Vulvo-vaginal ano-gingival syndrome: Another variant of mucosal lichen planus.

Vulvo-vagino-gingival syndrome was described as a distinctive pattern of erosive plurimucosal lichen planus (LP), and it is a clinical triad of vulval, vaginal, and gingival LP. It can lead to sequelae such as vaginal and urethral stenosis which can have severe implications on the quality of life. We report a case of a 40-year-old female who developed urethral, vaginal, as well as anal stenosis as a result of long-term exclusive mucosal LP involving vulvo-vaginal and anal mucosa along with oral LP without any other cutaneous involvement. This case is being reported because of the rare association of anal LP with vulvo-vagino-gingival syndrome and its gross similarity to lichen sclerosus.

INTRODUCTION

Lichen planus (LP) is an inflammatory disease involving skin, mucous membranes, hair, and nails, having a chronic course with characteristic clinical and histopathologic features. The oral mucosa is the most common mucosa involved in LP and may affect up to 1%–2% of the general population.[1] Approximately, 25% of women with oral LP also have vulvo-vaginal involvement.[2] Mucosal LP (MLP) of genital skin is usually located on the glans penis in males and vulva and vagina in females.[3] In a retrospective study, the frequency of vulval LP was found to be 3.7% in the population attending a vulval clinic.[4]

The vulvo-vagino-gingival syndrome (VVGS) has been described as a distinctive pattern of erosive plurimucosal LP characterized by a triad of vulval, vaginal, and gingival LP.[5] In a recent large series of VVGS, 90% of the cases were found to develop genital fibrosis and stricture, but there are only a few reports of isolated vulval LP leading to stenosis.[6] Vaginal and urethral stenosis can have a significant negative impact on the quality of life of the patient.

Lichen sclerosus (LS), on the other hand, is an autoimmune, inflammatory skin disease affecting anogenital region preferentially rather than other mucocutaneous sites and may be associated with cutaneous asymptomatic porcelain white atrophic macules. It can affect vulval and perianal mucosa severely and can cause vulvo-anal stenosis in a “figure of eight” configuration.[7]

CASE REPORT

A 40-year-old female presented with the complaints of narrowing of vaginal and urethral orifices along with moderate itching and erythema over genital region and chronic constipation for 5 years. In the subsequent year, her anal region was also involved presenting with pain and an inability in passing stools as well as dyspareunia and urinary retention. For the past 6 years, the patient was having recurring oral erosions over gums along with burning sensation on eating spicy food. She underwent multiple cystoscopies and proctoscopies in the past 3 years for urethral and anal dilatation but with only partial, short-term relief. There was no history of vaginal discharge. She gave a past history of dental filling 18 years back and was a known case of hypothyroidism on treatment. Family history was not significant.

On mucocutaneous examination, intense erythema of vulva, vagina, and partial fusion of labia minora and majora with burying of clitoris were present [Figure 1]. Few old-healed hypo- and hyper-pigmented patches were present over labia minora and clitoris. Per speculum examination could not be done due to Grade 2 + introital stenosis. Anal mucosa exhibited hyperpigmentation, fissuring, and scaling, along with marked stenosis [Figure 2]. On examination of the oral cavity, violaceous plaques were present over labial and lingual aspects of the maxillary and mandibular gingival surfaces [Figure 3]. Cutaneous examination revealed no violaceous or hypopigmented patches or plaques, and hair and nails were also normal. All the routine investigations were unremarkable. Provisional diagnosis of vulval LP with a differential diagnosis of LS of genital mucosa was kept.

Figure 1

Erythema of vulva, vagina, and partial fusion of labia minora and majora and burying of clitoris with histopathology revealing basal vacuolization, necrotic keratinocytes, Max Joseph spaces, and pigmentary incontinence, along with dermal lymphocytic proliferation

Figure 2

Hyperpigmentation, fissuring, and scaling with marked stenosis of anal mucosa and histopathology showing fibrosis, pigmentary incontinence, basal vacuolization, and necrotic keratinocytes, along with dense dermal lymphocytic infiltration

Figure 3

Hyperpigmentation and erosions along gingival margins

Three mucosal biopsies were done. The one from erythematous patch on labia minora revealed orthohyperkeratosis, basal vacuolization, squamatization, necrotic keratinocytes, Max Joseph spaces, and pigmentary incontinence, along with dermal lymphocytic proliferation [Figure 1]. The second biopsy from hyperpigmented patch near clitoris showed orthohyperkeratosis, focal atrophy, pigmentary incontinence and increased fibroblastic proliferation, collagen synthesis, and lymphocytic infiltrate. The third biopsy from perianal region exhibited fibrosis, pigmentary incontinence, basal vacuolization, and necrotic keratinocytes, along with dense dermal lymphocytic infiltration [Figure 2]. Thus, the diagnosis of vulvo-anal LP with severe secondary sequel leading to adhesions and narrowing of introitus and anal orifice along with oral mucosal involvement was made.

DISCUSSION

In 1982, Pelisse et al. coined the term “VVGS” for the triad of vulval, vaginal, and gingival erosive LP and proposed it to be a distinct clinical variant of LP.[5] Our case presented with a tetrad of mucosal involvement with the combination of VVGS along with MLP of anus in a “figure of eight” configuration, characteristically seen in LS.

LP affects approximately 1% of all women, most commonly on the oral mucosa almost anywhere over buccal mucosa, tongue, gingiva, and lips.[2] LS rarely affects the oral mucosa as white plaques on the lips or buccal mucosa, but gingiva is spared.[7] Approximately, 25% of women with oral LP also have vulvo-vaginal involvement mainly as erosive LP. Erosive LP typically presents with erosions involving the introitus, clitoris, clitoral hood, labia minora, and majora.[7] Vaginal involvement has been reported in up to 70% of patients with erosive vulval LP as erythema, contact bleeding, erosions, and scarring with synechiae.[2] The vagina is never affected in LS.[7] Approximately, 60% of patients of LS have perianal involvement, but there are only three published case reports of anal LP.[8]

The involvement of vagina and gingiva in our case along with the classical findings of basal layer vacuolization and interface dermatitis in the vulval and perianal histopathology helped in differentiating our case from LS. We labeled it as vulvo-vaginal ano-gingival syndrome (VVAGS).

All the four mucosal surfaces developed LP one after the other over an interval of 3–4 years in this case as it has been reported in other cases of VVGS. Only the mucosae were involved without any cutaneous lesion, thereby suggesting the interplay of specific host factors such as genetic or viral triggers in exclusive MLP. HLA-DQB1*0201 gene association was found in 80% of women with vulvo-vaginal-gingival syndrome, supporting the hypothesis of genetic predisposition for the syndrome.[6]

There was stenosis of urethral, vaginal, and anal orifices, thereby severely affecting patient's physiological and psychosexual health. Loss of vulval anatomy, stenosis, synechiae, and a remote possibility of malignant transformation can be explained on the basis of chronic repetitive inflammation, repair process, and scarring.[9] Therefore, clinical diagnosis of VVAGS demands early confirmatory diagnosis and effective management with the help of immunosuppressants.

CONCLUSION

VVGS is a clinical triad of vulval, vaginal, and gingival LP. This case is unique because of the following reasons:

LP of anus along with VVGS is being reported for the first time

As with most of the reported cases of VVGS, this case also did not have cutaneous LP, thereby suggesting LP to have a clinical spectrum with exclusive mucosal involvement at one end and cutaneous predominant disease at the opposite end

In this case, all the four mucosal surfaces developed LP asynchronously over an interval of 3–4 years, a pattern similar to other reports of VVGS.

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Conflicts of interest

There are no conflicts of interest.