Vulvovaginal-gingival Syndrome Research Articles

Vulvar-vaginal-gingival-otic syndrome.

In order to retrospectively analyse the multi-site involvement pattern of erosive lichen planus patients, we retrospectively reported the clinical and medical data of three patients with erosive lichen planus which involving their vulva, vagina, gingiva, and ear canal. We confirmed the existence of otic lichen planus, and found that it is more common in patients with vulvovaginal-gingival syndrome of erosive lichen planus. Therefore, we propose 'vulvovaginal-gingival-otic syndrome' to further describe this rare compound pattern of lichen planus.

Multi-modal approach in management of vulvo-vaginal gingival syndrome − a rare variant of oral lichen planus

Lichen planus (LP) is a chronic autoimmune relapsing mucocutaneous disease that significantly affects the patient's quality of life (QoL) and is associated with several etiological factors. Vulvo-vaginal gingival syndrome (VVGS) is characterized as a clinical triad of gingival, vaginal and vulval LP. In previous literature, it has been described as a distinctive pattern of erosive plurimucosal LP. This condition if progressed can lead to multiple complications such as urethral and vaginal stenosis. This case report highlights the management of VVGS using a multidisciplinary strategy that includes dermatological consultation, oral hygiene maintenance, topical steroid application using custom-made trays and photodynamic therapy.

Vulvar lichen planus — clinical spectrum (results of a cross-sectional uncontrolled study)

Background. Lichen planus (LP) is a chronic dermatosis, which can affect vulva. Currently, there are no sufficient data about clinical picture of vulvar LP and a valuation combined lesion on the vulva, oral mucous and smooth skin in patients with different forms of LP.
 Aims. Is to study the features of the vulvar LP clinical picture.
 Materials and methods. A cross-sectional (simultaneous) uncontrolled study of 46 patients with vulvar LP was performed. We assessed the structure of vulvar LP, the duration and the manifestation of disease, localization of lesions, the involvement of the skin and oral mucosa. LichenSclerosus-Square was used to assess the area of lesions in vulvar LP.
 Results. The erosive form dominated in the structure of vulvar LP morbidity, which was diagnosed in 73,9% of cases. The process in erosive and hypertrophic form of LP was more common than in the typical form of LP. Distinctive features of the erosive form of the disease were the involvement of the inner surfaces of the labia minora and the vestibule (73,5% и 70,6%, p 0,001). The erosive LP is also characterized by a damage of normal vulvar architectonics (58,8%, p = 0,007).
 Lesions in typical LP affected labia majora (66,7%) and labia minora (33,4%), and to a lesser extent the commissure posterior (16,7%). The involvement of labia majora was found in all patients with hypertrophic form LP. The involvement of inguinal folds distinguished the hypertrophic form from other forms of LP and was observed in 33,4% of patients.
 Vulva lesions without the involvement of the skin and mucous membranes of other localizations were more typical for patients with erosive and hypertrophic LP (35,4% и 33,3%). Almost half of the patients (47,0%) with erosive LP had the involvement of mucous membranes, 29,0% of them were diagnosed with vulvovaginal-gingival syndrome. In 14.7% of cases, vulvovaginal-gingival syndrome combined with lesions in the scalp (vulvovaginal-gingival-pillar syndrome).
 Conclusions. We have identified clinical features of different forms of vulvar LP, which can be used for differential diagnosis.

Lichen Planus and the Vulvovaginal Gingival Syndrome. Report of a Case and Review of the Literature

