Introduction
Vulvovaginal gingival lichen planus (VVG-LP), also known as Hewitt-Pelisse syndrome, is a rare mucosal variant of lichen planus (LP) that was first described in 1982 by M. Pelisse [1, 2]. The disease is characterized by a triad of symptoms including painful erosive lesions on the labia, erosive or desquamative lesions within the vagina, and erosive vestibular gingivitis [2]. VVG-LP is associated with an elevated risk of malignancy at the affected sites.
Objective
The aim of this paper is to present a case of a 60-year-old female patient diagnosed with VVG-LP, manifested by subclinical changes in the external genitalia.
Case report
A 60-year-old woman with a history of arterial hypertension and hypercholesterolemia, thyroidectomy, and left ovary resection, being on long-term treatment with telmisartan combined with hydrochlorothiazide, levothyroxine, rosuvastatin, bilastine, omeprazole, and vitamin D and calcium supplements, presented to the Dermatology Department in Olsztyn with symptoms of gum swelling, tenderness, and cherry-red discoloration (fig. 1). The patient reported that her last menstrual period occurred at the age of 50, and she was not receiving hormone replacement therapy. The onset of changes occurred after a dental procedure 6 years prior to admission (before the initiation of antihypertensive treatment). Direct immunofluorescence (DIF) examination of a specimen taken from affected gingiva (2018) revealed the presence of Civatte bodies without any findings characteristic of bullous diseases. Following COVID-19 in 2021, changes in the patient’s oral cavity became more severe. Based on the histopathological examination of a gingival specimen (2022), a diagnosis of desquamative gingivitis was established. Upon admission, the assessment revealed gingival hyperemia, hyperplasia, and petechiae on the hard palate. Additionally, examination of the anogenital area revealed a whitish appearance, labial atrophy, and erythematous lesions, none of which caused significant subjective discomfort (fig. 2). Laboratory tests showed a total cholesterol level of 301 mg/dl and LDL of 208 mg/dl, with no other abnormalities noted. Hepatitis B and C and diabetes were excluded. A second gingival specimen revealed a chronic mixed cellular infiltrate, while another specimen from the labia majora confirmed the diagnosis of LP (fig. 3). Patch testing demonstrated hypersensitivity to nickel, neomycin, palladium, Lyral, and sesquiterpenes. An analysis of allergen exposure showed no correlation with LP-like changes. The titer of antinuclear antibodies (ANA) was measured at 1 : 640. VVG-LP was diagnosed. The treatment regimen included prednisone 20 mg/day and cyclosporine 300 mg/day (4.5 mg/kg body weight), with a gradual dose reduction over a 6-month period, along with adjustments to antihypertensive and hypolipidemic medications. Cyclosporine was discontinued, and prednisone was continued at a dose of 5 mg every other day. Topical clobetasol was applied to the vulvar lesions every other day, while fluocinolone was applied to the oral lesions once daily. Two sessions of ALA-PDT (aminolevulinic acid-based photodynamic therapy; Ameluz, 630 nm red LED light, 37 J/cm², 80 mW/cm² in pulsed mode) were conducted 5 weeks apart (fig. 4). Gingival tenderness and hyperplasia were reduced, and erythematous lesions in the genital area resolved.
Discussion
VVG-LP typically occurs in the 5th to 6th decades of life and is more commonly observed in females. An association has been found between the onset of disease symptoms and the presence of the HLA DQB1*0201 allele [3]. A total of 20% of women with oral LP also experience involvement of the mucous membranes of their reproductive organs [4].
Patients with VVG-LP exhibit erythematous lesions with hyperkeratotic, serrated edges at the junction of the skin and mucosa on the labia, while the vaginal mucosa is affected by superficial erosions, erythema, and friability. Chronic inflammation may lead to scarring (90% of cases), atrophy and fusion of the labia, loss of interlabial sulcii, clitoral phimosis, and vaginal stenosis, resulting in symptoms such as bleeding, dyspareunia, pruritus, and a burning sensation during micturition [3, 5, 6]. In the anal region, the lesions appear as erythematous foci, erosions, and whitish patches. They may be accompanied by the characteristic skin symptoms of LP in other body areas [5]. Desquamative gingivitis with localized erosive changes, along with edema and pain, is frequently observed in the oral cavity [4]. White fibrous bands with a lace-like pattern may appear on the inner buccal mucosa [5, 6].
The severity of symptoms ranges from subclinical manifestations to lesions causing pain, burning, discomfort, and even bleeding during tooth brushing and sexual intercourse, significantly impacting quality of life [5, 6]. One study found that 77.42% of patients with oral LP experienced increased levels of psychological stress, which positively correlated with the intensity of perceived pain [7].
The syndrome is diagnosed based on histopathological confirmation of LP in a tissue specimen from at least one affected site, following the exclusion of bullous diseases through immunological testing [3]. The typical appearance of the specimen epidermal/epithelial, acanthosis with a ‘sawtooth’ pattern, vacuolar degeneration of the basal layer, band-like infiltration along the dermal-epidermal junction, and the presence of Civatte bodies [6].
Genital symptoms should be differentiated from idiopathic vaginitis, while oral lesions need to be distinguished from pemphigoid and pemphigus vulgaris [3, 5]. The differential diagnosis should also include lichen sclerosus, which tends to spare the vagina, less frequently affects the oral cavity, and is more commonly found in the anal region [8].
VVG-LP is frequently misdiagnosed due to the lack of oral cavity assessment by gynecologists and anogenital area examination by dentists. An additional challenge lies in differentiating vaginal and vulvar changes caused by VVG-LP from the atrophic changes that occur after menopause. Patients rarely consult dermatologists, as generalized skin lesions are present in only 40% of VVG-LP cases [4].
The primary pharmacotherapy for mild cases includes topical glucocorticoids (GCs) and calcineurin inhibitors. Second-line treatments include vitamin D derivatives and retinoids, with hyaluronic acid and aloe vera also showing potential in symptom relief [9, 10]. Additionally, photodynamic therapy proves effective for the treatment of localized lesions [11].
The chronic nature and severe subjective symptoms of VVG-LP often necessitate more intensive treatment than other subtypes of LP [4]. Systemic GC therapy, combined with immunosuppressive treatment, is reserved for more severe cases. When using high doses of GCs, potential adverse effects, such as insomnia, diarrhea, mood swings, irritability, fluid retention, hypertension, and increased susceptibility to infections, should be considered [5, 8, 11]. Systemic steroid therapy is used to halt the rapid progression of symptoms. Steroid-sparing agents include azathioprine, methotrexate, cyclosporine, mycophenolate mofetil, antimalarial drugs, dapsone, and retinoids, but their efficacy in VVG-LP is variable, and there are no strong recommendations for their use [9, 10]. Treatment decisions should be made on an individual basis for each patient.
Conclusions
Vulvovaginal-gingival syndrome is a variant of lichen planus that requires multidisciplinary management. Lesions that do not cause severe symptoms often lead to a delayed diagnosis, highlighting the importance of a thorough physical examination. Early diagnosis and prompt multidirectional treatment help to prevent irreversible complications and reduce the risk of malignant transformation of lesions. Education on VVG-LP should be promoted among healthcare professionals across various specialties, particularly dermatologists, dentists, gynecologists, and urologists.
Funding
No external funding.
Ethical approval
Not applicable.
Conflict of interest
The authors declare no conflict of interest.
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