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Vulval dermatoses
GENITAL SKIN CONDITIONS
Vulval dermatoses
What’s new?
Elizabeth K Derrick C
Sallie Neill C
Abstract Dermatological disorders in the vulva are common. They are often misdiagnosed and mistreated because of the patient’s delay in seeking help, or the doctor’s failure to examine the area and inadequate training in anogenital diseases. Knowledge of the anatomy and appearance of the normal vulva is essential in order to recognize any abnormality or disease. Common skin diseases (eczema and psoriasis) affect the vulva, but may look different because of the moist flexural site. A careful family history and the general examination of the rest of the patient’s skin, including the scalp and nails, may give clues as to the diagnosis. Treatment is similar to that used at other body sites. There are also skin diseases that preferentially affect the vulva (lichen sclerosus, lichen planus and vulval intraepithelial neoplasia). Women with complicated vulval skin disorders should be referred to a dermatologist or, preferably, a multidisciplinary vulval clinic.
C
Imiquimod, an immune response-modulating cream is proving effective in the treatment for VIN and extra-mammary Paget’s disease and offers a potential ‘cure’ in some patients Tacrolimus used short term is helpful in the treatment of oral gingival lichen planus; its use in hypertrophic ulcerated vulval lichen planus may be effective in some patients, though its long-term safety is unknown Less irritant formulations and products for flexural psoriasis (e.g. calcitriol, tacalcitol) are less irritant than the first vitamin D analogues and may improve tolerance at these sites
are distributed symmetrically, each papilla arising from its own solitary base. Vestibular papillae are considered a variant of normal and are the female equivalent of penile pearly papules; there is no association with any of the known types of human papillomavirus (HPV).1 They are normally asymptomatic, but may cause anxiety if brought to the patient’s attention, so reassurance is important.
Keywords Dermovate; lichen planus; lichen sclerosus; squamous cell carcinoma; tacrolimus; vulval dermatoses (psoriasis, eczema); vulval intraepithelial neoplasia (VIN); vulval papillomatosis; vulvo-vaginogingival syndrome
Sebaceous gland hyperplasia Sebaceous gland hyperplasia (Figure 3) may be seen on the labia minora. The sebaceous glands may be numerous and prominent, and are seen as yellow micropapules. These are best seen when the skin is stretched as shown in the picture. They are usually asymptomatic, but can be associated with a mild itch. A similar appearance may be seen on the lateral buccal mucosa and the lips. No treatment is necessary.
The normal vulva The normal vulva (Figure 1) varies in appearance, particularly in the size of the glans clitoris and labia minora. The outer mons pubis and labia majora are covered with hair-bearing skin, but the interlabial sulci and the labia minora are modified in that the pilosebaceous units lack hair and the sebaceous glands are prominent. The vestibule (the innermost part of the vulva) is a mucosal surface and lacks any part of the pilosebaceous unit. Hart’s line, a line of demarcation between the skin and the mucosal surface, can be seen. The vaginal and urethral openings open into the vestibule as do the minor (paraurethral) and major (Bartholin’s) vestibular glands.
Eczema Eczema affecting the vulval skin (Figure 4) is usually seborrhoeic in type. The main symptom is itching and the clinical signs are variable. There is often ill-defined erythema with mild scaling on
Vestibular papillae Vestibular papillae (Figure 2) are soft micropapules that may be rounded or filiform. They are located in the vestibule and are frequently most prominent in the posterior aspect. The papillae
Elizabeth K Derrick MBBS MRCGP FRCP is a Consultant Dermatologist at Brighton and Sussex University Hospitals Trust, UK. She qualified in London and has trained and worked as a GP before specializing in Dermatology. Her interests include vulval disease, skin cancer and medical management. Competing interests: none declared. Sallie Neill MBChB FRCP is a Consultant Dermatologist at St John’s Institute of Dermatology, St Thomas’ Hospital, London, UK and specializes in vulval disorders. Competing interests: none declared.
MEDICINE 38:6
Figure 1 Normal vulva.
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GENITAL SKIN CONDITIONS
Figure 2 Vestibular papillae.
the outer labia majora but occasionally the erythema can be more intense with superficial erosions. Lichenification and excoriation develop secondary to scratching. Other sites such as the scalp, eyebrows, face and flexures may be involved. Treatment includes avoidance of irritants (e.g. perfumes, soaps), washing with a soap substitute and using a mild to moderately potent topical corticosteroid ointment (1e2.5% hydrocortisone ointment). Contact dermatitis to topical medicaments may rarely develop and patch-testing is then required. Corticosteroid ointments should be used carefully on the anogenital skin e a ‘finger tip’ amount applied only once in 24 hours is sufficient. Courses of treatment should be intermittent on an as-required basis, a 30-g tube lasting at least 3e6 months.
