Diagnosis Of Vulvar Lichen Sclerosus Research Articles

A Longitudinal Multiinstitutional Study of Vulvar Lichen Sclerosus: From Childhood to Perimenopause.

The main outcome of this study was the evaluation of clinical characteristics, comorbidities, and therapeutic approaches in patients with vulvar lichen sclerosus (VLS) aged from childhood to perimenopause. Secondly, it was intended to compare these characteristics according to the menarchal status. Patients less than 45 years of age with a diagnosis of VLS from January 2002 to June 2022 in 10 referral centers were included in this retrospective longitudinal study. The univariate analysis compared the dependent variables according to menarchal status. One hundred eighty-six patients met the inclusion criteria. At diagnosis, between 25% and 40% of premenarchal patients reported signs related to subepithelial hemorrhage. A significantly greater presence of bleeding (p < .005), easy bruising (p = .028), fissures (p = .008), petechiae/splinter hemorrhages (p < .001), and bleeding/blistering or open sores (p = .011) was observed in premenarchal patients with respect to the postmenarchal group. The perineum (p = .013) and the perianal region (p < .001) were significantly more involved in the premenarchal group. Topical calcineurin inhibitors were more used in the premenarchal population (p = .004), whereas vitamin E oil and moisturizers were more used in the postmenarchal population (p = .047). Vulvar lichen sclerosus is a chronic condition that can cause vulvar changes that result in severe morbidity and affects sexual function and quality of life, even before menopause. Vulvar lichen sclerosus continues to be misdiagnosed in this population. This may lead to an average delay from symptom onset to diagnosis. Evaluating clinical manifestations of VLS in premenarchal and postmenarchal age allowed us to find different clinical characteristics between the 2 periods suggestive of the diagnosis.

Genital and extragenital oncological risk in women with vulvar lichen sclerosus: A multi-center Italian study.

A cohort of women diagnosed with and treated for vulvar lichen sclerosus in three Italian gynecological and dermatological clinics (Turin, Florence, and Ferrara) was retrospectively reviewed. Patient data were linked to cancer registries of the respective regions. The risk of subsequent cancer was estimated by dividing the number of observed and expected cases by the standardized incidence ratio. Among 3414 women with a diagnosis of vulvar lichen sclerosus corresponding to 38,210 person-years of follow-up (mean 11.2years) we identified 229 cancers (excluding skin cancers and tumors present at the time of diagnosis). We found an increased risk of vulvar cancer (standardized incidence ratio=17.4; 95% CL 13.4-22.7), vaginal cancer (standardized incidence ratio=2.7; 95% CL 0.32-9.771), and oropharyngeal cancer (standardized incidence ratio=2.5; 95% CL 1.1-5.0), and a reduced risk of other gynecological tumors (cervical, endometrial, ovarian) and breast cancer. Patients with vulvar lichen sclerosus should undergo annual gynecological check-up with careful evaluation of the vulva and vagina. The increased risk of oropharyngeal cancer also suggests the need to investigate oropharyngeal cavity symptoms and lesions in patients with vulvar lichen sclerosus.

Optimization of treatment of vulvar scleroatrophic lichen with using modern laser technologies

