Vulval Lichen Planus
Yüklə 238 Kb.
|
- Bu səhifədəki naviqasiya:
- Signs
- Diagnosis Characteristic clinical appearance
- Histology of vulval biopsy
- Management General Advice
- Treatment
- Pregnancy and Breast-feeding
- Symptoms
- Complications
- Management Information
- Vulvar care measures
- Multilevel anaesthetic nerve blocks
- Psychosexual interventions
- Vulvar Intraepithelial Neoplasia (VIN) Introduction
- Table 1. Evolution of the ISSVD terminology
- Bornstein et al (2016) [126]
LICHEN PLANUS Aetiology Lichen planus is an inflammatory disorder with manifestations in skin, hair, nails and genital and oral mucous membranes; more rarely it affects the lacrimal duct, oesophagus and external auditory meatus. It is an inflammatory condition of unknown pathogenesis, but is probably an immunological response by T-cells activated by, as yet, unidentified antigens. Weak circulating basement membrane zone antibodies have been demonstrated in 61% of 56 patients with biopsy-proven erosive lichen planus of the vulva but are of unknown significance [58]. In some cases there is overlap between lichen planus and lichen sclerosus [59]. Symptoms
Signs The anogenital lesions of lichen planus may be divided into three main groups according to their clinical presentation: 1. Classical Typical papules occur on the keratinised anogenital skin, with or without Wickham’s striae, on the inner aspect of the vulva. Hyperpigmentation frequently follows their resolution, particularly in those with dark skin. This type of lichen planus may be asymptomatic. Vulval lesions were found in 19 out of 37 women with cutaneous lichen planus, with four of the 19 having had no symptoms [60]. 2. Hypertrophic These lesions are relatively rare and can be difficult to diagnose. They particularly affect the perineum and perianal area, presenting as thickened warty plaques which may become ulcerated, infected and painful. The clinical appearance may mimic malignancy. They are not usually accompanied by vaginal lesions. 3. Erosive This the most common subtype to cause vulval symptoms. The mean age of onset of vulval symptoms in 114 women with erosive lichen planus was 56.9 years [61]. The mucosal surfaces are eroded. At the edges of the erosions the epithelium is red-to-purple coloured and a pale network of Wickham’s striae is sometimes seen. It is important to recognise vaginal involvement in erosive lichen planus and start treatment early, as it can lead to scarring and complete stenosis. The lesions consist of friable telangiectases with patchy erythema which are responsible for the common symptoms of dyspareunia, postcoital bleeding and a variable discharge, which is often serosanguinous. As erosions heal, synaechiae and scarring can develop [62]. This type is also seen in the oral mucosa although synechiae are uncommon. The term vulvo-vaginal gingival syndrome is used when erosive disease occurs in these three sites. The presenting symptoms are usually pain and soreness. Diagnostic criteria for vulval erosive lichen planus have been proposed in an international e-Delphi exercise [63]. It is suggested that at least three of the following criteria should be present to make the diagnosis: (i) well-demarcated erosions/erythematous areas at the vaginal introitus; (ii) presence of a hyperkeratotic border to lesions and/or Wickham striae in surrounding skin; (iii) symptoms of pain/burning; (iv) scarring/loss of normal architecture; (v) presence of vaginal inflammation; (vi) involvement of other mucosal surfaces; (vii) presence of a well-defined inflammatory band involving the dermo-epidermal junction; (viii) presence of an inflammatory band consisting predominantly of lymphocytes; and (ix) signs of basal layer degeneration. Complications
Diagnosis Characteristic clinical appearance: involvement of the vagina excludes lichen sclerosus. Skin changes elsewhere can be helpful, but overlap between lichen planus and lichen sclerosus is described. Immunobullous disorders such as cicatricial mucous membrane pemphigoid and pemphigus can clinically resemble erosive lichen planus. Dermoscopy: specific dermoscopic features that may aid clinical diagnosis include the presence of thick linear irregular vessels arranged diffusely throughout lesions, peripheral Wickham’s striae and an intense red background [65]. Histology of vulval biopsy: irregular saw-toothed acanthosis, increased granular layer, basal cell liquefaction and band-like dermal mainly lymphocytic infiltrate [66]. Further investigations
Whilst a link with hepatitis C and sometimes B has been noted in some (especially Mediterranean) countries, a UK study of 100 women with vulval mucosal lichen planus found no evidence of increased incidence and concluded that routine screening is unnecessary [68]. Nevertheless screening may still be prudent in populations with a high prevalence of viral hepatitis. Management General Advice Patients should be informed about the condition and given written information. Patients should be made aware of the small long-term risk of neoplastic change and advised to seek urgent medical advice if they notice a change in appearance or texture (e.g. lump, hardening of skin or persistent ulceration). Treatment There is no randomised controlled trial evidence to guide treatment of vulval erosive lichen planus [69]. Topical Treatment Recommended Regimen
Alternative Regimens
Systemic treatments There is no consensus and little evidence base for the use of systemic agents. In the vulvovaginal–gingival syndrome there is general agreement that azathioprine, dapsone, griseofulvin, chloroquine and minocycline, all tried empirically, are of little or no benefit. Cyclosporin may be considered.