Figure 4 Eczema.
and natal cleft. The typical silvery scale is absent on occluded sites but can be seen on the outer sites such as the labia majora and mons pubis. Psoriasis may be asymptomatic but usually there is itching, soreness and dyspareunia. Treatment includes washing with a bland emollient and the intermittent use of a moderate to potent topical corticosteroid (betamethasone valerate 0.1% Betnovate, clobetasol propionate 0.05% Dermovate ointments). Secondary infection may complicate psoriasis, and the addition of an antibacterial or anticandidal component may help (e.g. betamethasone valerate 0.1% and fusidic acid 2% Fucibet cream, betamethasone dipropionate 0.05% and clotrimazole Lotriderm cream). The newer topical calcineurin inhibitors tacrolimus and pimecrolimus may be useful, but their irritant effect often proves intolerable for the patient.
Psoriasis Psoriasis (Figure 5) is a common dermatosis and it is useful to ask if there is a family history of this skin disease. Vulval involvement may occur either with generalized disease or in isolation. Flexural psoriasis is the most frequent type seen in the anogenital area, presenting with well-demarcated, red patches involving any part of the vulva and extending to the perianal skin
Perianal psoriasis Perianal psoriasis (Figure 6) is characterized by well-defined erythema surrounding the anus with extension into the natal cleft
Figure 3 Sebaceous gland hyperplasia.
MEDICINE 38:6
Figure 5 Psoriasis on the mons pubis.
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GENITAL SKIN CONDITIONS
over the clitoris and shrinkage or loss of the labia minora. Anteroposterior narrowing of the introitus rarely occurs but there is no vaginal involvement. Treatment involves use of a soap substitute and a topical ultrapotent corticosteroid (e.g. clobetasol propionate 0.05% Dermovate).5 The regimen recommended by the authors is application nightly for 1 month, followed by alternate nights for 1 month and then twice weekly for 1 month. The patient should then be reviewed to assess response. Maintenance treatment is then applied as required. Patients must be reassured that this regimen is safe, but that 30 g of clobetasol propionate 0.05% Dermovate must last at least 3 months. There is no role for topical hormonal preparations (testosterone, progesterone or oestrogens), and lower-strength corticosteroids are not as effective. The topical calcineurin inhibitors, tacrolimus and pimecrolimus, have been used in the rare cases where there is a poor response to a topical ultrapotent corticosteroid. If these are used it must be with caution for a short time only in view of the question of their immunosuppressing properties and the risk of neoplastic change. They also tend to be poorly tolerated because of their irritant effect. Surgical removal of lichen sclerosus is inappropriate in uncomplicated disease. Lichen sclerosus is associated with other auto-immune diseases such as thyroid disorders, maturity-onset diabetes, vitiligo and alopecia areata.
Figure 6 Perianal psoriasis. Erythema with fissuring.
often with deep fissuring. Treatment is as for vulval psoriasis. A topical barrier cream (e.g. zinc oxide or titanium dioxide preparations Vasogen, Metanium) may be helpful. Look for involvement of other sites, including the scalp, nails, elbows, knees as well as other flexural sites. The differential diagnosis of flexural psoriasis includes eczema, a drug eruption, lichen planus, erythrasma, tinea infections and the rarer conditions, Haileye Hailey disease (benign familial pemphigus) and extra-mammary Paget’s disease (intraepithelial adenocarcinoma). Skin swabs for microbiological assessment, skin scrapings to exclude fungi and punch biopsy for histological evaluation may be required to make the diagnosis.
Squamous cell carcinoma on lichen sclerosus There is a well-recognized but uncommon risk of developing squamous cell carcinoma on lichen sclerosus (Figure 8), it is estimated to be less than 4%. Patients must be advised to seek immediate help if they notice any persistent eroded or red area, wart, lump or ulcer.
Lichen sclerosus Lichen sclerosus (Figure 7) is an auto-immune dermatosis with a predilection for the anogenital skin and is estimated to affect 1/1000 women. It can present at any age, with peaks in prepubertal children and post-menopausal women.2e4 Severe and persistent itching is the classical presentation, and is often misdiagnosed and mistreated as thrush. Lichen sclerosus is characterized by whitening of the skin with ‘cigarette paper’ epidermal atrophy and scarring. Ecchymosis is a distinctive finding. The scarring leads to changes in architecture, with fusion of the hood
Lichen sclerosus in prepubertal girls Lichen sclerosus can affect prepubertal girls, and often also involves the perianal area, giving a ‘figure-of-eight’ appearance (Figure 9). Ecchymoses are occasionally mistaken for sexual abuse. Constipation and perianal itching misdiagnosed as ‘worms’ are common presenting complaints. Treatment is the same as in adults.
