Vulval Lichen Planus
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- Bu səhifədəki naviqasiya:
- General advice for delivery of vulval care
- Physical examination of the patient
- General advice for all vulval conditions
- VULVAL DERMATITIS (Eczema)
- Aetiology Atopic dermatitis
- Irritant contact dermatitis
- Allergic contact dermatitis
- Symptoms Pruritus Soreness Pain Signs
- Complications Secondary infection Development of lichen simplex chronicus Diagnosis
- VULVAL PSORIASIS Aetiology
- Symptoms Vulval itch, pain or burning sensation can be present. Signs
- Diagnosis
- Management General Advice
- Pregnancy and Breast-feeding
- LICHEN SIMPLEX CHRONICUS
- Diagnosis History taking
- Management Recommended regimens
- Treatment of superinfection
- Allergies to topical preparations
- Onward referral criteria
2016 European guideline for the management of vulval conditions Michael J Boffa Department of Dermatology, Sir Paul Boffa Hospital, Floriana VLT 1941, Malta Bram ter Harmsel Dept of Gynaecology, Roosevelt kliniek, Rooseveltstraat 67, 2321 CT Leiden, Netherlands Gudula Kirtschig University Hospital of Tübingen, Tübingen, Germany Fiona Lewis St John’s Institute of Dermatology, Guy’s and St Thomas’ Hospital, London and Frimley Health NHS Trust, UK Micheline Moyal-Barracco Department of Dermatology, Tarnier-Cochin Hospital, 75006 Paris, France George-Sorin Tiplica Dermatology 2, Colentina Clinical Hospital, “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania Willem I. van der Meijden Department of Dermatology, New Cross Hospital, Wolverhampton, UK Editor: Jackie Sherrard Department of Sexual Health, Churchill Hospital, Oxford, UK Scope This guideline covers the more common conditions affecting the vulva:
General advice for delivery of vulval care. Vulval conditions may present to a variety of clinicians including Dermatologists, Genitourinary Medicine Physicians Gynaecologists and Primary Care Physicians or General Practitioners (GP). Investigations and management span across this spectrum, so women with vulval conditions are best managed by a multidisciplinary approach, which includes clear referral pathways between disciplines or access to a specialist multidisciplinary vulval service. There should also be access to clinico-pathological services to allow discussion and review of histology results. Physical examination of the patient Informed consent is a pre-requisite for all examinations, investigations and treatments. Consent is particularly important for intimate examinations of the anogenital area, and a chaperone should be offered in all cases and this offer should be documented clearly in the patient records. The proposed examination should be adequately explained to the patient before they undress. All attempts should be made to maintain patients’ dignity, providing privacy to dress and undress, and keeping them covered as much as possible. Appropriate facilities and equipment for investigations should be available prior to commencing the examination. The room should be well lit, private and soundproofed, with a suitable examination couch of adjustable height [1]. Screening for sexually transmitted infections (STI) should be considered in all patients, depending on symptoms and risk factors. If the patient presents with vulval itch, particularly with increased discharge, vulvovaginal candidiasis should be excluded. If the symptoms are not relieved by anticandidal treatment, especially if cultures are negative for candida, then a full genital examination should be undertaken and other causes considered. Possible alternate diagnoses include lichen sclerosus, lichen planus, lichen simplex chronicus, psoriasis or a neoplastic condition (particularly HPV related vulvar intra epithelial neoplasia in young women). Sexual dysfunction should be considered in all patients, either as the cause of the symptoms or developed secondary to the symptoms, and assessed if appropriate.
