestibulodynia’
Anne Edwards
1
Vulval pain syndromes are now well recognized in the developed world. The International Society for the Study of Vulvovaginal Disease has recently proposed changes to the classification of vulvodynia (vulval pain) (Figure 1).1
Course of the disease and prognosis Vulval pain syndromes typically have a relapsing and remitting course. However, it is likely that most patients improve and/or fully recover over time. In one study, 50% of patients recovered within 12 months of diagnosis.2 Data on the long-term outcome are not available, but most clinicians have experience of a few patients with intractable disease for whom no treatments are effective. Some women recover and then present with a recurrence. In the author’s experience, if they have previously responded to treatment they are likely to do so again.
Epidemiology There are no good data on the prevalence of vulval pain syndromes, but these are not rare disorders. In a GUM department with a total of 4000 new female attendances per year and a weekly specialist vulval clinic, there are about 20–30 new cases of vulval pain each year. Even more cases are seen in specialist vulval clinics taking referrals from primary care, and many gynaecology patients presenting with superficial (introital) dyspareunia have ‘localized vulval dysaesthesia’. Vulval pain syndromes can occur at any age throughout adult life. The localized vulval dysaesthesia variant is reported more commonly in premenopausal women, and ‘dysaesthetic vulvodynia’ is seen more commonly in perimenopausal and postmenopausal women. The possibility, and the potential challenges, of cases presenting in childhood has been raised, though not yet formally reported in the literature. The diagnosis of a vulval pain syndrome is seldom made in developing countries and is usually confined to white women.
History and examination Typically, the history is long, sometimes years. Patients may have tried treatments without success, and may see many doctors before a correct diagnosis is made (Figure 3). It is important to remember that, in vestibulodynia, pain is localized to the vestibule and commonly presents as dyspareunia.
Features and classification of vulval pain syndromes In all forms, the vulva appears normal. Some patients present with features of both generalized vulval dysaesthesia and localized vulval dysaesthesia.
Pathogenesis The aetiology remains unclear, but this group of disorders is best regarded as an example of a complex regional pain syndrome. The pathogenesis is similar to that of reflex sympathetic dystrophy, in which persistent pain occurs in the absence of tissue injury. This primary chronic pain syndrome subsequently impacts on the patient’s ability to function sexually.
Generalized vulval dysaesthesia • Tends to occur in older perimenopausal and post-menopausal women • Chronic vulval discomfort – burning, stinging, irritation or a feeling of rawness • May be worse at the end of the day • Intercourse does not usually aggravate symptoms
Diagnosis Localized vulval dysaesthesia Vestibulodynia (most common variant in this age group) • Young, sexually active women, usually premenopausal • Superficial dyspareunia • Pain on insertion of tampon/riding bicycle • Pain may persist after intercourse • Pain free at other times Clitorodynia Others
Vulval pain syndrome is a diagnosis of exclusion based on a typical history, absence of vulval pathology on examination and, in localized vulval dysaesthesia, the presence of touch tenderness. The symptoms are summarized in Figure 2. Other vulval disorders may coexist with vulval pain syndrome.
Anne Edwards is Consultant/Honorary Senior Lecturer in Genitourinary Medicine at the Radcliffe Infirmary, Oxford, UK. She is a Council Member of the British Society for the Study of Vulval Disease. Conflicts of interest: none declared.
MEDICINE 33:10
2
88
© 2005 The Medicine Publishing Company Ltd
GENITAL PAIN SYNDROMES
with a blunt instrument causes development of a red weal) and neurological disease.
When to consider a diagnosis of vulval pain • Long history/repeated consultations with a range of doctors and others • Chronic/recurrent symptoms • No response/partial response to a range of therapies • Vulva appears normal/ mild erythema • Microbiological tests repeatedly negative – notably for clinically diagnosed candidiasis
Management The approach to management is the same for the different vulval pain syndromes. Most specialists favour a combined medical and psychosexual/psychological approach. A definite diagnosis can be therapeutic in many patients with a long history of uncertainty, as can the knowledge that other women suffer the same problem. Reassurance about the lack of any sinister cause and the tendency to spontaneous improvement with time is essential. Written information, including details of support organizations, should be provided at the first consultation.
3
The vulva usually looks normal. In the original description of vestibulodynia, erythema was cited as one of the characteristics and part of the diagnostic criteria. It is now recognized that this is not an inflammatory process; the erythema reported in most cases reflects a subjective impression and is probably not real. • In generalized vulval dysaesthesia, touching the affected area does not exacerbate symptoms. • In localized vulval dysaesthesia, patients are symptom-free until the vulva is touched with, for example, a cotton bud. Commonly, the vestibule is affected, but in some women the whole introitus is involved. The distribution may be asymmetrical.
