Physician-administered clinical score of vulvar lichen sclerosus: A study of 36 cases.

INTRODUCTION: Vulvar lichen sclerosus (VLS) often remains undetected for years due to lack of awareness, as well as private nature of the disease. Advanced disease severely affects the quality of life and is associated with increased risk of vulvar squamous cell carcinoma (SCC). The aim of this study was to assess the usefulness of a physician-administered clinical scoring system for the clinical diagnosis and evaluation of VLS. To the best of our knowledge, this is the first study using the clinical score developed by Günthert et al on the VLS patients.
MATERIALS AND METHODS: The study conducted was an observational cross-sectional study of 36 cases attending Dermatology OPD of Government Medical College with clinically proven and previously untreated VLS enrolled over a 1 year period. Cases were retrospectively subjected to evaluation by physician-administered clinical score based on 6 clinical features, viz. erosions, agglutination, hyperkeratosis, stenosis, fissures, and atrophy.
RESULTS: The average age of 36 clinically diagnosed VLS cases was 56.4 years. Most common clinical features were hyperkeratosis and atrophy found in 86.11% cases followed by erosions (75%) with one-third cases having grade 2 (severe) changes. One case had well-differentiated SCC clitoris at presentation. The mean physician-administered clinical score was 5.583, and 80.56% cases had clinical score ≥4 validating the clinical diagnosis. DISCUSSION: Early diagnosis and prompt treatment is the key to prevent sequelae and complications of VLS. The physician-administered clinical score can be a useful tool to diagnose and later evaluate the response of treatment and prognosis of VLS cases.

INTRODUCTION

Although the vulva is the most visible female genital structure, it has received the least attention in the medical literature and has even been referred to as “the forgotten pelvic organ.”[1] Vulvar lichen sclerosus (VLS) is one of the chronic inflammatory vulvar dermatoses with a prevalence estimated to range from 1 in 300 to 1 in 1000 of all patients referred to dermatology departments. VLS often remains undetected for years. Advanced disease severely affects the quality of life and is associated with increased risk of vulvar squamous cell carcinoma (SCC).[2] The early diagnosis and prompt treatment is of the utmost importance.

Günthert et al[3] has developed a physician-administered clinical scoring system to validate the diagnosis made clinically and to evaluate treatment response during subsequent follow-ups. This can be an important tool in an otherwise difficult to diagnose condition. The purpose of our study was to assess the usefulness of physician-administered clinical score for the clinical diagnosis and evaluation of VLS in retrospect. To the best of our knowledge, this is the first study using the clinical score developed by Günthert et al[3] on the VLS patients.

MATERIALS AND METHODS

The study conducted was an observational cross-sectional study. Thirty-six cases attending Dermatology OPD of Government Medical College with clinically proven and previously untreated VLS were enrolled over a 1 year period. The clinical diagnosis was made in presence of following points:[4]

Chronic intractable itching over vulva

Pigmentary changes (depigmentation) of cutaneous/mucocutaneous aspect of vulva with or without presence of erythema/erosions and fissuring

Atrophic changes including labia minora fusion and clitoral atrophy (leading to burying of clitoris with/without introital narrowing).

Biopsy was done in doubtful cases.

Cases were retrospectively subjected to physician-administered clinical score developed by Günthert et al[3] to validate the diagnosis. The clinical score was evaluated based on clinical findings and photographs of patients.

The clinical score was based on six clinical features: Erosions, agglutination, hyperkeratosis, stenosis, fissures, and atrophy. Each feature was graded on a 3-point Likert scale with grade 0 as normal findings, 1-moderate, and 2-severe changes [Table 1].[3] Total score of ≥4 (range: 0-12) was considered VLS in the presence of typical symptoms.[3] Typical symptoms pointing toward diagnosis besides intractable itching and/or soreness were irritation, dyspareunia, dysuria, and fecal/urinary incontinence.

Table 1

Definition of clinical scores and their grades (0, 1, and 2)

RESULTS

Of 36 clinically diagnosed VLS cases, 31 (86%) were post-menopausal while 5 (14%) were in menstruating age group. Average age of the case series was 56.4 years. Most common symptom was intractable itching found in 36 (100%) cases followed by dysuria in 7 (19.4%) cases, dyspareunia in 2 (5.6%) cases, and soreness and urinary incontinence in 1 (2.8%) case each.

The presence and severity of each of the six clinical features were assessed [Table 2]. Most common features were hyperkeratosis and atrophy found in 86.11% cases with at least one-third cases having grade 2 (severe changes). Erosions were seen in 75% cases corresponding with intractable itching, found as the most common presenting symptom [Figure 1]. One case had well-differentiated SCC clitoris at presentation.