Topical treatments
Sexual partners
Dermatitis (also named “eczema”) is an inflammatory reaction characterized histologically by spongiosis, variable acanthosis and a superficial dermal lymphohistiocytic inflammatory infiltrate. The main symptom is itch. Exogenous and endogenous factors can be involved in aetiology. There is a danger in labelling any erythematous pruritic condition as dermatitis or eczema. Therefore, it is best practice to use the specific diagnosis instead of using these terms, namely atopic dermatitis or irritant /allergic contact dermatitis [2]. Aetiology Atopic dermatitis – there is increasing evidence that this is due to a defect in the barrier function of the skin [3]. In many atopic individuals, the genital area is spared, but vulval lichen simplex chronicus may be a manifestation of atopic dermatitis, either as isolated vulval disease or in association with disease at other sites [4]. Irritant contact dermatitis – this is the commonest type of eczema to affect the vulva. The vulval epithelium is less efficient as a barrier than skin elsewhere [5] and is in contact with moisture, sweat and urine and prone to friction. Cleansers, fragrances, lubricants and many other topical preparations can exacerbate the symptoms. Irritant dermatitis is a particular problem in those with urinary incontinence. Allergic contact dermatitis – this is a type IV delayed hypersensitivity reaction, where the individual has developed an allergy to a product applied topically. These are commonly fragrances, antibiotics, local anaesthetics and components of some topical treatments. Seborrhoeic dermatitis - this is an inflammatory, desquamative dermatosis affecting the scalp, face and more rarely, the trunk. Seborrhoeic dermatitis and psoriasis may be associated and have similar pathology. Yeast organisms on the skin may have a role in the development of seborrhoeic dermatitis in predisposed individuals [6]. This form of dermatitis rarely affects the vulva and does not have specific features. In contrast to vulval psoriasis, no specific clinical features of seborrheic dermatitis are recognized on the vulva and there is debate about whether this condition exists on the vulva. Therefore this diagnosis should be made only after exclusion of all other causes of vulvar erythema. Symptoms
Signs
Diagnosis'>Complications
Diagnosis The diagnosis is usually clinical, based on the clinical history and physical signs. It is helpful to check the rest of the skin for other features of atopic or seborrhoeic dermatitis. The main differential diagnoses are:
Investigation A biopsy is rarely necessary but should be performed if there are atypical features or failure to respond to treatment (IV, C). Patch testing (III, B) is useful if an allergic contact dermatitis is suspected, but is not necessary for all types of dermatitis. Patch testing should be performed in a clinic competent in this investigation and interpretation of the results. In addition to the standard series of allergens, any patient with a suspected vulval allergic contact dermatitis should be tested to medicaments, preservatives and specific products used on the vulva, at the appropriate dilutions [7,8]. The relevance of the results must be assessed carefully, as a positive test does not necessarily mean that the tested product is responsible for the vulval rash. Conversely, a negative test cannot completely prove the innocence of a topically applied product. Mycological and bacteriological specimens will exclude candidiasis, tinea and bacterial infection. Management___General_Advice'>Management
Calcineurin inhibitors (IV, C) (topical tacrolimus and pimecrolimus) can be tried for resistant cases, but their use is limited by stinging on application [9]. Follow-up Follow-up is not required routinely, but patients who need to use a more potent topical steroid or who have problems in controlling their symptoms should be reviewed. Patient information Patients should be given an explanation of their condition and the potential for a recurrence of symptoms. Advice about hygiene practices that can exacerbate the problem can be linked with useful patient information websites.
