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Introduction and methods Different clinical and histological variants of lichen planus (LP) exist, such as lichen planopilaris, pigmentosus, linear, or atrophic LP. Recently, some cases came to our attention of hyperpigmented and atrophic linear lesions of the face with lichenoid histology, suggesting a combination of these different variants. We carried out a single-center, retrospective descriptive study of 6 similar cases selected from our database and compared them with a literature review. Results There were 4 males and 2 females of mean age 42 years. Each had linear lesions located on one side of the face. All lesions were initially itchy; they appeared hyperpigmented in all patients and atrophic in 5 cases. Biopsies indicated lichen planopilaris in 5 patients, with deep peri-eccrine involvement in 4 of them. Only 2 of the 6 patients had extra-facial lesions. Discussion and literature review We found 24 cases in the literature having similar clinical and histological aspects. Men aged around 37 years seemed particularly affected. An atrophic course was noticed in 10 patients. Such a clinicopathological picture may suggest differential diagnoses like lichen striatus, lupus erythematosus, lichen sclerosus atrophicus, or Moulin's linear atrophoderma. Early histopathological examination could be of precious assistance in allowing the initiation of effective treatment immediately as of the initial inflammatory phase, thereby limiting the risk of cosmetic sequelae such as atrophy or residual pigmentation. Conclusion We describe a form of facial lichen planus that is highly particular in terms of its follicular tropism, its blaschkoid distribution, its pigmented character, and its atrophic progression.

Vulvar dermatoses are often debilitating chronic skin conditions associated with pain and pruritus. In oncologic patients, cancer treatments can precipitate and exacerbate vulvar dermatoses. Cytotoxic chemotherapy, hormonal therapies, and local pelvic radiation therapy can lead to vulvar symptoms, and cancer treatment-induced vulvar conditions include graft-versus-host-disease and radiation dermatitis. There have also been reports of lichen sclerosus development or exacerbation secondary to hormonal therapy and immune checkpoint inhibitors, attributed to proposed hormonal and immunologic pathogenesis of lichen sclerosus. Early recognition and treatment of these conditions can significantly improve quality of life. In this review, we summarize the clinical features and management characteristics of six types of common vulvar dermatoses that may present in the oncologic patient.

Vulvar squamous cell carcinoma arises from precursor lesions: vulvar intraepithelial neoplasias (VIN). Most of them are caused by the human papillomavirus (HPV), and the precursor lesion for this group is VIN usual type/high grade squamous intraepithelial lesion (uVIN/HSIL) of variable clinical presentation and having a light invasive potential. Some VIN are HPV-independent and arise in older women against the background of chronic dermatoses, mostly lichen sclerosus. Their histological diagnosis is more subtle. They have a higher invasive potential. A third precursor, leading to well-differentiated, or even verrucous, carcinomas, is still ill-defined to this day. We detail these lesions' clinics, histology, and biomarkers (immunohistochemical and molecular).

Vulvar Verrucous Carcinoma (VVC) is a rare lesion, with few described cases. It has low metastatic potential with high morbidity due to the necessity of extensive resections, although. Previously, VVC was considered a synonym to the Buschke-Lowenstein Tumor (BLT) or Giant Condyloma Acuminatum (GCA). Lichen Sclerosus (LS) is associated with Vulvar Intraepithelial Neoplasia (VIN) and Vulvar Squamous-cell carcinoma (SCC); association with VVC is also described. The case of a 60-year-old menopausal woman is reported; she had chronic itching and an extensive verrucous lesion in vulva, initially diagnosed and treated as condyloma acuminatum; there was recurrence as verrucous carcinoma associated to LS. Excision with margins was performed and clobetasol and imiquimod were used. Patient had complete remission with no further recurrences. Distinction between VVC and BLT can be difficult; current literature considers them different entities. Human papillomavirus (HPV) infection and the presence of LS play a controversial role in these injuries.

BACKGROUND: Lichen sclerosus (LS) affects the female anogenital area, causing anatomical changes. Reported symptoms include itching, soreness and dyspareunia.AIM: To evaluate the quality of life and sexual functioning in women with LS.METHODS: In total, 158 women aged over 18 years, diagnosed with LS, and referred to North Denmark Regional Hospital from January 2018 to November 2019, were included. Three questionnaires, the Female Sexual Function Index (FSFI), Dermatology Life Quality Index (DLQI) and the World Health Organization Five-Item Well-Being Index (WHO-5), were completed.RESULTS: The women (mean age 47 years, range 18-76) presented a low score on all FSFI scales, with a mean score of 13.83 (95% CI 12.46-15.20), indicating reduced sexual functioning. The subgroup evaluation scored for desire 2.32, arousal 2.23, lubrication 2.39, orgasm 2.28, satisfaction 3.02 and pain 1.59. The DLQI results revealed a mean score of 7.88 (95% CI 7.02-8.74), indicating a moderate effect on the women's everyday life. The mean subscores were treatment 0.32, sexual difficulties 1.56, relations 1.02, work/study 0.34, sport 0.45, social activities 0.54, clothing 0.89, shopping 0.22, embarrassment 0.99 and itching, soreness and pain 1.55. The mean score for the WHO-5 was 56.66 (95% CI 53.48-59.84) indicating that 40% of the women had signs of depression.CONCLUSION: LS has a considerable influence on the sexual functioning and quality of life of women. Healthcare professionals must not only consider the biological aspects but also the psychological and social aspects.