Background. Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis, which is characterized by focal skin atrophy of the vulva and mucous membranes. It leads to deformation of the perineum. The prevalence of VLS in children is up to 0.1%, among the adults — 3%. The average age of the first symptoms appearance in girls is 5.4 years, in women — 55.1 years. The diagnosis of VLS is made on the base of anamnesis, assessment of pubertal development, physical examination of the external genitalia, advanced vulvoscopy and vaginoscopy, bacteriological, bacterioscopic and cytological studies of vaginal wet mount, assessment of the hormonal profile. The treatment methods include high-potency steroids as the initial treatment, usage of diode, fractional CO2 and erbium lasers with different wavelengths and injections of platelet-rich plasma to improve the regeneration processes of the affected tissue by improving collagenogenesis, vasculogenesis, production of proteoglycans and glycoproteins.&#x0D; The aim of the study is to compare the effectiveness of photodestruction with a manual diode laser of the Dornier Medilas D LiteBeam, wavelength of 940 nm, minimum power in pulsed mode on the girls’ vulva mucosa with various types of VLS during neutral and preadolescent age. To obtain the results there were analyzed criteria including the size of the lesion after photodestruction therapy, the disappearance of cracks and angiomatosis in the LS area. The objectives of the complex therapy of VLS in girls were elimination of itching, decrease of the inflammatory changes, improvement of the skin and mucosa blood supply, and stimulation of local immunological mechanisms.&#x0D; Materials and methods. The study was conducted on the base of the gynecological department of Russian Children’s Clinical Hospital. The effectiveness of the laser-induced thermotherapy with a wavelength of 940 nm, minimum power in pulsed mode on the vulva of VLS girls of neutral and preadolescent periods as a part of the complex therapy was evaluated. Ninety 5 to 13 year VLS patients took part in the trial. They were divided into 3 groups. The first group included 30 patients (average age 7.5 ± 1.1) with atrophic form of lichen treated with a laser with a wavelength of 940 nm. The second group consisted of 30 patients (average age 6.8 ± 1.3) with erythematous-edematous lichen, who were treated with a laser with a wavelength of 635 nm. The third group included 30 patients (average age 7.3 ± 1.2) with erosive and ulcerative form of VLS, who received high-potency corticosteroids therapy. &#x0D; Results. The treatment results in the three subgroups of VLS patients show a diode laser with a wavelength of 940 nm therapy to be the most effective. After the first treatment course 27 (90%) patients in the first group showed a positive trend with a decrease in itching and burning, pink-color presence in vulva mucousа, a decrease of the skin pigmentation area and an increase in the elasticity of the skin. After 2 courses, 29 (96.7%) patients had positive trend. The second most effective method of treatment is photodestruction with a 635 nm wavelength laser. After the 4 courses (two hospitalisations), 25 (83,3%) patients noted a decrease in itching and in a “tightening” feeling. In 24 (80%) patients there was an improvement in the condition of the mucous membranes of the vulva. Reduction of edema was observed in 23 (76.7%) patients by the time of an examination in 3 weeks after the last therapy course. However, it was not possible to completely restore the mucous membranes of the vulva in patients of this group. The least effective — drug therapy with high-potency steroids. 8 (26.7%) patients noted positive dynamics only by the 4th day therapy, 11 (36,7%) by the time of the 3 weeks after the initial application of the ointment, 12 (40%) by the 2nd month. In 5 (16.7%) patients, the visible condition of the mucous membranes did not change, the number of bleeding ulcers decreased minimally.&#x0D; Conclusions. The results of the trial indicate the high efficiency of laser therapy of VLS in children compared with the topical glucocorticoids treatment. The duration of the therapy and condition of the external genitalia after treatment depends on the histological form and stage of VLS. According to our study, laser therapy contributes to the remission onset in pediatric patients with different types of VLS in the shortest possible time after the photodestruction procedure.

Presenting Symptoms and Diagnosis of Vulvar Lichen Sclerosus in Premenopausal Women: A Cross-Sectional Study.

Presenting symptoms of vulvar lichen sclerosus (LS) specific to premenopausal women are not well reported in the literature and may differ from those in postmenopausal women. This study aimed to characterize the presentation of vulvar LS among premenopausal women. An observational web-based study was conducted in premenopausal women with biopsy-confirmed vulvar LS between the ages of 18-50 years. Participants completed a 28-question survey evaluating characteristics of symptoms, timing of diagnosis, alternate diagnoses, and presence of concomitant autoimmune conditions. Of the 956 responses received, 503 met inclusion criteria of biopsy-confirmed LS and premenopausal status. Average age of symptom onset was 27 years, and average age of diagnosis was 32 years, with a 4-year delay in diagnosis. Symptoms most present were dyspareunia (68%) and tearing with intercourse or vaginal insertion (63%). Symptoms that affect the individual most were also dyspareunia (44%) and tearing with intercourse or vaginal insertion (39%). Symptoms that most frequently prompted patients to seek medical attention were dyspareunia (35%), pruritus (31%) and tearing with intercourse or vaginal insertion (26%). Most common skin changes included hypopigmentation (81%), vulvar fissures (72%), and labial resorption (60%), with fissures affecting the individual the most (48%). Sixty-six percent of the respondents initially received an alternative diagnosis, most commonly vulvovaginal yeast infection (49%). Hypothyroidism was the most common concurrent autoimmune condition (10%). Vulvar LS affects premenopausal women, commonly presenting with dyspareunia and tearing with intercourse. This condition should be considered and evaluated in premenopausal women presenting with vulvar symptoms and sexual pain.

