Abstract
The authors report a case of vulvar lichen planus–induced vulvovaginal stenosis along with a review of the current literature. The authors outline a case of a patient with biopsy-proven vulvar lichen planus who subsequently developed a vulvovaginal stenosis. Treatment was initiated with clobetasol ointment, oral prednisone, later transitioned to oral methotrexate and clobetasol, and then switched to acitretin. Collaboration with the patient’s family physician and the hypertension clinic has been sought to remove medications associated with lichenoid reactions from the patient’s regimen. Review of literature was conducted through Ovid MEDLINE. Only six cases of vulvar lichen planus–induced vulvovaginal stenosis had been found, suggesting the relative rarity of this severe disease presentation. The patient has achieved control with her current regimen, as well as some clinical improvement of the resulting vaginal stenosis. Vulvovaginal stenosis can be induced by vulvar lichen planus, and its management requires a multimodal and multidisciplinary approach.
Introduction
Vulvar lichen planus (VLP) is a disease of the middle-aged female population with an association to oral manifestations, where 19%–67% of women affected by oral lesions have concomitant vulvovaginal presentations. 1
The most common manifestation, erosive atrophy, can progress to loss of the labia minora and clitoral hood, desquamative vaginitis, and vaginal stenosis.1,2 Oral lichen planus (LP) may also present with desquamative gingivitis, and vulvovaginal gingival (VVG) syndrome is desquamative gingivitis associating with vulval and vaginal desquamation.3,4
To our knowledge, 23 reports have been published regarding LP-induced vulvovaginal stenosis,2,5–13 most recently in 2013 by Singh et al. 9 who commented on the rarity of vulvovaginal stenosis progressing from isolated VLP, a separation from VVG syndrome. 9
A search via Ovid MEDLINE identified 23 patients with LP-induced vulvovaginal stenosis. From these patients, six were determined to exclude gingival involvement, suggesting the rarity of isolated VLP-induced vulvovaginal stenosis (Table 1). The authors outline a case of a patient with biopsy-proven VLP who subsequently developed a vulvovaginal stenosis. Verbal and written patient consent were obtained for publication.
Summary of case reports describing LP-induced vulvovaginal stenosis.
LP: lichen planus; LS: lichen sclerosus.
Case report
A 56-year-old female patient presented with a 16-month history of vulvar irritation with itching and pain, as well as vaginal burning. She presented with dyspareunia, which limited her once regular sexual activity. Six months prior to presentation to clinic, intercourse had become too painful, and vaginal penetration could not be attained.
Her medical history included hypertension, gastroesophageal reflux disease, arthritis, two vaginal births, a tubal ligation, and a hysterectomy for abnormal uterine bleeding. She was post-menopausal and was initially started on oral Premarin, later switched to a transdermal patch and topical estrogen added by gynecology. Her other medications included diclofenac/misoprostol, perindopril, hydrochlorothiazide, pantoprazole, candesartan, and atenolol.
Her initial gynecologist for dyspareunia noted narrowing and shortness of the vagina, primarily at the vaginal vault, attributed to atrophic menopausal change and previous hysterectomy. She subsequently saw a dermatologist who biopsied the mucosal lesions and diagnosed LP. The condition worsened and was unresponsive to local therapy, precipitating referral to the multidisciplinary vulvar clinic with a dermatologist and gynecologist present concurrently. When seen at our clinic, a speculum or even a small finger could not be inserted due to a complete blockage just inside the introitus. No pelvic mass was palpated on examination. There were large erosions on the inner aspects of the labia and the introital area. In the oral mucosa, superficial erosions with Wickham striae characteristic of LP were noted on the lateral aspect of the tongue.
Vulvar biopsies showed a lichenoid reaction consistent with LP. As eosinophils were present, a lichenoid drug eruption was considered. Hepatitis C serology was negative.
Vulvar erosions (Figure 1) improved with oral prednisone 30 mg daily, topical clobetasol ointment twice daily, and triamcinolone acetonide paste was also prescribed for the oral erosions. The patient was then tapered off prednisone and started on oral methotrexate (5–20 mg/week). Methotrexate in combination with continued topical corticosteroid led to further re-epithelialization, and the use of a vaginal dilator was suggested for the introital opening. However, after over 3 years of therapy with methotrexate which improved symptoms of pain, burning, itching, and vulvar erosions, the patient’s disease became recalcitrant to methotrexate, and she was switched to acitretin. It is noted that the patient remained on atenolol and hydrochlorothiazide as per her cardiologist during this time.
Erythema with an erosion in the inferior vaginal introitus as well as vaginal stenosis.