Drug therapy Topical agents (e.g. moisturizing creams, corticosteroids) may be soothing, but seldom produce any long-term, sustained improvements. Some patients achieve temporary relief with topical local anaesthetic agents, which can be a useful adjunct. Low-dose tricyclic agents used for their analgesic, and possibly anxiolytic, effects may accelerate recovery and are the mainstay of treatment. Treatment is usually started with low-dose amitryptiline or impramine, 10 mg nocte increasing after 1 month to 25 mg nocte. Patients who are likely to respond do so at doses of 40–75 mg, though doses of up to 125 mg may be tried with occasional benefit. Those who respond should remain on a maintenance regimen for 3–6 months or longer, depending on individual wishes. After a period of stability, dose reduction can be tried. When a trial of therapy with a tricyclic agent is undertaken, compliance is improved if the patient is given a detailed explanation of what to expect (Figure 4). In patients who are intolerant of amitryptiline, other agents from the same family could be used. The fact that tricyclic drugs are not acting as antidepressants in vulval pain syndromes is highlighted by the observation that serotonin re-uptake inhibitors (SSRIs) appear to be ineffective in these conditions. Patients who are already taking SSRIs for depression can take low-dose tricyclics in addition. Some patients may prefer to gradually increase to an antidepressant dose of the tricyclic and discontinue the SSRI. Others can be maintained on both a low-dose tricyclic and an SSRI. In complicated cases, psychiatric advice should be sought.
Investigations There are no specific investigations to confirm vulval pain syndrome. Biopsy and/or screening for infection is indicated when the vulva looks abnormal. However, vulval pain and a specific skin disease may coexist. Formal neurological assessment may be useful in women with symptoms or signs supporting a neurological diagnosis.
Differential diagnosis A full history and thorough examination of the mouth, genitalia and skin should exclude other diagnoses, most of which are uncommon. These include recurrent hymenal/posterior fourchette tears (examine patients within 48 hours of intercourse if this is suspected – a magnifying instrument such as a colposcope may be useful), symptomatic dermographism (writing on the skin
Psychosexual and psychological management Most patients benefit from a combined approach in addition to the medical management described above. Psychosexual approaches – although pain is the primary symptom, most patients (particularly those with localized vulval dysaesthesia) experience impairment of sexual function. Commonly, this results in loss of desire and/or avoidance of sex. Vaginismus can occur in localized vulval dysaesthesia, which compounds the pain associated with sexual intercourse. Psychosexual approaches may help, including an explanation of how pain can be provoked during intercourse, and the effects of muscle contraction, which may worsen the pain. Some practical hints include: • use of lubrication (e.g. baby oil) • Kegel’s exercises • positional advice, showing the patient how penetration can be achieved with minimal discomfort.
Counselling for patients with vulval pain starting tricyclic therapy for analgesia • These drugs are not acting as antidepressants in vulval pain syndromes • Side-effects tend to resolve after 2–3 weeks of use (or an alternative tricyclic can be tried) • A therapeutic benefit may not be noticed for 3–4 weeks or even longer (most commonly, not until doses of 40 mg are reached) • These are not drugs of dependency
4
MEDICINE 33:10
89
© 2005 The Medicine Publishing Company Ltd
GENITAL PAIN SYNDROMES
Exercises for relaxing the pelvic floor musculature may speed recovery. In the USA and Europe, this latter approach has been formalized with the development of biofeedback and surface electromyography.3 If available, a physiotherapist with an interest in biofeedback may provide additional help. In chronic cases, involvement of the partner can be helpful. Partners may avoid initiating sexual contact, and this can reinforce the patient’s self-perceived feeling of unattractiveness, thereby reducing her libido. Psychological approaches – in some cases, particularly those with generalized vulval dysaesthesia, psychological assessment may help to provide an overview of the patient and assist her in developing strategies to cope with chronic pain.
In a few women, vulval pain remains an intractable problem for which there is no effective treatment.
Non-responders Management of non-responders is difficult. There are anecdotal reports suggesting that gabapentin (as an analgesic) may be beneficial. Some authorities have advocated surgical resection of the vestibule in intractable localized vulval dysaesthesia, but this is an invasive procedure, there is no good evidence to support its efficacy, and many patients subsequently relapse. In the late 1980s, superficial laser vulvectomy was tried for vulval dysesthesia, but this has since been abandoned because it was ineffective and associated with complications. Further options are limited; they include: • referral for advice from a chronic pain clinic • alternative therapies such as acupuncture • strong reassurance – the limited data available suggest that most patients improve over time.
REFERENCES 1 International Society for the Study of Vulvovaginal Disease. The terminology and classification of vulvodynia: past, present and future. www.vulvodynia.com.au 2 Peckham B M, Maki D G, Patterson J J et al. Focal vulvitis: a characteristic syndrome and cause of dyspareunia. Am J Obstet Gynecol 1986; 154: 855–64. 3 Jantos M. The role of biofeedback in the management of vulvodynia. Application of surface electromyography in chronic vulvar pain. www.vulvodynia.com.au/articles/001.html FURTHER READING Edward L, ed. Genital dermatology atlas. London: Lippincot, Williams & Wilkins, 2004. Edwards A, Wojnarowska F. The vulval pain syndromes. Int J STD AIDS 1998; 9: 74–7.
MEDICINE 33:10
Follow-up Patients probably benefit from follow-up in a specialist unit. Some start treatment immediately; others prefer to consider the diagnosis, obtain information and access the Internet. An initial follow-up appointment at 4–6 weeks allows discussion of questions that may have arisen. In patients who have started therapy, it is useful to review possible side-effects and consider further dose increases. When a tricyclic agent has not been tolerated, it is usually possible to find an alternative. Once patients are stabilized on therapy, follow-up may be extended to every 3–6 months.
90
© 2005 The Medicine Publishing Company Ltd