To assess long-term outcomes of lichen sclerosus (LS) in the female pediatric population, specifically in relation to patient age, treatment type and duration, and remission. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Retrospective chart review was conducted to identify female pediatric patients (0-18 years of age) who were diagnosed with LS between January 1, 2015 and January 1, 2020 at the University of North Carolina Dermatology and/or Obstetrics and Gynecology Departments. Patients were contacted via telephone for follow-up interviews consisting of a series of questions regarding patient age, symptom onset, time of diagnosis, treatment, and current symptoms.Of the 128 patients identified, 61 patients consented and participated in follow-up interviews. At the time of study follow-up, 55/61 (90%) of participants reported their symptoms were improved. Patients reported using a variety of treatments, with medium- to high-potency topical steroids being the most common. At the time of follow-up, 53/61 (87%) of patients reported being asymptomatic, 37/53 (70%) of whom were not using any form of maintenance therapy. Those who achieved symptom resolution did so at an average of 8.4 years of age. There was no significant difference in age in asymptomatic patients receiving maintenance therapy and those receiving no maintenance therapy. There was a positive correlation for the duration of LS treatment and time in remission (P.001). Increased patient age at time of follow-up also correlated positively with time in remission (P.001).In our cohort, the need for continued maintenance therapy was not correlated with age or, by proxy, pubertal status. Thus, LS remission might be determined more by early and successful pharmacological interventions.

Performing 1-stage urethroplasty in patients with urethral strictures caused by lichen sclerosus (LS) is hotly debated among reconstructive urologists due to conflicting reports of success. Therefore, the objective of this study was to determine the pooled incidence of stricture recurrence following 1-stage buccal mucosal graft (BMG) urethroplasty in patients with LS, to determine the impact of surgical technique on recurrence and to compare recurrence risk between patients with and without LS after 1-stage repairs.A systematic review was conducted in accordance with PRISMA criteria. The primary outcome was pooled incidence of recurrence, which was calculated using a Der-Simonian-Laird binary random effects model with a Freeman-Tukey arcsine transformation. A total of 21 studies were included, of which 15 provided data for comparative analyses.Pooled data from 625 LS patients revealed a stricture recurrence rate of 10% (95% CI 6-14). Among studies with longer followup (≥24 months), this increased to 18%. Among patients with penile urethral involvement, studies utilizing a penile skin incision had significantly higher pooled recurrence rates than those utilizing penile invagination (p=0.004). Across all studies, there was no evidence to suggest a difference in pooled recurrence rate between patients with and without LS (p=0.36). However, across only long-term studies, recurrence risk was significantly higher for patients with LS (OR 1.83, p=0.05).One-stage BMG urethroplasty is likely a viable surgical option for patients with LS-related strictures; however, high-quality data are limited. Future multi-institutional, long-term prospective studies are needed to assess durability of 1-stage repair.

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.

Aim to evaluate the correctness of the diagnosis of lichen sclerosus with an evaluation of complications in the form of urethral strictures and to ensure long-term monitoring of patient management in order to avoid recurrence of fibrous complications and ascending infection in case of lower urinary tract obstruction. Material and methods a total of 55 men aged from 17 to 85 years with lichen sclerosus complicated by urethral stricture who were treated from 2005 to 2020 were included in the study. All patients underwent a comprehensive urological examination. From them, 18 (32.7%) patients had previously undergone various types of urethral procedures and were admitted with recurrent urethral stricture for repeated surgery. Results an examination of patients with lichen sclerosus complicated by urethral strictures is presented, including a morphological assessment of the resected tissue and analysis of etiopathogenetic factors. In addition, the results of complex treatment of patients with urethral strictures associated with sclerosus were evaluated. An increase in the maximum urine flow rate and a decrease in post-void residual volume by almost three times was noted 1-3 months after urethroplasty. After 6 months, a recovery of an adequate micturition and complete absence of residual urine were revealed. The morphological picture in patients with urethral strictures associated with lichen sclerosus is characterized by focal atrophy of the epidermis, replacement of the columnar epithelium with a stratified epithelium with signs of hyperkeratosis and acanthosis, histiolymphocytic infiltration, and the formation of microcalcifications under the basement membrane. Conclusion the correctness of the diagnosis verification and the choice of the treatment strategy in patients with urethral strictures caused by lichen sclerosus significantly contributes to the favorable outcomes. Based on comparative analysis, we can conclude than complex approach to the treatment of patients with urethral strictures associated with lichen sclerosus is highly effective.

Neuropathic pruritus is defined as an itch arising from diseases of the central or peripheral nervous system, including nerve damage, compression, or irritation. It can be generalized or localized and is encompassed within a broad range of diagnoses, including notalgia paresthetica, brachioradialis pruritus, certain forms of vulvar itch, and small fiber neuropathy, amongst others.1 Brachioradial pruritus is a well-known type of neuropathic itch that is believed to result from an insult to cervical nerves C5-C8 from degenerative disc disease or trauma.2 Symptoms include itch, burning, numbness, and tingling affecting the dorsolateral aspects of the upper extremities. Although most cases of vulvar itch can be attributed to infectious causes or inflammatory dermatoses, such as psoriasis, lichen simplex chronicus, lichen sclerosus, or contact dermatitis, some cases of chronic vulvar itch can be categorized under the umbrella of genitofemoral neuralgia or vulvodynia.3 Classically regarded as a type of neuropathic pain, itch and burning can accompany vulvodynia in 20% and 70% of cases, respectively,4 and are similarly addressed in the same way with medications targeting neurogenic pathways. In many cases of localized neuropathic itch, treatment can be challenging because topical therapies are limited and systemic treatments may provoke many side effects. Treatment options for neuropathic itch typically include topical local anesthesia, capsaicin formulations, calcineurin inhibitors, menthol or camphor, and systemic approaches with gabapentinoids, selective serotonin reuptake inhibitors, and tricyclic antidepressants.5 We report 3 cases of localized neuropathic itch successfully treated by dronabinol, an oral synthetic formulation of delta-9-tetrahydrocannabinol.