Aetiology Psoriasis is an immune-mediated disease with genetic predisposition. Several trigger factors are described (some of them present in population at risk for STI) [10]: infections (e.g. streptococcal, HIV), smoking, alcohol excess, physical factors (e.g. tattoos, piercing) and medication (e.g. systemic steroids, beta-blockers, lithium, non-steroidal anti-inflammatory drugs). Clinical features Genital skin is affected in 29-46% of psoriatic patients [11]. Vulvar psoriasis can be present in patients with psoriasis vulgaris as localized disease (2-5% of cases [12]) or in a disseminated form (affecting also the extensor regions or the gluteal cleft). Vulvar involvement can occur in patients with inverse psoriasis (rare cases with involvement of the flexural folds and genitalia). Generalized pustular psoriasis (a rare form of exudative psoriasis) can start spreading form the genital area. Impetigo herpetiformis is a rare dermatosis of pregnancy with similar pustular lesions that develop from the intertriginous areas (including vulva). Typical onset is during the last trimester of pregnancy with rapid resolution in the postpartum period. Clinically and histologically, it is consistent with pustular psoriasis. Erythrodermic psoriasis, also an uncommon form of psoriasis that is covering more than 90% of body surface, can be present in the vulvar region. Symptoms
Signs
Associated disorders Psoriatic arthritis [14] occurs in 15% - 30% of patients with psoriasis vulgaris or exudative psoriasis. Cardiovascular disease, hypertension, malignancy, diabetes, metabolic syndrome, inflammatory bowel disease or autoimmune diseases can also be associated with psoriasis. Diagnosis The diagnosis can usually be made from the history combined with the physical examination. Investigation The histopathological examination is characteristic: parakeratosis, Munro micro-abscesses (neutrophils in the stratum corneum), absent granular cell layer, epidermal hyperplasia, frequent mitoses in the basal cell layer and dilated tortuous capillaries in the dermal papillae [15]. Investigations for possible associated inflammatory diseases should be considered. Joint radiographs can be relevant for psoriatic arthritis. Management General Advice The patient should avoid all known trigger factors including scented detergents, synthetic underwear and tight pants [13]. Topical treatment is indicated for localized vulvar psoriasis. In patients with disseminated or generalized lesions of psoriasis the systemic therapy is also effective for the genital lesions – this treatment is described in other guidelines [16]. Due to possible local adverse reactions (mainly irritant), it is recommended to avoid the use of anthralin, tazarotene and ultraviolet therapy when treating vulvar psoriasis (II, B) [13]. Recommended Regimens Treatment will reduce the thickness of the lesions, the degree of erythema and remove scales. All therapeutic possibilities should be presented in order to obtain a tailored therapy that is acceptable to the patient. If pubic hair is present, the vulvar lesions will be better treated with solutions, foams or gels [17]. In order to cover the whole genital area 0.5 fingertip-units should be sufficient [18].
Emollients are recommended to reduce local irritation induced by other topical treatment and to maintain the therapeutic results (I, B) [22,25]. Unlicensed treatments Topical calcineurin inhibitors (tacrolimus, pimecrolimus) are reported to be effective in vulvar psoriasis (III). Contact dermatitis and local infections (mycotic, viral) can be induced [21, 26]. Dapsone is reported to be effective in vulvar pustular psoriasis (100 mg/day, one month) in combination with topical treatment (IV) [27]. Pregnancy and Breast-feeding Emollients are considered safe during pregnancy and lactation [17]. Pregnant and breast-feeding mothers were excluded from above clinical studies involving topical corticosteroids and vitamin D analogues. There is no information on medication excretion in breast milk. Topical calcineurin inhibitors are not recommended in pregnancy and in breast-feeding mothers. Topical coal tar usage for short periods of time during pregnancy is considered to only have a small risk [17, 28]. Follow-up Active disease should be assessed as clinically required. Stable disease should be reviewed after 1-3 months.