Table 2

Physician-administered clinical score (n=36)

Figure 1

A 65-year-old patient with vulvar lichen sclerosus score 9 showing grade 2 erosions, grade 2 hyperkeratosis, grade 2 fissures, grade 1 agglutination, grade 1 atrophy, and grade 1 stenosis

Comparison between present study (n = 36) and study by Günthert et al[3] (n = 24) revealed that atrophy, hyperkeratosis, and erosion were three most common clinical signs at presentation in both of them [Figure 2 and Table 3].

Figure 2

A 50-year-old patient with vulvar lichen sclerosus score 4 showing grade 1 erosions, grade 1 hyperkeratosis, grade 1 fissures, and grade 1 atrophy

Table 3

Comparison of clinical signs at presentation in two studies

The mean physician-administered clinical score in the present study was 5.583 (5.5 in Günthert et al[3] ). Out of 36 patients, 29 had clinical score ≥4; thus validating the clinical diagnosis in 80.56% cases [Figure 3].

Figure 3

Clinical scores of present study (n = 36; range of clinical scores 0– 12; clinical score validating diagnosis of vulvar lichen sclerosus ≥4)

DISCUSSION

VLS is a chronic inflammatory (lymphocyte-mediated) vulvar dermatosis characterized by ivory-white plaques, “cigarette paper” such as epidermal atrophy.[5] There is often a delay in diagnosis of VLS with causes including lack of awareness in patients as well as physicians, failure to examine genital skin properly, and embarrassment to patients due to private nature of the disease. This delay not only affects the quality of life but may also lead to sexual dysfunction and avoidance of sexual intimacy.[1]

VLS has two peak ages of presentation, prepubertal girls and postmenopausal women (mean age of onset is fifth or sixth decades), but is most commonly present in peri- or post-menopausal women.[26] In the present study, there were no prepubertal girls with 86% cases being menopausal.

Women complain of intractable pruritus (worse at night), irritation, soreness, dyspareunia, dysuria, and urinary or fecal incontinence. Nine percent of cases may be asymptomatic.[6] Recalcitrant cases, especially those associated with erosion or progressive scarring may result in severe sexual dysfunction.[5] Intractable pruritus was found in all patients of the present study. In a study by Singh et al,[7] 24 (92.6%) out of 26 VLS patients had intractable itching, 2 (7.7%) had dyspareunia, and 1 (3.8%) had burning micturition. The dyspareunia and problems related to sex are under-reported in Indian studies due to sociocultural taboo in discussing such private issues.

VLS is associated with a 4-6% risk of SCC, making long-term follow-up essential in these patients[2] (level of evidence - C[8] ). In the present study, one patient at the time of presentation had well-defined SCC clitoris associated with VLS highlighting the delayed diagnosis [Figure 4].

Figure 4

A 70-year-old patient with vulvar lichen sclerosus score 9 showing grade 1 erosions, grade 2 hyperkeratosis, grade 2 agglutination, grade 2 atrophy, and grade 2 stenosis, with well-differentiated squamous cell carcinoma clitoris

Other sequelae/complications include scarring, introital narrowing (stenosis), pseudocyst of the clitoris, sensory abnormalities (dysesthesia, vestibulodynia, and vulvodynia) and psychosexual problems.[4] Intriguingly, the present study had 63.89% cases presenting with stenosis and 86.11% cases with atrophy (commonest presentation along with hyperkeratosis) highlighting that the sequelae/complications were already set in during presentation [Figure 5].

Figure 5

A 75-year-old patient with vulvar lichen sclerosus score 11 showing grade 2 erosions, grade 2 hyperkeratosis, grade 1 fissures, grade 2 agglutination, grade 2 atrophy, and grade 2 stenosis

The physician-administered clinical score ≥4 was found in 80.56% cases emphasizing that this clinical score indeed helps in validating diagnosis of VLS with clinical confidence. Remaining 20% cases had typical symptoms, so must be followed periodically (or confirmed with biopsy).

The proper and timely usage of clinical score helps in early diagnosis and prompt treatment of the disease. This early diagnosis and treatment in turn helps alleviate symptoms, prevent architectural damage, and reverse histologic changes.

Advantages of clinical scoring

The validation of clinical diagnosis can help in cases refusing biopsy

Ensures early diagnosis and prompt treatment of LS which helps prevent the irreversible changes to ensue

Good for estimating severity and progression of the disease

Good tool to assess treatment response.

Limitations of clinical score

Clinical score in the presence of typical symptoms is not sufficient to replace confirmation of VLS by histopathology, so biopsy is still mandatory in doubtful cases.

Take home message

VLS is not only chronic but also affects the quality of life including sexual health and has a potential for developing into vulvar malignancy

The disease remains under-diagnosed because of lack of awareness on patient's and physician's sides

Clinical scoring of vulva is a handy tool to diagnose and later evaluate the progression of the disease

Early diagnosis and initiation of treatment to prevent irreversible sequelae holds the center-stage in VLS management.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.