The benefits and harms of the available types of surgical management for lichen sclerosus-related (LS) strictures remain unclear and, thus, clear and robust clinical practice recommendations cannot be given. To assess the role of single-stage OMGU in the management of LS strictures and explore how its benefits and harms compare with the alternative management options. Medline, Embase and Cochrane controlled trial databases (CENTRAL, CDSR) were systematically searched. Randomized (RCTs) and nonrandomized studies (NRCSs) comparing single-stage OMGU with other surgical management options for LS strictures and single-arm studies on single-stage OMGU were included. Risk of bias (RoB) was assessed. Of the 1912 abstracts identified, 15 studies (1 NRCS and 14 single-arm studies) were included, recruiting in total 649 patients. All studies were at high RoB. In the only NRCS available, stricture-free rate (SFR) for single-stage and staged OMGU was 88% vs 60%, respectively (p = 0.05), at a mean follow-up of 66.5 months. SFR range for single-stage OMGU in single-arm studies was 65–100% (mean/median follow-up, 12–59 months). Single-stage OMGU had low complication rates and beneficial impact on LUTS and QoL. The present SR highlights the methodological limitations of the available literature. In the absence of adverse local tissue conditions, and taking into consideration benefit–harm balance and surgeon’s skills and expertise, single-stage OMGU can be justified in patients with LS strictures.

Lichen sclerosus of the vulva (LSV) is seen frequently enough to warrant knowing how to diagnose it and institute appropriate treatment strategies. LSV is a chronic skin disorder, very likely of autoimmune origin, which may affect various areas of the perineum, although some women may be affected in extragenital areas. The disease has significant adverse impact on quality of life and sexual activity and may undergo malignant transformation. History of symptomatology and clinical examination is sufficient to make the diagnosis. Skin biopsy is only necessary in specific scenarios. Topical corticosteroids are still the mainstay of therapy, initially to institute remission and then for maintenance. Long-term therapy and surveillance are invariably necessary. Surgery does have a role, but only in specific associated conditions. A number of alternative treatment options have been mooted, especially if the disease is resistant to topical corticosteroids, but these options are still being assessed and studied.

The aim of this study is to describe the outcomes for a modified glans-resurfacing technique for benign and malignant penile conditions in which the uninvolved glans corona is preserved in order to maintain glans erogenous sensation. A total of 13 patients underwent coronal-sparing glans resurfacing (CSGR), with follow-up every 3 months for ≥2 years. Positive surgical margin and local recurrence (LR) rates were evaluated. Surgical complications and cosmetic outcomes were also recorded. Patients were asked to complete the International Index of Erectile Function-5 (IIEF-5) questionnaire starting 12 months after the surgery. The median (interquartile range [IQR]) age and follow-up periods were 63 (53-68) years and 29 (14-38) months, respectively. Eight patients were diagnosed with primary penile squamous cell carcinoma (SCC), three had refractory lichen sclerosus, and two had penile intraepithelial neoplasia (PeIN). No surgical complications were recorded. All patients had a complete graft take and reported satisfactory cosmetic results with preserved erogenous sensation. Two cancer patients developed LR which was managed with further penile preserving surgery. The median (IQR) postoperative IIEF-5 value was 20 (17-23). This modified coronal-sparing technique was suitable for glans lesions that spare the coronal ridge and coronal sulcus. Preservation of the coronal ridge helps maintain sexual function and provides excellent cosmetic outcomes.

Lichen sclerosus (LS) is a chronic inflammatory disease that is 6-10 times more common in women than in men. The drug affects between 1 in 1000 and 1 in 300 people in the general population, but the exact prevalence is unknown due to the fact that the disease is often asymptomatic. The article presents modern ideas about lichen sclerosus. Issues of various etiological theories of origin and pathogenesis are considered. The description of the clinical picture of lichen sclerosus is given. Diagnostic approaches are described, including the histological picture. Possible complications, including anatomical abnormalities of the vulvar architectonics and the risk of malignant transformation, are discussed in detail. Various methods of treatment and care for this disease, local treatment schemes are presented, an overview of systemic drugs and surgical operations, as well as further observation and management of patients is given.

Objective The aim of the study was to identify whether erosive lichen sclerosus (LS) is a distinct clinicopathologic subtype. Materials and methods The pathology database was searched for "erosion," "erosive," "ulcer," and "lichen sclerosus." Inclusion criteria were histopathologic diagnosis of LS and erosion or ulcer overlying a band of hyalinization and/or fibrosis. Exclusions were concurrent neoplasia and insufficient tissue. Histopathologic review documented site, epithelial thickness, adjacent epidermal characteristics, infiltrate, and dermal collagen abnormality. Clinical data included demographics, comorbidities, examination findings, microbiologic results, treatment, and response. Results Ten examples of erosive LS and 15 of ulcerated LS occurred in 24 women with a mean age of 67 years. Ulcerated LS was associated with diabetes and nontreatment at time of biopsy. Clinicians identified red patches in all but 1 case of erosive LS. Ulcerated LS was documented as fissure, ulcer, or white plaque, with 8 (53%) described as lichenified LS with epidermal breaches. Erosive LS favored hairless skin with normal adjacent stratum corneum sloping gently into erosion, whereas most ulcers in LS had an abrupt slope from hair-bearing skin. All cases were treated with topical steroids; 2 patients with erosive LS and 10 with ulcerated LS also had oral antifungals, topical estrogen, antibiotics, and/or lesional excision. Treatment yielded complete resolution in 50%. Conclusions Erosive LS is an unusual clinicopathologic subtype characterized by red patches on hairless skin seen microscopically as eroded epithelium overlying a band of hyalinized or fibrotic collagen. In contrast, ulcerated LS is usually a traumatic secondary effect in an uncontrolled dermatosis.