Risk of Development of Vulvar Cancer in Women With Lichen Sclerosus or Lichen Planus: A Systematic Review.

Vulvar lichen sclerosus (VLS) and possibly vulvar lichen planus (VLP) are associated with an increased vulvar cancer (VC) risk. We analyzed the risk of VC and its precursors after a diagnosis of VLS or VLP. A search was performed to identify articles describing the development of vulvar neoplasia in women with VLS or VLP. This systematic review was registered with the PROSPERO database. Fourteen studies on VLS included 14,030 women without a history of vulvar neoplasia. Vulvar cancer, differentiated vulvar intraepithelial neoplasia (dVIN), and vulvar high-grade squamous intraepithelial lesion occurred in 2.2% (314/14,030), 1.2% (50/4,175), and 0.4% (2/460), respectively. Considering women with previous or current VC, the rate was 4.0% (580/14,372). In one study, dVIN preceded VC in 52.0% of the cases. Progression of dVIN to VC was 18.1% (2/11).The risk was significantly higher in the first 1-3 years after a biopsy of VLS and with advancing age; it significantly decreased with ultrapotent topical steroid use.For the 14,268 women with VLP (8 studies), the rates of VC, dVIN, and vulvar high-grade squamous intraepithelial lesion were 0.3% (38/14,268), 2.5% (17/689), and 1.4% (10/711), respectively. Vulvar lichen sclerosus is associated with an increased risk of VC, especially in the presence of dVIN and with advancing age. Ultrapotent topical steroids seem to reduce this risk. An increased risk of developing VC has been suggested for VLP. Hence, treatment and regular life-long follow-up should be offered to women with VLS or VLP.

Vulvar lichen sclerosus in children: A prospective study over a 3 year period

Context: Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease affecting prepubertal girls and postmenopausal women. In prepubertal girls, it causes anxiety in parents and affects quality of life considerably. There is a paucity of data about VLS in children from India. Aims: We aimed to study the demographic and clinical profile of VLS in children. Settings and Design: It is a prospective study over a period of 3 years from October 2017 to September 2020. Materials and Methods: All the children up to the age of 15 years with VLS, attending to our outpatient department between October 2017 and September 2019, were enrolled in the study after taking informed consent from the parents/guardian. The age, duration, clinical features, and comorbidities were noted. Biopsy was done only in clinically doubtful cases. All the cases were treated with topical clobetasol 0.05% ointment once daily for 3 months, and tacrolimus 0.03% or pimecrolimus 1% ointment was given as maintenance therapy. Cases were followed up for a period of 6 months–3 years. Results: Twenty-five girls presented with VLS during the study period. The mean age was 8.44 years, and the mean duration at presentation was 1.14 years. Pruritus was present in 14 (56%); constipation and dysuria were noted in 2 each. Classical figure-of-eight appearance was observed in only 4 cases; atrophy and erythema were noted in 11 cases and 3 cases, respectively. All symptoms improved in 3–4 weeks after treatment and depigmentation completely subsided in 8 cases by 3 months. Limitations: The diagnosis of VLS was made clinically in most cases. Dermoscopy was not done. Biopsy was done in only clinically doubtful cases. Conclusions: VLS in children needs special attention as it causes anxiety in parents thinking it as vitiligo. Pruritus is the common symptom. Ultrapotent topical steroid helps in the management. It may not resolve completely at puberty.