Figure 7 Lichen sclerosus.
MEDICINE 38:6
Figure 8 Squamous cell carcinoma.
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GENITAL SKIN CONDITIONS
Lichen planus Lichen planus (Figure 10) is a mucocutaneous disorder (i.e. it affects both skin and mucosae) that may appear in various forms (papulosquamous, erosive, pigmented and hypertrophic) on the vulva.6 The disease is usually limited to the genital and oral mucosae, but is sometimes associated with more generalized, papular lichen planus in other typical sites (the volar aspects of the wrists, the scalp and the nails). The perianal area is often involved. Itching is the main symptom but erosive disease causes pain, dyspareunia and vaginal discharge. The treatment is similar to lichen sclerosus, requiring an ultrapotent topical corticosteroid (e.g. clobetasol propionate 0.05% Dermovate). Vaginal involvement requires hydrocortisone foams. The topical calcineurin inhibitors tacrolimus and pimecrolimus may be used in refractory cases with the same cautionary note as advised for the treatment of lichen sclerosus. Secondary dysaesthesia, vulvodynia, may follow both lichen planus and lichen sclerosus, and should be recognized and treated appropriately. It is important to ascertain if there are any problems with swallowing as oesophageal lichen planus does occur in patients with erosive genital disease particularly if there is oral involvement.
Figure 10 Lichen planus.
an asthma corticosteroid inhaler sprayed directly on to the involved sites. Tacrolimus ointment has been used with some success, but again the safety of long-term use is unknown. Severe oral disease should be referred to an oral medicine clinic as systemic treatment with steroid or immunosuppressant therapy may be required.
Oral lichen planus The typical appearance of lichen planus in the mouth (Figure 11) may accompany vulval lichen planus and helps confirm the diagnosis. The buccal mucosa may be covered with the lacy, white network of Wickham’s striae. The tongue, buccal sulci and hard palate may also be involved. The gingiva are typically involved in the erosive form of lichen planus (vulvo-vaginogingival lichen planus e see below). The gingivae have an intense red erythema with a glazed appearance. Treatment is not always necessary; but if there is erosive disease topical corticosteroids will improve the problem. Applications include a topical corticosteroid paste (Adcortyl in Orabase), or mouthwashes of betamethasone sodium phosphate 0.5 mg q.d.s, Betnesol. The Betnesol is dissolved in water, swished around the mouth and gums for 5 min and then spat out. An alternative method is using
Erosive vulvo-vagino-gingival lichen planus Erosive vulvo-vagino-gingival lichen planus (VVG, Figure 12) is an uncommon, distressing condition causing painful erosions of the mucosae of the mouth, vagina and vulva.7 Architectural destruction occurs in the vulva. Adhesions may form in the vagina, leading to narrowing, shortening or even total sealing. Patients present with pain, discharge and/or dyspareunia. Treatment is difficult, because the condition is often resistant to therapy. Very potent topical corticosteroids, including foams and pessaries, may help, but immunosuppressive therapy with oral corticosteroids or ciclosporin may be necessary. These patients should be managed in a specialized, multidisciplinary vulval clinic. Vaginal adhesions can be divided under anaesthesia, with immediate postoperative treatment with corticosteroids and dilators to avoid resealing. Reconstructive surgery may have to be considered when the vaginal stenosis is irreversible, but the
Figure 9 Lichen sclerosus in a prepubertal girl.
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Figure 11 Oral lichen planus.
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GENITAL SKIN CONDITIONS
Figure 14 Multifocal undifferentiated vulval intraepithelial neoplasia. Figure 12 Erosive vulvo-vagino-gingival lichen planus.
presenting complaint is itchy papules, plaques or nodules, which may vary in size and colour (white, red or pigmented). Any site in the anogenital area can be affected and there may be lesions in the vagina and anal canal. A biopsy is essential to confirm the diagnosis and exclude any early invasive disease. The risk of an invasive squamous cell carcinoma is generally small, but this depends on other host factors such as disease duration, patient’s age, smoking and immunosuppression. Vaginal and cervical examination, including cervical smear, must be included in the assessment of any patient with VIN and, if there is perianal disease, the anal canal must be included in the assessment. Treatment of extensive multifocal intraepithelial neoplasia of the anogenital area is difficult, because the surgery required may be mutilating and is not always curative. Laser therapy, cryosurgery, interferon and topical 5-fluorouracil cream have been used with limited benefit. The new topical local immune response modifiers (e.g. imiquimod) have been successful in some
results are disappointing. Differential diagnosis includes mucous membrane pemphigoid and pemphigus, and a biopsy for immunofluorescence studies may need to be done. In a small number of these patients the oesophagus, external auditory canal8 and lachrymal duct can be affected.9
Erosive gingivitis Erosive gingivitis (Figure 13) is part of VVG syndrome and can be very painful. Topical corticosteroid washes and pastes may help, as may tacrolimus ointment.