During this period, a brief trial of topical tacrolimus was attempted but discontinued due to pruritus and irritation. At the 3-year follow-up, acitretin (25 mg/day) was started. The patient achieved some symptom control of her vulvar pain and some re-epithelialization as she continued along with two additional tapering courses of prednisone and clobetasol ointment.
The patient’s vulvar LP remitted, and her vaginal stenosis improved, after a switch of her anti-hypertensive medicine to amiloride, but unfortunately recurred when metoprolol was prescribed for her atrial fibrillation. Metoprolol is a medication associated with LP. This flare was treated with a tapering course of prednisone, clobetasol ointment on the affected vulvar areas, and 25 mg hydrocortisone suppository. Simultaneously, the patient was advised to continue calcium and vitamin D supplements, and a bone densitometry was obtained for chronic steroid use. The patient initially declined the use of vaginal dilators and now uses these irregularly. The patient continues to decline surgical release for penetrative intercourse and is content with her current clinical presentation.
Discussion
VLP-induced vulvovaginal stenosis is a debilitating condition that significantly affects a patient’s quality of life and requires a multidisciplinary approach to treatment. The pathogenesis of LP has not been conclusively proven. An autoimmune etiology is likely, involving basal keratinocyte damage by T-cells. 1 There have been speculations regarding the role of genetics through HLA-DR1 typing studies. 14 Past publications have indicated associations with hepatitis C and systemic medications, including glimepiride, carbamazepine, methyldopa, and diuretics such as hydrochlorothiazide, non-steroidal anti-inflammatory drugs (NSAIDs), angiotensin-converting enzyme (ACE) inhibitors, and beta-blockers.1,15–18
There are limited published data on therapies for this condition; current available regimens do not have large-scale studies to prove efficacy and despite treatment, recurrence rates remain high. 19 Initial treatment of VLP consists of ultrapotent topical corticosteroids such as clobetasol propionate and betamethasone dipropionate, with addition of calcineurin inhibitors, systemic corticosteroids, methotrexate, and retinoids with increased severity.1,4,10 Although topical tacrolimus applications have been reported to show favorable response in resolution of the VLP in many patients,1,4,6,10,11,19 monotherapy could be ineffective, or even adversely affecting some patients.1,6 Unfortunately, the treatment course of recalcitrant disease can be complicated and may require multiple modalities of treatment to resolve symptoms and signs of active disease. 19 Systemic immunosuppressions, such as prednisone, azathioprine, methotrexate, mycophenolate mofetil, cyclosporine, adalimumab, and tildrakizumab, were reported to be more effective in combating VLP in combination with topical corticosteroids or tacrolimus.4,5,6,9,20–22 Other drugs, such as cyclosporine, were also used but with less success and more adverse effect on patients.5,6 The efficacy of using oral prednisone alone and with topical corticosteroids to achieve initial control has been shown with eventual transition to topical tacrolimus to maintain remaining disease activity. 6 For recurrent disease requiring systemic control, weekly methotrexate has been used. 19
Singh et al. 9 commented on the importance of multidisciplinary involvement. This is consistent with our approach, where gynecology has been integrated from the outset. Surgical treatment has been shown to address vulvovaginal agglutination, stenosis, and other severe vaginal scarring despite treatments of systemic immunosuppression, 23 sometimes leading to improved intercourse and decreased urinary symptoms. However, surgical therapy is sometimes rejected by patients as post-procedure treatments in many cases require ongoing self-management with dilators, local estrogen, and vaginal moisturizers, on a consistent basis which is often difficult to maintain. Patency, especially if the disease remains somewhat active, can be unattainable.4,5,9,10,24,25
Given that drug-induced LP is a common cause of recalcitrant disease, a drug review to screen for possible drug culprits and either discontinue or switch the class of medication is part of the treatment strategy.1,15–18 With the involvement of the patient’s family physician, the patient has discontinued her diclofenac/misoprostol use. As the patient was on multiple blood pressure medications that could exacerbate LP, a referral to the hypertension clinic was made. However, after a failed trial to discontinue atenolol, patient remains on atenolol in addition to hydrochlorothiazide and candesartan. Ongoing collaboration with the patient’s family physician and the hypertension clinic continues to explore strategies to further reduce the risk of medication-induced lichenoid reactions. Vulvovaginal stenosis can be induced by VLP, and its management requires a multimodal and multidisciplinary approach.
Footnotes
Acknowledgements
The authors would like to acknowledge Dr. Lynette Margesson and Dr. Sue Ross for their helpful review of this case.
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship, and/or publication of this article.
Informed consent
Informed consent was obtained for this case.