Vulvar intraepithelial lesions are a heterogenic group of diseases, which can be easily misdiagnosed. The case of a 61-year-old woman with a history of genital intraepithelial lesions and infection with HPV is presented. Her main complaint was vulvar pruritus. Vulvoscopy revealed the presence of two skin lesions: the first one had the morphology of lichen sclerosus, and the second of a Bowenoid lesion. The biopsy of the first lesion revealed vulvar intraepithelial neoplasia, whereas cells of squamous vulvar cancer were identified in the second lesion. After staging, the patient was advised to undergo hemivulvectomy and lymphadenectomy. The coexistence of morphologically diverse vulvar skin lesions may cause difficulties with diagnosis and the selection of an adequate treatment. Long-term follow-up and regular examination are essential for diagnosis of vulvar malignancies in the early stage.

Oral submucous fibrosis (OSF) is a precancerous condition of the oral cavity associated with habitual chewing of quid, with a high incidence among populations of the Indian subcontinent and Southeast Asia. Clinically, its initial manifestation may mimic oral lichen planus or lichen sclerosus. If the habit is not halted, the mucosa gets leathery and thickened, and fibrous bands form causing significant morbidity. Microscopically, it is characterized by atrophic epithelium, loss of rete ridges, and hyalinization of lamina propria. Of note, these hallmark histopathological features may be overlooked in the unusual presence of lichenoid interface changes, which may lead to the wrong diagnosis. We present herein five cases in which the rare joint appearance of OSF and lichenoid reaction features posed a diagnostic challenge. Due to its progressive nature and malignant potential, the presence of oral lichenoid changes overlying submucous hyalinization, in the right clinical and demographic setting, should raise suspicion of OSF and prompt actions directed at quid-chewing discontinuation.

The interface dermatitides encompass a vast array of cutaneous entities which, at times, may present with particular clinical variants with adnexal predilection. Similarly, hair follicle and eccrine gland involvement of some of these entities has been observed on histopathology. This review aims to describe the various adnexotropic presentations of the interface dermatitides. Recognizing that the adnexa can be a frequent site of involvement of these conditions may aid dermatopathologists in making the correct diagnosis and avoid misinterpreting adnexotropism for other conditions such as the great imitator, mycosis fungoides.

Mucous membrane pemphigoid (MMP) is a rare chronic immunobullous disease that involves the mucous membranes and may result in significant scarring and complications if diagnosis is delayed. MMP typically occurs in elderly patients, with very few cases reported in children. Here, we present a 12-year-old female patient with childhood-onset oral and genital MMP, clinically suspected to be lichen sclerosus, but eventually diagnosed as MMP after multiple supportive biopsies and confirmatory direct immunofluorescence. Although treatment was challenging, the combined use of systemic corticosteroids, dapsone, and mycophenolate mofetil was ultimately successful in achieving disease control.

Background Whilst there is a trend away from aggressive nonorgan sparing surgical treatments for malignant penile disease, a variety of penile preservation options exist but functional outcomes and patient reported outcomes (PROs) in this area are poorly reported to date. Aim The aim of this study is to report functional outcomes and PROs of total glans resurfacing (TGR) in a consecutive series of patients with lichen sclerosis (LS) or localized penile cancer (PC). Methods From 2004 to 2018 a consecutive series of patients underwent TGR for the management of LS or localized PC in a tertiary referral network. Patient clinical records and operative notes were retrospectively reviewed. Statistical analysis was conducted with Stata 12. Outcomes Urinary and sexual outcomes were recorded utilizing both the International Index of Erectile Function (IIEF) and International Prostate Symptom Score (IPSS) validated questionnaires while PROs were extrapolated from a 5-item “ad hoc” telephone questionnaire administered at 1 year post procedure. RESULTS 37 consecutive patients were enrolled. Histology results demonstrated LS in 16 patients, with the remaining 21 having a diagnosis of PC. The most common reasons for patient presentation were local pain (32.4%), pruritus (37.8%) and bleeding (29.7%). Median follow-up was 22 (IQR 13–77) months. Median age was 62 (IQR 55–68). Neither of the questionnaires assessing urinary and sexual function showed any significant deterioration after surgery. Glans sensitivity was fully maintained in 89.2% of cases. 94.5% of patients reported to be fully satisfied with the aesthetic appearance of the penis and would consider undergoing the same procedure again if necessary. 91.9% of patients would recommend the same procedure to someone else. An overall improvement of the quality of life was reported by 86.4% of patients. Clinical Implications TGR should be considered a treatment of choice for selected cases of benign or malignant penile lesions Strengths and Limitations Our study has some limitations, the first being its retrospective nature. Furthermore, despite being one of the largest series to date, follow-up duration is somewhat limited and a control group is lacking. CONCLUSION TGR represents an excellent surgical option ensuring satisfactory voiding and sexual function, as well as cosmesis for selected cases of penile lesions.