The new biological agents have shown varying results. However the rising trend of TNF-α inhibitors inducing lichen planus-like eruptions including erosive oral and vulval disease [75] reserves these drugs for only the most recalcitrant cases. Basiliximab was reported to be effective in erosive oral lichen planus, although its use has not been evaluated in vulval disease [76]. All these potentially toxic therapies need careful monitoring and are best supervised by a dermatologist in the context of a specialised clinic (IV, C). Surgery Surgery may be necessary for management of symptomatic vulval and vaginal adhesions and scarring, but is contraindicated in patients with active, inflammatory disease [77]. In a study of 11 women with lichen planus scarring, surgical lysis of vulvo-vaginal adhesions allowed intercourse in 55% and decreased urination difficulties in 75%. Of the patients, 91% stated they were happy with the surgery and would recommend it to others. However, sexual difficulties may persist even after surgery [78]. Pregnancy and Breast-feeding
Onward referral Referral to a multidisciplinary vulval clinic is recommended for erosive disease, recalcitrant cases or those in whom systemic therapy is considered. Follow-up
According to the 2003 International Society for the Study of Vulvovaginal Diseases (ISSVD) terminology, vulvodynia is defined as ‘vulvar discomfort, most often described as burning pain, occurring in the absence of relevant visible findings or a specific, clinically identifiable, neurologic disorder’ [79]. Vulvodynia is also categorised by the ISSVD, as generalized or localized, provoked, unprovoked or mixed (both provoked and unprovoked) (see symptoms). Aetiology Vulvodynia is currently considered as a dysfunctional sensory processing in the central nervous system, involving both central and peripheral pain generators [80-82]. This mechanism is similarly observed in other painful conditions (fibromyalgia, interstitial cystitis/painful, bladder, irritable bowel, temporomandibular dysfunction) which have a significant association with vulvodynia [83,84]. A genetic predisposition to both vulvodynia and these other pain conditions is suspected [85]. Triggering or maintaining factors have been identified: candidiasis [86], psychological disturbances, either resulting from the chronic pain or preexisting to it [87-89], and pelvic floor muscle dysfunction [90]. Symptoms The mnemonic (memory aide) OPQRST-A can be used to describe and evaluate the vulvar discomfort. Onset Candidiasis is frequently an initiating event of vulvodynia (IIb) but any acute painful vulval, urinary or anal condition (e.g. infection, surgical procedure) may precede the occurrence of vulvodynia, especially if these physical events occur in a context of emotional stress (IV). Provocation The discomfort may be either provoked or unprovoked or mixed.
Quality Burning is the main symptom, but many other sensations are reported (e.g. tingling, stinging, rawness, irritation). When present, itch is not the predominant symptom. Region The discomfort may be either localised or generalised.