Новый метод мультимодальной оптической когерентной томографии для диагностики склеротического лихена вульвы

Новый метод мультимодальной оптической когерентной томографии для диагностики склеротического лихена вульвы

Psycho-emotional profile of patients with vulvar lichen sclerosus

Aim: to assess the psycho-emotional profile in patients with vulvar lichen sclerosus.Materials and methods. We examined 57 patients with a diagnosis of vulvar lichen sclerosus, the average age of 35.0 ± 0.6 (from 18 to 45) years. The control group included 45 healthy women without dystrophic changes in the external genital organs, who applied for a routine examination. The following methods were used to analyze the psycho-emotional profile: WAM questionnaire (well-being, activity, mood), Hospital Anxiety and Depression Scale (HADS), the Dermatology Life Quality Index (DLQI).Results. In patients with vulvar lichen sclerosus a decrease in indicators “well-being” (24.3 ± 1.7 versus 52.4 ± 5.6 scores) and “mood” (28.7 ± 3.6 versus 58.1 ± 6.1 scores) was revealed; “activity” was changed slightly (41.2 ± 3.2 against 47.4 ± 4.9 scores). Depressive (75.4 % versus 15.6 %) and anxiety (68.4 % versus 20 %) disorders were also more common in this group. Significant deterioration in the quality of life in patients was confirmed by a DIQL (18.79 ± 4.98 scores).Conclusion. Psycho-emotional profile of patients with vulvar lichen sclerosus is characterized by a change in most of the analyzed parameters. The presence of anxiety-depressive disorders can aggravate both the general somatic state and the course of the underlying disease. So activities aimed at improving the quality of life should be included in the rehabilitation of these patients.

Vulvar Lichen Sclerosus: Outcomes Important to Patients in Assessing Disease Severity.

The aim of the study was to determine outcome measures that women with vulvar lichen sclerosus (LS) rate as important in assessing disease severity with the ultimate goal of including these items in a disease severity rating tool. An online survey of women older than 18 years with a diagnosis of vulvar LS was performed. The survey was posted in Facebook LS support groups. Participants rated items on a scale from 1 to 5 (not important to include to essential to include) in a disease severity scale. Participants also rated how often they were affected by various symptoms on a scale from 1 to 5 (never to daily). Mean rating of importance and mean rating of frequency for each sign and symptom were calculated. T tests were used to compare patients with biopsy-proven disease with those with a clinical diagnosis of LS. Nine hundred fifty-eight participants completed the survey (86% completion rate). Patients felt that the most important items to assess disease severity were irritation (4.39), fusion of the labia (4.38), soreness (4.37), itch (4.34), change in vulvar skin (4.34), and decrease in quality of life (4.33). The most frequently experienced items by those with LS were irritation (3.92), changes in appearance of vulvar skin (3.92), and discomfort (3.89). There were no differences between patients with biopsy-proven LS versus those diagnosed on clinical examination. Future LS severity assessment tools will need to include a combination of patient-rated symptoms, clinical rated signs and anatomical changes, and quality of life measures.

Comorbid Vulvar Lichen Planus and Lichen Sclerosus.

The aims of the study are to assess the histopathologic characteristics of vulvar biopsies consistent with lichen planus (LP) in women with a previous or concurrent histopathologic diagnosis of vulvar lichen sclerosus (LS) and to describe the clinical features of comorbid LP and LS. Patients were included if a diagnosis of LP was confirmed after review of the hematoxylin and eosin slides and the histopathology reporting LS noted a band of abnormal collagen. Data were collected on anatomic site, clinical appearance, histopathology, microbiology, treatment, and follow-up. There were 31 cases with a mean age of 69.5 years. Thirty specimens showed erosive LP, of which 22 were from inner labium minus and 8 from vestibule. There were no significant differences between biopsy site in epithelial thickness, erosion, lymphocytic infiltrate, or basal layer pattern. One third of cases showed a regenerative pattern of LP. Of the 26 patients with clinical records available, erythema at the biopsy site was noted in all cases; in 23 the notes specified central erythema and peripheral pallor. Forty-six percent were prescribed topical corticosteroids before biopsy. All 26 were treated with topical corticosteroids, 23% were prescribed antimycotics, and 38% required other supplemental therapies. Comorbid vulvar LP and LS are not rare; clinicians suspecting one should evaluate for the other and consider separate biopsies of morphologically distinct areas. Clinicopathological correlation is an invaluable tool in assessing biopsies when both diagnoses are suspected, because the regenerative pattern of LP may otherwise be overlooked or misdiagnosed.