<h3>Introduction</h3> Lichen planus is a dermatologic disease that affects both skin and mucosa. Cutaneous lesions of lichen planus present as small polygonal violaceous papules that present most often on the flexor surfaces of the extremities. These lesions may also be present on the vulvar skin and the hair-bearing aspects of the vulva. Lichen planus of the mucous membranes may affect buccal or gingival mucosa and the vulvar vestibule and vagina. Examination of the mouth reveals the white, lacy pattern of hyperkeratotic striae present on buccal mucosa, often bilaterally. The gingiva may be diffusely swollen, erythematous, and exhibit desquamation. In 1982 Pelisse first reported the vulvovaginal gingival syndrome and lichen planus. The main clinical manifestations of the syndrome are a triad of vulvar, vaginal and gingival lichen planus. <h3>Case Report</h3> A sixty-five year old female was referred to the oral surgeon for evaluation of red patches in her mouth. The patient reported that she had a biopsy of her vulvar for which a diagnosis of lichen sclerosis et atrophicus was made. A biopsy of her maxillary gingiva was submitted to the oral pathology laboratory and diagnosed as lichen planus. The vulvar biopsy was sent for comparison. After consultation with Dermatopathology the diagnosis of lichen planus and the vulvovaginal gingival syndrome was made. <h3>Conclusion</h3> We report a case of severe erosive vulvovaginal lichen planus that preceded the onset of gingival lichen planus by nine months. Not uncommonly, patients with severe oral lesions displayed mild genital disease and patients with asymptomatic gingival disease suffered with severe erosive vulvovaginal disease. The diagnosis of vulvovaginal gingival syndrome requires multidisciplinary evaluations by various medical specialists. We have reviewed the literature of this rather rare condition and report an additional case, with the intention of making the practicing clinician aware of this unusual clinical entity.

VULVOVAGINAL GINGIVAL SYNDROME: A CHALLENGING DIAGNOSIS

Vulvovaginal gingival syndrome is defined as a type of lichen planus involving the oral cavity causing desquamative gingivitis with the presence of erythema, pain, halitosis, and bleeding and provoking intensive erythema, pain, loss of the labia minora, vaginal stenosis, and sexual activity impairment due to inflammation and atrophy. A 45-year-old Caucasian woman visited the oral medicine clinic complaining gingival bleeding, lip sores, and severe pain. The patient reported a case of genital candidiasis for more than 10 years without success, with no other diseases reported. She was referred to the gynecology department of the CAISM-Unicamp, São Paulo/Brazil, where a biopsy from the genital mucosa was performed and confirmed genital lichen planus. The oral biopsy had the same result, and lichen planus was confirmed again. With the result of vulvovaginal gingival syndrome, this case is being managed with the use of topical and systemic corticosteroids. Vulvovaginal gingival syndrome is defined as a type of lichen planus involving the oral cavity causing desquamative gingivitis with the presence of erythema, pain, halitosis, and bleeding and provoking intensive erythema, pain, loss of the labia minora, vaginal stenosis, and sexual activity impairment due to inflammation and atrophy. A 45-year-old Caucasian woman visited the oral medicine clinic complaining gingival bleeding, lip sores, and severe pain. The patient reported a case of genital candidiasis for more than 10 years without success, with no other diseases reported. She was referred to the gynecology department of the CAISM-Unicamp, São Paulo/Brazil, where a biopsy from the genital mucosa was performed and confirmed genital lichen planus. The oral biopsy had the same result, and lichen planus was confirmed again. With the result of vulvovaginal gingival syndrome, this case is being managed with the use of topical and systemic corticosteroids.

Prevalence and characteristics of various types of lichen planus in the Moscow Region

Rationale: Lichen planus (LP) is an important problem for the healthcare due to its increasing prevalence, as well as typical, atypical and prone to malignization types of the disease that can be both severe and poorly responding to treatment. Accurate data on the prevalence of LP are lacking. Aim: To assess the prevalence of various LP types in the patient population of the Moscow Region. Materials and methods: We analyzed the results of examination of 338 patients with LP, who were getting standard treatment in the Department of Dermatovenereology and Dermato-oncology of the Moscow Regional Research and Clinical Institute (MONIKI) in 1999 to 2017. Results: Among 338 LP patients, atypical LP of the skin was diagnosed in 81 (23.96%): hypertrophic type, in 31 (38.27%), pigmented, in 23 (28.4%), atrophic, in 11 (13.58%), follicular, in 8 (9.88%), lichen obtusus, in 3 (3.7%), bullous, in 1 (1.23%), erythematous, in 1 (1.23%), vulvovaginal-gingival syndrome, in 2 (2.47%), and Grinspan's syndrome, in 1 (1.23%). In 35 (43.21%) of patients, cutaneous LP was associated with atypical oral LP (OLP): in 17 (48.57%) of cases with hyperkeratotic, in 11 (31.43%), with erosive, and in 7 (20%), with exudative-hyperemic type. In 19 (23.46%) cases, atypical cutaneous LP was associated with LP of the genital mucosae, including in 12 (63.16%) cases with a typical reticular type, in 6 (31.58%), with atypical erosive, and in 1 (5.26%), with hypertrophic type. In 16 (4.73%) of the patients there was atypical OLP only; in 6 (37.5%) of them it had the exudative-hyperemic type, in 5 (31.25%), erosive, and in 5 (31.25%), hyperkeratotic. The most severe course of the dermatosis was observed for follicular, atrophic, hypertrophic types of cutaneous LP, vulvovaginal-gingival syndrome, and erosive OLP. Conclusion: Atypical LP was diagnosed in 81 (23.96%) cases, and in 45 (55.6%) cases it was associated with the OLP. The latter in 35 (43.21%) of cases was characterized by atypical lesions of OLP, including 11 (31.43%) cases of obligate precancerous erosive LP, which necessitates that this patient category should be managed with high cancer vigilance.