In 2008 a vulval clinic was established at the University Clinic of Schleswig Holstein, Campus Luebeck, Department of Dermatology. A total of 1227 patients were referred to the clinic between 2008 and October 2020, including 91 children (age range 1-13 years) and 17 adolescents (age range 14-17 years). The most common paediatric vulval conditions encountered were lichen sclerosus (33%), vulvitis (23%) and vulval psoriasis (7%). Quality of life was measured in 81 children using the paediatric version of the Dermatology Life Quality Index (DLQI). Of a maximum 30 points, the mean score was 7.2, confirming the association between vulval diseases and impaired quality of life in children and adolescents.

Aim: to comparatively assess sexual function and intensity of vulvovaginal symptoms in patients with vulvar lichen sclerosus and mixed vulvar dystrophy. Materials and Methods. There were examined 57 patients with vulvar lichen sclerosus and 63 patients with mixed vulvar dystrophy, with mean patient age 35.0 ± 0.6 (18–45) years. Prior to therapy, the study participants completed the Female Sexual Function Index (FSFI) and the Vulvovaginal Symptoms Questionnaire (VSQ). Results. Severe sexual dysfunction (FSFI score 2) was detected in 14 % of cases (n = 8) in group 1 (sclerotic lichen vulva) and 17 % (n = 11) in group 2 (mixed dystrophy). Remaining respondents had total score below the normal cut-off. The mean FSFI score for group 1 and 2 was 17.68 and 16.78, respectively. VSQ testing found that most common complaint in both groups was itching (91 and 95 %, respectively). The majority of patients also noted a deteriorated emotional state and disease-related limitations in everyday life. The maximum VSQ score was 20 corresponding to the peak negative disease impact found in 23 % and 37 % patients in group 1 (n = 13) and group 2 (n = 23), respectively. Many patients also noted lack of lubrication most likely associated with discomfort and pain during intercourse, as well as worsened relationship with partner. Conclusion. The vulvar dystrophy negatively affects sexual function in young women. Symptoms typical to such conditions impose marked restrictions on intimate life, relationships and quality of general life. It accounts for why it is important to include questionnaires in the set of measures to assess sexual function and vulvovaginal symptoms both before and after treatment.

Objective: To review the short-term effects and safety of vulvovaginal fractional microablative CO2 laser therapy on atrophy symptoms using validated questionnaires pre- and posttreatment. Methods: This is a retrospective chart review from January, 2016 to December, 2019 on 139 women with vulvovaginal atrophy symptoms, who completed three treatments about 6 weeks apart. All were >18 years old and nonpregnant. As is the practice in our clinic for all women receiving treatment, they were surveyed prior to the 1st and 3rd treatments with validated questionnaires, Female Sexual Function Index (FSFI) and Vulvovaginal Symptoms Questionnaire (VSQ), as well as a visual analog scale (VAS). Paired t test was completed on the pre- and post FSFI and VAS scores. Pre- and postproportions of the VSQ were evaluated by the Fisher's exact test. Means were presented for each study variable. Multivariable regression analysis was completed on continuous and binomial variables for scores predictors. Results: Mean age was 62 years with a mean follow-up of 13.8 weeks. Concomitant topical estrogen was reported in 53% (n = 74). Breast cancer diagnosis was documented in 27% (n = 38), and lichen sclerosus in 22% (n = 31). All FSFI scores improved (pre: 12.7, post: 19.0, P < 0.001). The VSQ showed 18 of 21 questions significantly improved (P < 0.05). The VAS showed significant improvement in painful intercourse (pre: 6.6, post: 2.4, P < 0.001), and vulvar dryness (pre: 4.6, post: 1.5, P < 0.001). Posttreatments, 17 additional women became sexually active. No major adverse events were reported. Conclusions: Fractional CO2 laser may be effective and safe for the treatment vulvovaginal atrophy. Video Summary:http://links.lww.com/MENO/A700.

Precursor lesions of vulvar squamous cell carcinoma (VSCC) can be divided into two major biologic and prognostic groups: HPV-associated and HPV-independent VSCC. These two pathways are categorized as usual vulvar intraepithelial neoplasia (uVIN) with progression to basaloid or warty VSCC and differentiated vulvar intraepithelial neoplasia (dVIN) with progression to the more common keratinizing VSCC. While the HPV-dependent pathway to squamous cell carcinoma is well-understood, the development of squamous cell carcinoma from HPV-independent lesions is less clear. The majority of HPV-independent lesions fall into the dVIN category, and mutations in TP53 have been implicated as the driver behind their development. Other less common HPV-independent precursor lesions, termed differentiated exophytic vulvar intraepithelial lesion (DEVIL) and vulvar acanthosis with altered differentiation (VAAD), have also been characterized as precursors to keratinizing and verrucous VSCC. Inflammatory conditions of the vulva such as lichen sclerosus and lichen simplex chronicus also put patients at risk for developing VSCC. We herein evaluate the available evidence and biologic basis for these VSCC precursor lesions, among other speculated entities, and discuss their clinical, diagnostic, and prognostic features.

Lichen planus (LP) is a mucocutaneous immune-mediated disease of unknown etiology. It is more prevalent in women and usually occurs between the third and sixth decades of life. Oral lesions may or may not be associated with skin and genital lesions. Although the role of genetic factors is still undetermined, reports of LP in more than one family member are not uncommon. However, the occurrence of LP in monozygotic twins is rare. We report a rare case of 42-year-old female monozygotic twins presenting oral LP. This report is even rarer because one of the patients had cutaneous lesions of an unusual variant of LP (LP pigmentosus) and the other had an uncommon association with lichen sclerosus. The etiology and pathogenesis of LP are still uncertain. However, despite being rare, its occurrence in family members and monozygotic twins suggests that genetic factors are involved in its development.