Tubo-Ovarian Abscess in a Virginal Adolescent With Labial Agglutination due to Lichen Sclerosus

Lichen sclerosus is a chronic inflammatory disease affecting primarily the anogenital region and may cause symptomatic labial agglutination. We describe an unusual presentation of this condition in a postmenarchal, virginal girl. A 14 year-old girl presented to the Emergency Department of a tertiary children's hospital with acute urinary retention. A perineal inspection revealed labial fusion starting anteriorly and covering up almost two third of the introitus (Figure 1A). Because she was able to void during the assessment period in the Emergency Department, her labial adhesions were managed conservatively with topical estrogen and sitz baths. Ten days after initial presentation, she developed acute onset abdominal pain with fever. Abdominal ultrasound and magnetic resonance imaging revealed tubo-ovarian abscesses mainly in the right ovary and both fallopian tubes (Figure 2A and 2B). The patient was admitted to the hospital and started on intravenous antibiotherapy according to recommendations of infectious diseases specialists. Since adequate clinical response was not achieved with optimal medical treatment, drainage of bilateral tubo-ovarian abscesses was done by the interventional radiology under general anesthesia. In the same session, a simple division of the labial agglutination and vulvar biopsies were performed by the pediatric and adolescent gynecology team with a preliminary diagnosis of vulvar lichen sclerosus based on the clinical features of the patient (Figure 1B). Pathology, although not conclusive, supported the clinical impression. During her inpatient follow up, a second imaging-guided abscess drainage was required, four days after the first procedure, due to insufficient clinical improvement and residual right tubo-ovarian abscess detected on repeated abdominal magnetic resonance. After 3 weeks of intravenous antibiotherapy, the patient was discharged with oral antibiotics and clobetasol propionate 0.05% ointment for local treatment of the lichen sclerosus. The outpatient follow up has gone uneventfully and no recurrence of labial agglutination has been observed. The proposed mechanism for this rare complication of vulvar lichen sclerosus is vaginal pooling of urine due to significant labial agglutination, leading to ascending infection, pelvic inflammatory disease and tubo-ovarian abscesses. In conclusion, vulvar lichen sclerosus should be considered in cases of labial agglutination with atypical findings such as older age and anterior location. Finally, this rare presentation of vulvar lichen sclerosus reminds us that pelvic inflammatory disease and tubo-ovarian abscess must be kept in mind in the presence of fever, abdominal pain and adnexal mass, even in virginal adolescents. The existence of obstructive lesions of the genital tract should further increase the suspicion for these infections.Figure 2A and B. Pelvic MRI showing the tubo-ovarian abscessesView Large Image Figure ViewerDownload Hi-res image Download (PPT)

Vulvar Lichen Sclerosus and Neoplastic Transformation: A Retrospective Study of 976 Cases.