Vulval intraepithelial neoplasia (VIN) The terminology has changed recently to undifferentiated (usual type VIN) and differentiated type VIN. This new terminology replaces the older VIN 1e3 categories.10 Undifferentiated VIN (usual type) Undifferentiated VIN (usual type) is 2/3 to full-thickness atypia of the epithelium, It may be multifocal or unifocal (Figures 14 and 15). Multifocal VIN is usually a problem seen in younger women and is associated with human papilloma virus (HPV) infection; the oncogenic HPV types 16, 18, or 33, are the commonest types involved. Interestingly, there is not always a history of genital warts but up to 90% are smokers. The
Figure 13 Erosive gingivitis.
MEDICINE 38:6
Figure 15 Unifocal undifferentiated vulval intraepithelial neoplasia.
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GENITAL SKIN CONDITIONS
6 Lewis FM. Vulval lichen planus. Br J Dermatol 1998; 138: 569e75. 7 Pelisse M, Leibowitch M, Sedel D, Hewitt J. Un nouveau syndrome vulvovagino-gingival: lichen plan erosif plurimuqueux. Ann Dermatol Ve´ne´re´ol 1982; 109: 797e8. 8 Martin L, Moriniere S, Machet M-C. Bilateral conductive deafness related to erosive lichen planus. J Laryngol Otol 1998; 112: 365e6. 9 Setterfield J, Neill S, Shirlaw P, et al. The vulvovaginal gingival syndrome: a severe subgroup of lichen planus with characteristic clinical features and a novel association with the class II HLA DQB1*0201 allele. J Am Acad Dermatol 2006; 55: 98e113. 10 Sideri M, Jones RW, Wilkinson EJ, et al. Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 2005; 50: 807e10.
patients and may offer a potential ‘cure’. The current management includes surgery for small lesions and topical imiquimod. Large polypoid, indurated or ulcerated lesions should be removed as these may harbour areas of microinvasion. Longterm, frequent, close monitoring is required in these patients. Undifferentiated VIN is often unifocal (Figure 15), occurs in older women and does not seem to be associated with HPV infection. This condition is perhaps the equivalent of Bowen’s disease of the skin elsewhere, and probably carries a significant risk of development of invasive squamous cell carcinoma (SCC). The disease is localized, so simple excision is the treatment of choice. Undifferentiated VIN may occasionally arise on a background of lichen sclerosus and lichen planus and must always be excised as there is a high risk of invasive change. Differentiated VIN Differentiated VIN is defined as severe atypia confined to the lower third of the epithelium with normal differentiation above the abnormal area. It is rare and is usually seen in patients with lichen sclerosus. It carries the greatest risk of developing an SCC and surgical removal is essential. The lesions are clinically small, firm, punched-out ulcers or firm warty papules. A
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REFERENCES 1 Moyal-Barracco M, Leibowitch M, Orth G. Vestibular papillae of the vulva. Lack of evidence for human papillomavirus aetiology. Arch Dermatol 1990; 126: 1594e8. 2 Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995; 32: 393e416. 3 Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999; 353: 1777e83. 4 Powell J. Paediatric vulval disorders. J Obstet Gynaecol 2006; 26: 596e602. 5 Neill SM, Tatnall FM, Cox NH. Guidelines on the management of lichen sclerosus. Br J Dermatol 2002; 147: 640e9.
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Always examine the whole of the patient’s anogenital area Ask about a personal or family history of skin disease Look for clues at other sites on the patient, including scalp and nails Common rashes (e.g. eczema and psoriasis) may look different in flexural sites Excessive use of soap-based cleansers can cause irritant dermatitis in the anogenital skin, and exacerbate pre-existing dermatoses. Washing with non-soap cleansers (e.g. aqueous cream or emulsifying ointment) is preferable Do not treat for ‘thrush’ unless proven by microbiological swab. Common anticandidal creams can irritate vulval skin and possibly encourage the development of vulvodynia Reassure patients about the small risk of squamous cell carcinoma in lichen sclerosus and lichen planus and advise about self-monitoring Refer complex patients to a dermatologist, or multidisciplinary vulval clinic
Ó 2010 Elsevier Ltd. All rights reserved.
Vulval dermatoses
What’s new?