Objective of the study.To evaluate the effectiveness of systemic interferon alpha‑2b in the complex therapy of lichen scleroatrophic of the penis.Material and methods. The study involved 26 male patients with a verified diagnosis: lichen scleroatrophic of the penis. The main group was 19 people, the comparison group was 7 men. The duration of the disease in men was from 9 to 12 months. To estimate the area of lesion as well as to estimate their dynamic changes were developed: Scleroatrophic Lichen Area Index (LS-S in men) and Scleroatrophic Lichen Activity Index (LS-A). Treatment in the main group: topical corticosteroid momentasone cream two times a day for 21 days, as well as interferon alpha‑2b suppositories in a dose of 3 million ME two times a day, daily for 10 days, then three times a week for 2 months with subsequent external application of 1–2 times a day for 2 months of ointment with interferon alpha‑2b. In patients with balanopostitis the cream was used in combination with momentazone, economazole, gentamycin and dexpanthenol. Patients from the comparison group received momentazone cream for 21 days, and in case of recurrence of symptoms – the prescription of topical calcineurin inhibitors. Patients of both groups received vitamin E of 200 mg per day for 2 months. The end point of observation for patients of both groups was 3 months after the end of therapy.Results. Against the background of treatment, improvement was achieved in all patients in the main group within 21 days. Subsequent cancellation of topical corticosteroid and continuation of therapy with suppositories and ointment of the interferon alpha‑2b allowed to maintain positive dynamics of the skin process during the whole period of observation. The control group also achieved a positive effect from therapy with topical corticosteroids (cream with momentazone), but after its cancellation, in the period from 4 to 6 weeks, symptoms were recurrent, which required the continuation of therapy with topical calcinerin inhibitors – tacrolimus ointment 0.1% 1–2 times a day for 4 weeks. The use of the LS-A Index showed that the indices of skin manifestations were the fastest to regress than those of the LS-S index assessing the area of lesion.Conclusions. 1. The use of system therapy with interferon alpha‑2b, along with topical GCSs, in the complex treatment can reduce the severity of clinical manifestations of GSAL without resorting to prolonged use of topical GCSs. 2. The use of interferon alpha‑2b in the complex treatment of the genital lichen sclerosus has demonstrated its effectiveness in a small group of patients. 3. Application of the developed Lesion Area Index and Lichen Sclerosus Activity Index in men allow to objectively assess the effectiveness of treatment. 4. Lichen sclerosus assessment indices allow to develop an individual plan of rehabilitation measures. 5. It is expedient to continue studies on pathogenetic mechanisms of influence of alpha‑2b interferon in genital lichen sclerosus.

Objectives Lichen sclerosus (LS), a chronic inflammatory dermatosis localized in the anogenital region, is known to be associated with lower urinary tract symptoms (LUTS) in postmenopausal women; however, there is a paucity of data on prepubertal girls. We sought to characterize the relationship between LS, LUTS, and constipation among premenarchal girls. Materials and methods We conducted a retrospective chart review of premenarchal girls presenting at the pediatric adolescent gynecology and pediatric urology clinics at our institution diagnosed with vulvar LS during a 4-year period. Demographics, clinical presentation, time from onset of urinary and/or bowel symptoms to LS diagnosis, and treatment response were assessed. Analysis was completed using median, range, and nonparametric statistical analysis where appropriate. Results Nineteen premenarchal girls with LS met inclusion criteria. The median age at LS diagnosis was 6.2 years (range = 3.3-13.3 yrs). Most girls (12/19, 63.2%) presented with LUTS during LS evaluation, and 11 (57.9%) of 19 patients had constipation. Pruritus and hypopigmentation were the most frequently encountered LS symptom and sign, respectively. Fourteen (73.7%) of the 19 girls had resolution/improvement of LS symptoms after LS treatment. Of the 12 patients with LUTS, 7 (58.3%) had resolution/improvement of LUTS symptoms, 3 (25.0%) had unchanged voiding symptoms, and 2 (16.7%) had unknown changes. Conclusions Although further studies are needed to better characterize the relationship between LUTS, constipation, and LS, our findings suggest that premenarchal subjects presenting with LUTS should be assessed for LS. Likewise, patients presenting with LS should be assessed for LUTS and constipation.

The diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for phimosis from 2015 to 2019. Histology was routinely performed. Cohen’s kappa coefficient, sensitivity, specificity, predictive values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended. What is Known:• Occurrence of balanitis xerotica obliterans may be underestimated in children and it could lead to long-term complications.• The diagnostic accuracy of clinical examination is controversial. What is New:• Clinical signs together with patients’ medical history present high specificity and positive predictive values but low sensitivity.• When suggestive clinical aspects are present, patient should be referred to surgery avoiding prolonged conservative treatment; and preputial histology is highly recommended.

The purpose of this review is to provide the diagnosis and management of inflammatory vulvar dermatoses in the geriatric population. The increasing geriatric population in the USA necessitates informing a broad base of healthcare providers on this topic. In the elderly, there are increasing case reports of contact dermatitis due to sanitary napkins, incontinence pads, and over the counter medications. Superimposed vulvovaginal candidiasis and lichen simplex chronicus can occur simultaneously with the primary vulvar disease. The limited research specific to geriatric populations confirms the current state of vulvar dermatosis underdiagnosis. In order to close the gaps in diagnosis and treatment of vulvar dermatoses, providers should carefully review the patient’s medical history and complete a physical examination of the genitoanal area for asymptomatic and symptomatic elderly women. Prompt, consistent treatment can vastly improve the patient’s quality of life and is critical to preventing the advancement of lesions to squamous cell carcinoma in conditions that incur this increased risk such as lichen sclerosus and lichen planus.