Severity The severity of the discomfort is highly variable, impacting both daily life (impossible to concentrate on normal activities) and sexual activity (painful sex leading to fear and avoidance, with consequences on the partner and relationship). Time Vulvodynia is a chronic pain condition having usually lasted months or years before the diagnosis is made. The intensity of the discomfort is often variable over time. Significant improvement or complete remission may occur, following treatment, or spontaneously (IIa) [91,92]. Associated symptoms Other pain conditions (mentioned before) may be associated, particularly interstitial cystitis/painful bladder [93] (level B). No sphincter disturbance occurs in vulvodynia. Signs Inspection of the vulva reveals no relevant physical findings. This means that the vulva has a normal appearance or that, if a lesion is found, this lesion cannot explain the discomfort (e.g. a wart cannot explain diffuse burning). In provoked vestibulodynia, tenderness is elicited by gentle application of a cotton wool tip on the vestibule. Neurological examination is normal (in particular, there is no perineal anaesthesia). Complications Impact on general well-being, particularly on psychosexual function and relationships [94]. Diagnosis It is a clinical diagnosis based on signs and symptoms. Differential diagnosis
Management Information Patients should be given a full explanation of their condition verbally, and then reinforced with written information. Do not cast doubt about the reality of the pain (not “in the head”) and acknowledge its significant impact on all aspects of the quality of life. Explain simply the current knowledge about mechanisms, contributing factors, treatment and prognosis. Treatment A multidisciplinary approach to patients with vulvodynia is widely recommended [95,96]. The levels of evidence are poor, however [97]. Delays in diagnosis and inappropriate treatments may have a negative prognostic impact. Vulvar care measures . Avoidance of irritating factors (IV, letter??) . Use of emollient soap substitute (IV, letter??).
Local anaesthetics, e.g. 5% lidocaine ointment or 2% lidocaine gel, are mainly prescribed in patients with introital dyspareunia resulting from provoked vestibulodynia. Lidocaine should be applied 15-20 minutes prior to penetrative sex and washed off just before penetration (IV, C). Long term daily use of lidocaine is commonly recommended in practice, although in one randomized controlled study, 12 week application of 5% lidocaïne 4 times a day was not more effective than placebo in reducing vestibular pain [98] (I, A). Others
Oral pain modifiers Mostly prescribed in case of unprovoked vulvodynia. Amitriptyline is a tricyclic antidepressant with analgesic properties. Low doses of amitriptyline (titration from 5 to 25 mg daily) are widely used, although one randomized study has not confirmed the beneficial effect of this treatment in vulvodynia [104] (Ib, A). Gabapentin, an anticonvulsant, is another treatment of neuropathic pain at an initial dose of 300 mg per day, with an increase up to 1200 mg [105] (III, B). There is little evidence for the use of pregabalin [106] (IIb, B). Multilevel anaesthetic nerve blocks (caudal, pudendal) [107,108] (III,C) Neuromodulation Transcutaneous Electrical Nerve stimulation (TENS) may be a self administered home protocol. [109,110] (Ib, B). Acupuncture [111] (Ib, B) Physical therapies Pelvic floor muscle dysfunction should be addressed in patients with vulvodynia, particularly when introital dyspareunia is present.
Psychosexual interventions Cognitive-behavioural therapy (CBT) [114,115] (III, C) is the mainstay approach and is superior to supportive psychotherapy [116] (Ib, A). As dyspareunia affects the sexual well-being of both the patient and her partner [117], couples’ CBT therapy seems a logical approach [118] (III, C). Mindfulness has also been used [119] (IV). Surgery Vestibulectomy (posterior or total; with or without vaginal advancement to cover the defect) is usually considered a “last resort”, after failure of all the available therapeutical options [120]. However the level of evidence is low [121] (III, C) and one study shows that, at long term follow-up, vestibulectomy is not more effective that CBT [122,123]. In addition, surgery may aggravate pain related to a dysfunction in pain processing. Follow-up
Introduction VIN is a chronic vulvar skin disorder characterized by dysplastic changes of the squamous epithelium. VIN is a premalignant lesion, although spontaneous regression has been reported [124]. In the last 100 years premalignant lesions of the vulva have been described, but there always was a debate about the clinical and pathological characteristics of these lesions. The terminology has changed several times since the first description of VIN in 1922: “ dyskeratose erythroplasiforme de la muqueuse vulvaire” [125]. The International Society for the Study of Vulvovaginal Disease (ISSVD) had been leading in the process of chosing new terminology for premalignant vulvar lesions. The last version of the terminology was accepted by the ISSVD in 2015 (Table 1):
Table 1. Evolution of the ISSVD terminology
Yüklə 238 Kb. Dostları ilə paylaş:
|