Elizabeth K Derrick C
Sallie Neill C
Abstract Dermatological disorders in the vulva are common. They are often misdiagnosed and mistreated because of the patient’s delay in seeking help, or the doctor’s failure to examine the area and inadequate training in anogenital diseases. Knowledge of the anatomy and appearance of the normal vulva is essential in order to recognize any abnormality or disease. Common skin diseases (eczema and psoriasis) affect the vulva, but may look different because of the moist flexural site. A careful family history and the general examination of the rest of the patient’s skin, including the scalp and nails, may give clues as to the diagnosis. Treatment is similar to that used at other body sites. There are also skin diseases that preferentially affect the vulva (lichen sclerosus, lichen planus and vulval intraepithelial neoplasia). Women with complicated vulval skin disorders should be referred to a dermatologist or, preferably, a multidisciplinary vulval clinic.
C
Imiquimod, an immune response-modulating cream is proving effective in the treatment for VIN and extra-mammary Paget’s disease and offers a potential ‘cure’ in some patients Tacrolimus used short term is helpful in the treatment of oral gingival lichen planus; its use in hypertrophic ulcerated vulval lichen planus may be effective in some patients, though its long-term safety is unknown Less irritant formulations and products for flexural psoriasis (e.g. calcitriol, tacalcitol) are less irritant than the first vitamin D analogues and may improve tolerance at these sites
are distributed symmetrically, each papilla arising from its own solitary base. Vestibular papillae are considered a variant of normal and are the female equivalent of penile pearly papules; there is no association with any of the known types of human papillomavirus (HPV).1 They are normally asymptomatic, but may cause anxiety if brought to the patient’s attention, so reassurance is important.
Keywords Dermovate; lichen planus; lichen sclerosus; squamous cell carcinoma; tacrolimus; vulval dermatoses (psoriasis, eczema); vulval intraepithelial neoplasia (VIN); vulval papillomatosis; vulvo-vaginogingival syndrome
Sebaceous gland hyperplasia Sebaceous gland hyperplasia (Figure 3) may be seen on the labia minora. The sebaceous glands may be numerous and prominent, and are seen as yellow micropapules. These are best seen when the skin is stretched as shown in the picture. They are usually asymptomatic, but can be associated with a mild itch. A similar appearance may be seen on the lateral buccal mucosa and the lips. No treatment is necessary.
The normal vulva The normal vulva (Figure 1) varies in appearance, particularly in the size of the glans clitoris and labia minora. The outer mons pubis and labia majora are covered with hair-bearing skin, but the interlabial sulci and the labia minora are modified in that the pilosebaceous units lack hair and the sebaceous glands are prominent. The vestibule (the innermost part of the vulva) is a mucosal surface and lacks any part of the pilosebaceous unit. Hart’s line, a line of demarcation between the skin and the mucosal surface, can be seen. The vaginal and urethral openings open into the vestibule as do the minor (paraurethral) and major (Bartholin’s) vestibular glands.
Eczema Eczema affecting the vulval skin (Figure 4) is usually seborrhoeic in type. The main symptom is itching and the clinical signs are variable. There is often ill-defined erythema with mild scaling on
Vestibular papillae Vestibular papillae (Figure 2) are soft micropapules that may be rounded or filiform. They are located in the vestibule and are frequently most prominent in the posterior aspect. The papillae
Elizabeth K Derrick MBBS MRCGP FRCP is a Consultant Dermatologist at Brighton and Sussex University Hospitals Trust, UK. She qualified in London and has trained and worked as a GP before specializing in Dermatology. Her interests include vulval disease, skin cancer and medical management. Competing interests: none declared. Sallie Neill MBChB FRCP is a Consultant Dermatologist at St John’s Institute of Dermatology, St Thomas’ Hospital, London, UK and specializes in vulval disorders. Competing interests: none declared.
MEDICINE 38:6
Figure 1 Normal vulva.
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GENITAL SKIN CONDITIONS
Figure 2 Vestibular papillae.
the outer labia majora but occasionally the erythema can be more intense with superficial erosions. Lichenification and excoriation develop secondary to scratching. Other sites such as the scalp, eyebrows, face and flexures may be involved. Treatment includes avoidance of irritants (e.g. perfumes, soaps), washing with a soap substitute and using a mild to moderately potent topical corticosteroid ointment (1e2.5% hydrocortisone ointment). Contact dermatitis to topical medicaments may rarely develop and patch-testing is then required. Corticosteroid ointments should be used carefully on the anogenital skin e a ‘finger tip’ amount applied only once in 24 hours is sufficient. Courses of treatment should be intermittent on an as-required basis, a 30-g tube lasting at least 3e6 months.