Clinical analysis of 11 cases of vulvovaginal-gingival syndrome

Objective To investigate clinical features and therapeutic protocols of vulvovaginal-gingival syndrome (VVGS) . Methods Clinical data were collected from 11 female patients with VVGS and analyzed. Results Among the 11 patients, the mean and median ages at a definite diagnosis of VVGS were both 46 years, and the average disease duration was 4 years. Painful erosive erythema was found on the vaginal vestibule in 7 patients, on the gingiva in 10, as well as on the bilateral buccal mucosa in 5. Additionally, 8 and 4 patients reported dyspareunia and vaginal hemorrhage, respectively. Seven patients were treated with low-dose oral methylprednisolone combined with hydroxychloroquine or colchicine, and the other 4 were treated with oral hydroxychloroquine alone. Furthermore, all of the 11 patients applied topical tacrolimus 0.1% ointment. Finally, 8 patients were cured or markedly improved. No obvious adverse reactions were observed in any of the patients. Conclusions VVGS mostly occurs in middle-aged women, with the vaginal vestibule and gingiva as predilection sites of painful erythema and dyspareunia as common symptoms. Patients have good responses to methylprednisolone combined with hydroxychloroquine, and hydroxychloroquine alone also shows good efficacy in patients with mild VVGS. Tacrolimus 0.1% ointment can be one of drugs for topical maintenance treatment of VVGS. Key words: Vulvar diseases; Lichen planus; Mucous membrane; Treatment outcome; Vulvovaginal-gingival syndrome

Clinical patterns of lichen planopilaris in patients with vulval lichen planus

Funding sources: none. Conflicts of interest: none declared. Dear Editor, Lichen planus (LP) is a common inflammatory dermatosis that can affect the skin, scalp or nails, as well as mucosal surfaces, including oral, genital and oesophageal sites and the lacrimal duct. On the scalp, lichen planopilaris (LPP) may precede lesions of classic LP on the body in > 50% of cases,1 2 and clinically it may present with patchy or diffuse hair loss, or in the patterned variant of frontal fibrosing alopecia (FFA).1 In the vulva, three forms of LP are recognized – classic, erosive and hypertrophic. The most common type of vulval LP is erosive LP, which can lead to significant scarring with alteration of the normal vulval architecture. In the vulvovaginal–gingival syndrome subtype of erosive LP, vaginal stenosis can lead to functional and psychosexual sequelae.3 The aims of our study were to determine the frequency of patients with vulval LP and concomitant scalp LPP, and to establish the patterns of scalp LPP in these patients. A secondary aim was to identify the frequency of oral LP in this cohort.

Systematic review of current systemic treatment options for erosive lichen planus

Erosive lichen planus (ELP) is an extremely painful condition characterized by ulcerative oral and genital lesions that cause desquamation of the epithelium and bleeding. ELP is more common in women, often recalcitrant to medical therapy and may result in transformation to squamous cell carcinoma. The objective of this review was to assess the evidence in support of various systemic therapies for ELP. PubMed and the Cochrane Database of Systematic Reviews were queried from 1966 to 2009 using the term ‘treatment’ in combination with ‘erosive lichen planus’, ‘mucosal lichen planus’, ‘vulvar lichen planus’, ‘oral lichen planus’ or ‘vulvovaginal gingival syndrome’. A total of 424 cases, 417 of which met the selection criteria, were identified. Each therapeutic option was scored using the stevens healthcare needs assessment scale for quality and level of evidence, and then weighted based on prevalence of use to arrive at an overall assessment score and rank order.