Modern literature information on the etiopathogenetic prevalence of lichen sclerosus has been presented by the authors; clinical manifestations and classification of the disease have been outlined; diagnosis and treatment issues have been discussed in the article. 3 clinical cases of genital lesions in the form of lichen sclerosus in patients with limited scleroderma have been described. In our opinion, lichen sclerosus is one of the forms of limited scleroderma. Purpose. To investigate the conditions for the diagnosis and treatment of genital lesions in the form of lichen sclerosus in patients with limited scleroderma. Methods. The first line of therapy for lichen sclerosus is the use of super-powerful topical glucocorticoids. Clobetasol propionate 0.05% is to be used for 3 months (1 month daily, then every other day for a month, then 2 times a week for a month). After 3 months of treatment, if a positive effect is declared, maintenance therapy is recommended once a week. Topical calcineurin inhibitors are recommended as a second-line therapy. Effective and safe excipients are emollients that moisturize the skin and mucous membranes, prevent transdermal fluid loss, and reduce subclinical inflammation. The possibility of using triamcinolone (1-2 mg) onto pathologically altered tissue in women with lichen sclerosus where there are hyperkeratotic sites resistant to GCS can be considered after exclusion of intraepithelial neoplasia or malignant transformation. The effectiveness of physiotherapeutic methods, such as low-intensity helium-neon laser radiation in the red or infrared range, treatment with sunlight and radiation generated by medical equipment, photodynamic therapy has been described. Results. One of the important measures is the elimination of irritating factors, as well as careful care of the vulva and the treatment of secondary infection. It consists in adherence to a diet (exclusion of spicy, salty, sweet foods, caffeine-containing products, alcohol) and intimate hygiene rules (limitation / exclusion of soap-containing products, deodorants, synthetic underwear, pads, tampons). The underwear should not be very tight. In addition, patients should exclude mechanical irritation of the affected area, including shaving. With extreme manifestation of itching, it is recommended to prescribe desensitizing therapy and sedatives. Conclusions. Clinical cases of practical interest in connection with the clinical manifestations of limited scleroderma on the skin and genitals have been considered. Frequent lesions of the genital organs in the form of a typical clinic of lichen sclerosus in women suffering from limited scleroderma, the similarity of their clinic, histological picture and pathogenesis have been noted. All this gives a reason to consider lichen sclerosus as a manifestation of limited scleroderma.

Lichen sclerosus (LS) may cause the glans and prepuce to become fused, making a standard circumcision impossible. Most authorities recommend excision of the fused area with glans resurfacing, although partial circumcision is often performed.To evaluate an alternative technique that preserves the fused area and allows a complete circumcision without grafting.Over 3 yr (January 2016-March 2018), 28 men (age 28-93 yr; mean 62 yr) underwent the restoration of lost obscured coronal sulcus (ROLOCS) procedure with over 1 yr of follow-up. Complications were reviewed retrospectively with an additional survey.The shaft skin is incised at the corona. Dartos is divided, which allows antegrade dissection just outside the fused glans membrane. The foreskin is removed and shaft skin sutured to dartos below the corona.Postoperative pain, aesthetic satisfaction, sexual enjoyment, glans sensation, and urinary symptoms were measured.There were no major complications. In all cases, the coronal sulcus was restored and the glans skin became soft without skin grafting. All were satisfied with the aesthetics. Of the patients,70% experienced mild to low-moderate pain; 55% and 25% had, respectively, improved or reduced glans sensation; and 40% reported improved enjoyment of sex. Histology showed LS in all cases with squamous cell carcinoma in four, including three out of five patients who had previously undergone partial circumcision. Although this is the largest series reported yet, the numbers were too small for a meaningful statistical analysis.The ROLOCS operation offers an aesthetically superior alternative to partial circumcision and is easier to perform with less morbidity than skin grafting.The restoration of lost obscured coronal sulcus (ROLOCS) procedure provides an alternative to partial circumcision or circumcision with skin grafting when the foreskin is welded to the head of the penis (glans) due to lichen sclerosus. It produces a good cosmetic result, but the glans can be sore until it heals.

Background Differentiated vulvar intraepithelial neoplasia (dVIN), the precursor lesion to human papillomavirus (HPV)-independent vulvar squamous cell carcinoma (VSCC), can be difficult to distinguish from vulvar inflammatory dermatoses. p53 has been suggested to be a useful biomarker for dVIN, but the percentage, intensity and patterns of staining have not been well characterized in dVIN and its histologic mimics. Method We studied p53 immunohistochemical staining patterns in 16 dVIN cases and 46 vulvar non-neoplastic squamous lesions (12 lichen sclerosus (LS); 7 lichen simplex chronicus (LSC); 3 lichen planus (LP); 6 psoriasis (PSO); 13 spongiotic dermatitis (SPO); 5 candidiasis (CAN)). dVIN cases were adjacent to a p16-negative invasive VSCC in resection specimens. Results All dVIN cases demonstrated null-type or moderate-strong uniform p53 staining in over 70% of basal cells, with moderate to strong continuous parabasal staining extending to two-thirds of the epidermis. This was in contrast to weak or weak-moderate patchy p53 staining in the majority of other lesions. Moderate-strong and increased basal p53 staining (≥70%) was also observed in a subset of lichen sclerosus (5/12, 42%), lichen planus (1/3, 33%) and spongiotic dermatitis (36%, 4/11), however in all categories, this was limited to the basal layer and any staining in the parabasal layers was patchy. Conclusion Strong and uniform p53 staining of basal cells, extending into the parabasal layers, and a complete absence of staining (null-type) is useful in distinguishing dVIN from other mimics in the vulva. p53 staining of lesser intensity or quantity, particularly basal-overexpression only, overlaps with vulvar inflammatory lesions.