Figure 4 Eczema.
and natal cleft. The typical silvery scale is absent on occluded sites but can be seen on the outer sites such as the labia majora and mons pubis. Psoriasis may be asymptomatic but usually there is itching, soreness and dyspareunia. Treatment includes washing with a bland emollient and the intermittent use of a moderate to potent topical corticosteroid (betamethasone valerate 0.1% Betnovate, clobetasol propionate 0.05% Dermovate ointments). Secondary infection may complicate psoriasis, and the addition of an antibacterial or anticandidal component may help (e.g. betamethasone valerate 0.1% and fusidic acid 2% Fucibet cream, betamethasone dipropionate 0.05% and clotrimazole Lotriderm cream). The newer topical calcineurin inhibitors tacrolimus and pimecrolimus may be useful, but their irritant effect often proves intolerable for the patient.
Psoriasis Psoriasis (Figure 5) is a common dermatosis and it is useful to ask if there is a family history of this skin disease. Vulval involvement may occur either with generalized disease or in isolation. Flexural psoriasis is the most frequent type seen in the anogenital area, presenting with well-demarcated, red patches involving any part of the vulva and extending to the perianal skin
Perianal psoriasis Perianal psoriasis (Figure 6) is characterized by well-defined erythema surrounding the anus with extension into the natal cleft
Figure 3 Sebaceous gland hyperplasia.
MEDICINE 38:6
Figure 5 Psoriasis on the mons pubis.
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GENITAL SKIN CONDITIONS
over the clitoris and shrinkage or loss of the labia minora. Anteroposterior narrowing of the introitus rarely occurs but there is no vaginal involvement. Treatment involves use of a soap substitute and a topical ultrapotent corticosteroid (e.g. clobetasol propionate 0.05% Dermovate).5 The regimen recommended by the authors is application nightly for 1 month, followed by alternate nights for 1 month and then twice weekly for 1 month. The patient should then be reviewed to assess response. Maintenance treatment is then applied as required. Patients must be reassured that this regimen is safe, but that 30 g of clobetasol propionate 0.05% Dermovate must last at least 3 months. There is no role for topical hormonal preparations (testosterone, progesterone or oestrogens), and lower-strength corticosteroids are not as effective. The topical calcineurin inhibitors, tacrolimus and pimecrolimus, have been used in the rare cases where there is a poor response to a topical ultrapotent corticosteroid. If these are used it must be with caution for a short time only in view of the question of their immunosuppressing properties and the risk of neoplastic change. They also tend to be poorly tolerated because of their irritant effect. Surgical removal of lichen sclerosus is inappropriate in uncomplicated disease. Lichen sclerosus is associated with other auto-immune diseases such as thyroid disorders, maturity-onset diabetes, vitiligo and alopecia areata.
Figure 6 Perianal psoriasis. Erythema with fissuring.
often with deep fissuring. Treatment is as for vulval psoriasis. A topical barrier cream (e.g. zinc oxide or titanium dioxide preparations Vasogen, Metanium) may be helpful. Look for involvement of other sites, including the scalp, nails, elbows, knees as well as other flexural sites. The differential diagnosis of flexural psoriasis includes eczema, a drug eruption, lichen planus, erythrasma, tinea infections and the rarer conditions, Haileye Hailey disease (benign familial pemphigus) and extra-mammary Paget’s disease (intraepithelial adenocarcinoma). Skin swabs for microbiological assessment, skin scrapings to exclude fungi and punch biopsy for histological evaluation may be required to make the diagnosis.
Squamous cell carcinoma on lichen sclerosus There is a well-recognized but uncommon risk of developing squamous cell carcinoma on lichen sclerosus (Figure 8), it is estimated to be less than 4%. Patients must be advised to seek immediate help if they notice any persistent eroded or red area, wart, lump or ulcer.
Lichen sclerosus Lichen sclerosus (Figure 7) is an auto-immune dermatosis with a predilection for the anogenital skin and is estimated to affect 1/1000 women. It can present at any age, with peaks in prepubertal children and post-menopausal women.2e4 Severe and persistent itching is the classical presentation, and is often misdiagnosed and mistreated as thrush. Lichen sclerosus is characterized by whitening of the skin with ‘cigarette paper’ epidermal atrophy and scarring. Ecchymosis is a distinctive finding. The scarring leads to changes in architecture, with fusion of the hood
Lichen sclerosus in prepubertal girls Lichen sclerosus can affect prepubertal girls, and often also involves the perianal area, giving a ‘figure-of-eight’ appearance (Figure 9). Ecchymoses are occasionally mistaken for sexual abuse. Constipation and perianal itching misdiagnosed as ‘worms’ are common presenting complaints. Treatment is the same as in adults.