Anogenital lichen simplex chronicus is a common condition. However, the incidence and prevalence have not been established properly. It is estimated to occur in approximately 0.5% of the Western European and American population. In vulvar clinics it may comprise 10-35% of patients seen. The condition usually develops in mid- to late-adult life [4]. Aetiology Anogenital lichen simplex chronicus is most often encountered in persons with an atopic diathesis: up to 75% of patients have a personal or immediate family history of atopy [4]
The condition is triggered by psychological distress and local environmental factors, such as heat, sweating, dryness of the skin, friction and harsh skincare products. Although probably rare, it may sometimes be worthwhile to consider neuropathic itch as a possible cause. This could be associated with sacral spinal compression, postherpetic neuralgia, and diabetic neuropathy [29]. The itch-scratch-itch cycle plays a pivotal role in maintaining chronicity of the condition. Symptoms
Signs
Complications
Diagnosis History taking
Clinical examination is usually sufficient to make a diagnosis. The presence of skin disease elsewhere may be helpful in establishing a differential diagnosis. Investigation
Management Recommended regimens
Alternative regimens
Follow-up
Lichen sclerosus (LS) is an inflammatory skin disease that involves the anogenital area more often than other cutaneous sites; typically it does not affect the vagina and very rarely involves the oral mucosa. It is mainly seen in adult women, but children may be affected. LS is probably underdiagnosed. In females the course is usually chronic, but should be diagnosed as soon as possible, as early treatment prevents scarring and possibly malignant change. Spontaneous remission is observed. Aetiology LS is an inflammatory dermatosis of unknown aetiology. A genetic predisposition is implicated. A positive family history is observed in about 10% of patients with vulval LS. An increased incidence of autoantibodies to the extracellular matrix protein 1 and autoantibodies to BP180 antigen are reported. Their significance is not known, but may support the idea of LS being a (humoral) autoimmune disease [35,36]. Oxidative DNA damage was detected throughout LS biopsies, indicating that oxidative damage to lipids, DNA and proteins may contribute to sclerosis, autoimmunity and carcinogenesis in LS. The possible role of TP53 mutations in the development of vulval cancer in LS is postulated. Symptoms
Signs
Complications
Diagnosis Characteristic clinical appearance. In typical cases a biopsy may not be needed, but many clinicians prefer to take a biopsy at presentation. A biopsy should be performed if the clinical diagnosis is uncertain, dysplasia / carcinoma is suspected or there is failure of first line treatment. Clinical and pathological correlation is essential. In early disease histology can be non-specific. Key histopathological features (biopsy has to be taken from a typical lesion) [38]:
Further investigations Investigation for autoimmune disease if clinically indicated, because some diseases (e.g. thyroid disease, pernicious anaemia, vitiligo, diabetes mellitus) are associated with LS in females (IV,C) [36]. These conditions may be asymptomatic. Skin swabs for bacterial, fungal or viral infection are only useful to exclude co-existing infection, if there are symptoms or signs suggestive of this. Patch testing: rarely required and only if secondary (medicament) allergy is suspected. The advice of a dermatologist should be sought. Management General advice Patients should be informed about the condition and given written information. Patients should be made aware of the small risk of neoplastic change, although this may be less in well controlled LS [39]. They should be advised to contact the doctor if they notice a change in appearance (e.g. lump, ulceration or hardening of skin), or if there is a major change in symptoms. LS needs to be treated. About 10% of patients have no itch, but will have clinical signs of LS and should also be treated (III, C). After initial treatment (usually 3 months) some patients will become asymptomatic with few remaining signs of LS, others may be left with irreversible scarring. There is debate amongst specialists about further treatment once symptoms and signs are suppressed by the initial treatment. This is because it can be difficult to decide whether there is still active disease. However, as it is known that LS may progress and lead to more scarring despite the lack of symptoms after initial treatment, some propose continuous, preventive treatment for many years in order to prevent progression (III, C). Lee et al. in a recent series, with a mean follow up of 5 years, showed that continuous treatment with individually chosen applications of a topical steroid will prevent symptoms, further scarring and carcinoma development in 58% vs. 93.3%, 40% vs. 3.4% and 0% vs. 4.7%, respectively [39]. Emollients may give symptom relief after initial steroid treatment (IIb B) [40].