The aim of the study was to estimate the neoplastic potential of vulvar lichen sclerosus (VLS). This was a retrospective study of 976 women with VLS. We recorded age at diagnosis of VLS, length of follow-up, and type of neoplasia, categorized as the following: (1) vulvar intraepithelial neoplasia (VIN), further subdivided in differentiated VIN (dVIN) and high-grade squamous intraepithelial lesion; (2) superficially invasive squamous cell carcinoma; and (3) frankly invasive squamous cell carcinoma. Neoplasia incidence risk, neoplasia incidence rate, and cumulative probability of progression to neoplasia according to the Kaplan-Meier method were estimated. Log-rank test was used to compare the progression-free survival curves by age at diagnosis of VLS. The mean age at diagnosis of VLS was 60 (median = 60; range = 8-91) years. The mean length of follow-up was 52 (median = 21; range = 1-331) months. The following 34 patients developed a neoplasia: 8 VIN (4 dVIN, 4 high-grade squamous intraepithelial lesions), 6 keratinizing superficially invasive squamous cell carcinoma (5 with adjacent dVIN), and 20 keratinizing invasive squamous cell carcinoma (1 with adjacent dVIN). The neoplasia incidence risk was 3.5%. The neoplasia incidence rate was 8.1 per 1,000 person-years. The cumulative probability of progression to neoplasia increased from 1.2% at 24 months to 36.8% at 300 months. The median progression-free survival was significantly shorter in older women (≥70 years) when compared with that in younger women (p = .003). Vulvar lichen sclerosus has a nonnegligible risk of neoplastic transformation and requires a careful and lifelong follow-up in all patients, particularly in elderly women. Early clinical and histological detection of preinvasive lesions is essential to reduce the risk of vulvar cancer.

Lichen scléro-arophique vulvaire chez l’enfant : diagnostic différentiel d’abus sexuel

With the frequency of lawsuits for sexual abuse increasing, high-quality care for victims is of utmost importance. Genital examination is difficult to perform, especially on children, but is a key item for diagnosis. A case report is presented herein to illustrate this issue. A sexual assault was suspected on 2.5-year-old female twins. Clinical examination primarily detected vulvar micro-hematoma and hypopigmentation, discreetly sclerosing, as well as atrophic labia and a beginning of lichenification on one of them. The other twin showed an unspecific and erythematous lesion. Elimination of the supposedly dangerous environment and a remote consultation brought out a paradoxical development to the story and confirmed the diagnosis of vulvar lichen sclerosus. Traumatic lesions caused by sexual abuse are uncommon. Coexisting allegations and lesions in the genital area lend support to the hypothesis of sexual abuse. However, genital-anal tropism dermatosis can mimic traumatic injuries. Lichen sclerosus is an example of a differential diagnosis of vulvar traumatic lesions, which is quite often unknown to physicians, including pediatricians. This case report shows that taking the appropriate time in multidisciplinary councils can suggest a differential diagnosis. Nonetheless, it should be remembered that identifying the pathology behind the lesions cannot allow the physician to negate the child's story about potentially sexual acts, although the truth of this story and the perpetrator must still be proven by a court of law.

Does treatment of vulvar lichen sclerosus influence its prognosis?

To record the clinical features, symptomatic response to topical steroids, and resolution of clinical signs in a large cohort of female patients with vulvar lichen sclerosus. Descriptive cohort study with a mean follow-up of 66 months. The vulvar clinics of a teaching hospital and of a district general hospital in Oxfordshire, England. Three hundred twenty-seven patients (74 girls and 253 women) with a definite clinical diagnosis of vulvar lichen sclerosus. Interventions The patients received topical steroids as part of their normal care. Symptomatic response to treatment (good, partial, or poor); response of the vulvar signs (total, partial, minor, or poor); and the presence or absence of moderate or severe scarring. The mean age at onset was 5.4 years in girls and 55.1 years in women and first-choice therapy was an ultrapotent topical steroid for 50% of the girls and 89% of the women. Response to treatment was recorded in 255 patients. In 244 patients (96%) symptoms improved with treatment, as 168 (66%) became symptom free and 76 (30%) showed partial response; 11 (4%) had poor response. Among the 253 patients in whom a response of the vulvar signs to topical steroid was recorded, 58 (23%) showed total response, with return to normal skin texture and color; and 173 (68%) showed partial, 18 (7%) showed minor, and 4 (2%) showed poor response. Moderate or severe scarring occurred less often in girls (P<.001). Squamous cell carcinoma developed in 6 women (2.4%). Topical ultrapotent steroid is an effective treatment for vulvar lichen sclerosus, giving relief of symptoms in most and completely reversing the skin changes in approximately one fifth of patients.