Figure 7 Lichen sclerosus.
MEDICINE 38:6
Figure 8 Squamous cell carcinoma.
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GENITAL SKIN CONDITIONS
Lichen planus Lichen planus (Figure 10) is a mucocutaneous disorder (i.e. it affects both skin and mucosae) that may appear in various forms (papulosquamous, erosive, pigmented and hypertrophic) on the vulva.6 The disease is usually limited to the genital and oral mucosae, but is sometimes associated with more generalized, papular lichen planus in other typical sites (the volar aspects of the wrists, the scalp and the nails). The perianal area is often involved. Itching is the main symptom but erosive disease causes pain, dyspareunia and vaginal discharge. The treatment is similar to lichen sclerosus, requiring an ultrapotent topical corticosteroid (e.g. clobetasol propionate 0.05% Dermovate). Vaginal involvement requires hydrocortisone foams. The topical calcineurin inhibitors tacrolimus and pimecrolimus may be used in refractory cases with the same cautionary note as advised for the treatment of lichen sclerosus. Secondary dysaesthesia, vulvodynia, may follow both lichen planus and lichen sclerosus, and should be recognized and treated appropriately. It is important to ascertain if there are any problems with swallowing as oesophageal lichen planus does occur in patients with erosive genital disease particularly if there is oral involvement.
Figure 10 Lichen planus.
an asthma corticosteroid inhaler sprayed directly on to the involved sites. Tacrolimus ointment has been used with some success, but again the safety of long-term use is unknown. Severe oral disease should be referred to an oral medicine clinic as systemic treatment with steroid or immunosuppressant therapy may be required.
Oral lichen planus The typical appearance of lichen planus in the mouth (Figure 11) may accompany vulval lichen planus and helps confirm the diagnosis. The buccal mucosa may be covered with the lacy, white network of Wickham’s striae. The tongue, buccal sulci and hard palate may also be involved. The gingiva are typically involved in the erosive form of lichen planus (vulvo-vaginogingival lichen planus e see below). The gingivae have an intense red erythema with a glazed appearance. Treatment is not always necessary; but if there is erosive disease topical corticosteroids will improve the problem. Applications include a topical corticosteroid paste (Adcortyl in Orabase), or mouthwashes of betamethasone sodium phosphate 0.5 mg q.d.s, Betnesol. The Betnesol is dissolved in water, swished around the mouth and gums for 5 min and then spat out. An alternative method is using
Erosive vulvo-vagino-gingival lichen planus Erosive vulvo-vagino-gingival lichen planus (VVG, Figure 12) is an uncommon, distressing condition causing painful erosions of the mucosae of the mouth, vagina and vulva.7 Architectural destruction occurs in the vulva. Adhesions may form in the vagina, leading to narrowing, shortening or even total sealing. Patients present with pain, discharge and/or dyspareunia. Treatment is difficult, because the condition is often resistant to therapy. Very potent topical corticosteroids, including foams and pessaries, may help, but immunosuppressive therapy with oral corticosteroids or ciclosporin may be necessary. These patients should be managed in a specialized, multidisciplinary vulval clinic. Vaginal adhesions can be divided under anaesthesia, with immediate postoperative treatment with corticosteroids and dilators to avoid resealing. Reconstructive surgery may have to be considered when the vaginal stenosis is irreversible, but the
Figure 9 Lichen sclerosus in a prepubertal girl.
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Figure 11 Oral lichen planus.
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Figure 14 Multifocal undifferentiated vulval intraepithelial neoplasia. Figure 12 Erosive vulvo-vagino-gingival lichen planus.
presenting complaint is itchy papules, plaques or nodules, which may vary in size and colour (white, red or pigmented). Any site in the anogenital area can be affected and there may be lesions in the vagina and anal canal. A biopsy is essential to confirm the diagnosis and exclude any early invasive disease. The risk of an invasive squamous cell carcinoma is generally small, but this depends on other host factors such as disease duration, patient’s age, smoking and immunosuppression. Vaginal and cervical examination, including cervical smear, must be included in the assessment of any patient with VIN and, if there is perianal disease, the anal canal must be included in the assessment. Treatment of extensive multifocal intraepithelial neoplasia of the anogenital area is difficult, because the surgery required may be mutilating and is not always curative. Laser therapy, cryosurgery, interferon and topical 5-fluorouracil cream have been used with limited benefit. The new topical local immune response modifiers (e.g. imiquimod) have been successful in some
results are disappointing. Differential diagnosis includes mucous membrane pemphigoid and pemphigus, and a biopsy for immunofluorescence studies may need to be done. In a small number of these patients the oesophagus, external auditory canal8 and lachrymal duct can be affected.9
Erosive gingivitis Erosive gingivitis (Figure 13) is part of VVG syndrome and can be very painful. Topical corticosteroid washes and pastes may help, as may tacrolimus ointment.