Lacrimal Canalicular Duct Scarring in Patients With Lichen Planus

Lichen planus (LP) is a mucocutaneous inflammatory disease that affects multiple sites, including the skin, oral cavity, vulva, and vagina and can result in scarring and stricture formation. It has also been shown to cause lacrimal canalicular blockage in a series of patients attending an ophthalmology clinic. We describe a cohort of women with vulvovaginal LP who also had signs of lacrimal canalicular scarring on examination. We report 9 cases of LP with scarring of the conjunctiva around the lacrimal ducts. Seven of 9 women had symptoms of epiphora, and in 2 women lacrimal canalicular scarring was an incidental finding. Seven of 9 cases were diagnosed by an ophthalmologist. All women had biopsy-proven LP at 1 mucocutaneous site each. Seven of 9 women had vulvovaginalgingival syndrome, which is a subgroup of severe erosive LP. Given the strong association between erosive mucocutaneous LP and multisite scarring sequelae, it is not unexpected that ocular inflammation may lead to lacrimal duct stenosis. We believe that this complication has been underreported among patients with LP and that an ophthalmological history and examination of the punctum of the lacrimal duct should be sought, especially in patients with the erosive subtype of LP.

Treatment of recalcitrant vulvovaginal gingival syndrome with adalimumab

To the Editor: Vulvovaginal gingival syndrome (VGS) consists of the triad of erosive/desquamative vulvitis, vaginitis, and gingivitis.1 Previous studies have shown that tumor necrosis factor-alpha (TNF-α) is overexpressed in the cytoplasm of basal epithelial keratinocytes from patients with VGS. Since TNF-α is a key player in cell-mediated cytotoxicity, its overexpression may play a role in VGS pathophysiology by intensifying and perpetuating the inflammatory process.2,3 We therefore decided to pursue off-label treatment of recalcitrant VGS with adalimumab, a human IgG1 monoclonal antibody TNF-α antagonist.

Vaginal Agglutination

Graft-versus-host (GVH) disease is a common problem in transplant patients, whereas vulvovaginal gingival syndrome is an uncommon and severe variant of lichen planus characterized by erosions of characteristic mucosal surfaces, with frequent vaginal involvement, resulting in scarring or stricture formation. Both conditions have the potential to present with similar clinical pictures. We report the history, evaluation, and treatment of a woman who had recently undergone stem cell transplant for acute lymphoblastic leukemia who presented with vaginal agglutination. A clinical diagnosis of erosive lichen planus versus chronic GVH disease was considered. Lichen planus and GVH disease are both inflammatory processes, which can present with a range of clinical conditions. Each may result in the development of irritative symptoms and erosive lesions on mucosal surfaces. Although lichen planus is a well-defined dermatosis, GVH disease is an iatrogenic process. We report the case history of a patient with erosive vulvovaginal lesions with scarring, likely caused by GVH disease, which mimicked erosive vulvovaginal lichen planus. Although the clinical presentation and treatment of these 2 entities are similar, this case demonstrates the subtle diagnostic difference between the 2 diseases.

Lichen planus

Lichen planus

The Vulvovaginal-Gingival Syndrome of Lichen Planus

The vulvovaginal-gingival syndrome is a variant of mucosal lichen planus characterized by erosions and desquamation of the vulva, vagina, and gingiva. The clinical features of patients initially evaluated for vulvitis who exhibited the additional features of this syndrome have been described in one previous study. In the current study, 22 women with biopsy-proved oral lichen planus and clinical evidence of vulvovaginal lichen planus were examined and treated. This group of patients represents the largest reported series demonstrating the vulvovaginal-gingival syndrome. Gingival lichen planus, present in all patients, was characterized by erosions and erythema in 16 subjects and by white, reticulated lesions in six others. Vulvovaginal lichen planus also displayed erosions in the majority of patients. The response to a wide variety of therapeutic agents was satisfactory in most patients. However, concomitant use of several drugs was usually required to achieve beneficial results. Patients with oral lichen planus should be routinely examined for the presence of disease on other mucosal surfaces. The recognition of this syndrome, which is undoubtedly more common than previously reported, will avoid unnecessary delay in the treatment of these patients.