Potent [41] or ultra-potent topical steroids [42] e.g. mometasone furoate or clobetasol proprionate are first line recommendations for genital LS (Ib, A). Recommended regimen Various regimens are used; one of the most common being daily use of potent to ultra-potent topical steroids (usually once daily) for three months. Others use the steroid daily for one month, then alternate days for one month, twice weekly for one month (this may be preferred in children to avoid skin atrophy) with review at 3 months. Twice daily application may occasionally be of additional benefit in resistant LS. Maintenance treatment Proactive maintenance therapy with twice-weekly application of mometasone furoate 0.1% ointment is effective and safe in maintaining remission, and may help to prevent malignant change (Ib, A) [39, 43]. 30g of an ultra-potent steroid should last at least 3 months. Treatment of superinfection An ultra-potent or potent topical steroid preparation combined with antibacterial and antifungal agents e.g. gentamycin or fucidic acid and nystatin or azole antifungals or an alternative preparation that combats secondary infection may be appropriate if secondary infection is a concern. These should only be used for a short period of time to clear infection (IV, C). Allergies to topical preparations Allergies to any compound (also steroids) of a topical preparation may occur after long-term use. In case of a waning effect of a previously good treatment allergy testing may be indicated. Alternative second line treatments Topical calcineurin inhibitors are not licensed for the treatment of LS. However, the efficacy of topical tacrolimus 0.1% has been demonstrated in the treatment of vulval LS [44] (Ib, A) and when used for 16 to 24 weeks in males and females with genital and extra genital LS [45] (IIb, B). Topical tacrolimus 0.03% ointment appears to be an effective treatment for children with anogenital LS and as maintenance treatment (twice a week), possibly reducing recurrences [46] (III, B). Comparing pimecrolimus 1% cream and clobetasol propionate 0.05% cream, both treatments showed improvement in pruritus and burning/pain after 12 weeks in vulval LS, but clobetasol was found to be superior in improving inflammation [47] (Ib, A). Another study of pimecrolimus showed that 42% of patients were in ‘complete remission’ after 6 months application [45] (IIb, B). Local irritation was the most common side effect with both tacrolimus and pimecrolimus but usually improved after the initial period of use [48, 49]. The long-term risks need to be studied in view of concerns about the possibility of topical immunosuppression increasing risk of malignancy [50]. Limited data from two small RCTs demonstrates efficacy of systemic retinoids in the treatment of genital LS [51-53]. Retinoids may be considered if standard therapy for LS has failed but should only be given by a dermatologist, experienced in the use of these agents. They are severely teratogenic and pregnancy must be avoided for 2 years after finishing treatment (Ib, A). Topical hormonal therapy (progesterone or testosterone preparations) failed to demonstrate efficacy in controlled trials (IIa, B) [42]. Phototherapy is effective in some LS patients. In vulval LS, UVA1 may be considered if topical corticosteroids have failed. However, the well documented development of carcinomas after PUVA and UVB gives cause of concern, in particular at the genital site [54,55] (Ib, A). Surgery in vulval LS should only be used for the treatment of coexistent VIN / SCC or fusion [56]. Disease tends to recur around the scar in females (III, B). Clitoral LS Lichen sclerosus may appear isolated at the clitoral hood, however, often other vulval parts are also affected. Early signs of clitoral LS are swelling of the prepuce; white plaques, fissures and scarring may follow. If the clitoral prepuce is affected by LS this may lead to fusion of the skin and a burrowed clitoris. Clitoral involvement should be searched for and treated like LS at other genital sites. As mechanical triggers are thought to be important in maintaining LS e.g. any tight clothing should be avoided. Topical preparations should be massaged in gently. Surgery, to treat fusion is only indicated in rare situations (e.g. severe problems with self-esteem, sexual function or urination). There is a chance of recurrence after surgery because the inflammatory process may not have ceased. Surgery should only be performed by an experienced surgeon and after careful counseling about the intervention, adverse effects and potential recurrences. Extragenital LS There are fewer studies for the treatment of extragenital LS. UVA1 phototherapy is a potential first-line treatment option [55,57] (Ib, A). Potent topical steroids and topical calcipotriol, possibly under occlusion, may be tried in extragenital LS [57] (III, B).
Onward referral criteria Those with active disease which has not responded adequately to treatment should be referred to a physician specialized in the condition. Any patient who develops differentiated or undifferentiated VIN or an SCC on a background of LS should be seen and followed up by an experienced specialist. Follow-up
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