Vulval intraepithelial neoplasia (VIN) The terminology has changed recently to undifferentiated (usual type VIN) and differentiated type VIN. This new terminology replaces the older VIN 1e3 categories.10 Undifferentiated VIN (usual type) Undifferentiated VIN (usual type) is 2/3 to full-thickness atypia of the epithelium, It may be multifocal or unifocal (Figures 14 and 15). Multifocal VIN is usually a problem seen in younger women and is associated with human papilloma virus (HPV) infection; the oncogenic HPV types 16, 18, or 33, are the commonest types involved. Interestingly, there is not always a history of genital warts but up to 90% are smokers. The
Figure 13 Erosive gingivitis.
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Figure 15 Unifocal undifferentiated vulval intraepithelial neoplasia.
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6 Lewis FM. Vulval lichen planus. Br J Dermatol 1998; 138: 569e75. 7 Pelisse M, Leibowitch M, Sedel D, Hewitt J. Un nouveau syndrome vulvovagino-gingival: lichen plan erosif plurimuqueux. Ann Dermatol Ve´ne´re´ol 1982; 109: 797e8. 8 Martin L, Moriniere S, Machet M-C. Bilateral conductive deafness related to erosive lichen planus. J Laryngol Otol 1998; 112: 365e6. 9 Setterfield J, Neill S, Shirlaw P, et al. The vulvovaginal gingival syndrome: a severe subgroup of lichen planus with characteristic clinical features and a novel association with the class II HLA DQB1*0201 allele. J Am Acad Dermatol 2006; 55: 98e113. 10 Sideri M, Jones RW, Wilkinson EJ, et al. Squamous vulvar intraepithelial neoplasia: 2004 modified terminology, ISSVD Vulvar Oncology Subcommittee. J Reprod Med 2005; 50: 807e10.
patients and may offer a potential ‘cure’. The current management includes surgery for small lesions and topical imiquimod. Large polypoid, indurated or ulcerated lesions should be removed as these may harbour areas of microinvasion. Longterm, frequent, close monitoring is required in these patients. Undifferentiated VIN is often unifocal (Figure 15), occurs in older women and does not seem to be associated with HPV infection. This condition is perhaps the equivalent of Bowen’s disease of the skin elsewhere, and probably carries a significant risk of development of invasive squamous cell carcinoma (SCC). The disease is localized, so simple excision is the treatment of choice. Undifferentiated VIN may occasionally arise on a background of lichen sclerosus and lichen planus and must always be excised as there is a high risk of invasive change. Differentiated VIN Differentiated VIN is defined as severe atypia confined to the lower third of the epithelium with normal differentiation above the abnormal area. It is rare and is usually seen in patients with lichen sclerosus. It carries the greatest risk of developing an SCC and surgical removal is essential. The lesions are clinically small, firm, punched-out ulcers or firm warty papules. A
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REFERENCES 1 Moyal-Barracco M, Leibowitch M, Orth G. Vestibular papillae of the vulva. Lack of evidence for human papillomavirus aetiology. Arch Dermatol 1990; 126: 1594e8. 2 Meffert JJ, Davis BM, Grimwood RE. Lichen sclerosus. J Am Acad Dermatol 1995; 32: 393e416. 3 Powell JJ, Wojnarowska F. Lichen sclerosus. Lancet 1999; 353: 1777e83. 4 Powell J. Paediatric vulval disorders. J Obstet Gynaecol 2006; 26: 596e602. 5 Neill SM, Tatnall FM, Cox NH. Guidelines on the management of lichen sclerosus. Br J Dermatol 2002; 147: 640e9.
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Always examine the whole of the patient’s anogenital area Ask about a personal or family history of skin disease Look for clues at other sites on the patient, including scalp and nails Common rashes (e.g. eczema and psoriasis) may look different in flexural sites Excessive use of soap-based cleansers can cause irritant dermatitis in the anogenital skin, and exacerbate pre-existing dermatoses. Washing with non-soap cleansers (e.g. aqueous cream or emulsifying ointment) is preferable Do not treat for ‘thrush’ unless proven by microbiological swab. Common anticandidal creams can irritate vulval skin and possibly encourage the development of vulvodynia Reassure patients about the small risk of squamous cell carcinoma in lichen sclerosus and lichen planus and advise about self-monitoring Refer complex patients to a dermatologist, or multidisciplinary vulval clinic
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