Besides the well-known use in supporting the non-invasive diagnosis of non-tumoral dermatoses (general dermatology), dermoscopy has been shown to be a promising tool also in predicting and monitoring therapeutic outcomes of such conditions, with the consequent improvement/optimization of their treatment. In the present paper, we sought to provide an up-to-date overview on the use of dermoscopy in highlighting response predictor factors and evaluating therapeutic results in the field of general dermatology according to the current literature data. Several dermatoses may somehow benefit from such applications, including inflammatory conditions (psoriasis, lichen planus, dermatitis, granulomatous conditions, erythro-telangiectatic rosacea, Zoon balanitis and vulvitis, cutaneous mastocytosis, morphea and extra-genital lichen sclerosus), pigmentary disorders (vitiligo and melasma) and infectious dermatoses (scabies, pediculosis, demodicosis and viral warts).

Supplemental digital content is available in the text., Objective The aim of the study was to describe the demographic, clinical, and histopathologic features of differentiated vulvar intraepithelial neoplasia (dVIN) and vulvar aberrant maturation (VAM). Methods Specimens from 2010 to 2020 reported as dVIN or VAM were reviewed. Clinical data included age, rurality, symptoms, and evidence of lichen sclerosus (LS). Histopathologic data included epithelial thickness, keratinization, architectural and dyskeratotic features, stroma, p16, and p53. Differentiated vulvar intraepithelial neoplasia and VAM were distinguished by assessment of basal nuclear chromatin, enlargement, pleomorphism, and mitoses. Results One hundred twenty women with a median age of 71 years had 179 examples of dVIN and VAM. Squamous cell carcinoma was concurrent in 66% and associated with rurality. Ten percent were asymptomatic, and all but 3 had evidence of LS. Differentiated vulvar intraepithelial neoplasia showed a range of thickness, architecture, and dyskeratosis; its unifying !feature was basal atypia. Differentiated vulvar intraepithelial neoplasia displayed hyperchromasia in 83% and easily observed mitoses in 70%. Nonkeratinizing morphology, subcategorized into basaloid and intermediate, occurred in 24% of women with dVIN. Traditional dVIN represented 62% of keratinizing cases; the remainder were atrophic (13%), hypertrophic (13%), acantholytic (8%), or subtle (5%). Vulvar aberrant maturation had abnormal stratum corneum, acanthosis, premature maturation, and enlarged vesicular nuclei. Null p53 helped distinguish dVIN from VAM and dermatoses. Conclusions The morphology of dVIN encompasses nonkeratinizing and keratinizing types, the latter subdivided into traditional, acantholytic, atrophic, hypertrophic, and subtle. Diagnosis relies on basal atypia with supportive p16 and p53. Atypia exists on a biologic spectrum with mild abnormalities of VAM and reactive change. Identification of dVIN and VAM requires collaboration between clinicians and pathologists experienced in vulvar disorders.

Objectives: To investigate the prevalence of pathological disease and spectrum of human papillomavirus (HPV) types among symptomatic foreskin tissue. Patients and Methods: Consecutively excised symptomatic foreskins from 351 men were sent for histopathological evaluation. During the surgical procedure, a fresh biopsy was taken for HPV analysis by modified general primer polymerase chain reaction. A medical questionnaire regarding medication, smoking habits, number of lifetime sexual partners, former diseases and surgery performed on penis was completed by all participants. Results: The most common clinical diagnosis and cause for circumcision was phimosis, seen in 85.2%. Histopathologically inflammatory dermatological conditions were present in 87% of the men. The most common histopathological diagnosis was lichen sclerosus (LS) observed among 58.7%. Notably, penile intraepithelial neoplasia (PeIN) was present in 2% without former clinical suspicion. Overall, HPV was detected in 17.1% of the men and 28 different HPV types were found. High-risk (HR) HPV types were identified in 9.1% and HPV16 was present in 2.3%. Current smoking increased the risk of HPV (crude odds ratio [OR] 2.8, confidence interval [CI] 1.4–5.6; P = 0.005). Having >15 lifetime sexual partners increased the risk of HPV (crude OR 2.6, 95% CI 1.4–5.1; P = 0.003) and when adjusted for current smoking the OR was substantially increased (OR 6.0, 95% CI CI 2.2–16.8; P < 0001). Conclusions: Histopathological evaluation of circumcised symptomatic foreskin revealed PeIN in 2% of the men without any clinical suspicion of malignancy and that treatable dermatological conditions were present in 87%, LS being the most common. HR-HPV types were present in 9%. Due to risk of malignant development both in PeIN and in inflammatory skin diseases we recommend sending all excised foreskins from patients with symptoms for histopathological evaluation as guidance for further clinical management. (Less)

Despite increasing incidence, vulvar squamous cell carcinoma (VSCC) is still a rare disease. Until now, two etiological pathways have been described: a high-risk human papillomavirus (HPV)-dependent route and an HPV-independent pathway often associated with lichen sclerosus. To date, therapeutic strategies in VSCC are not influenced by molecular pathological information and therapeutic options for advanced or recurrent disease are limited.Whole exome sequencing of DNA, isolated from 34 VSCC samples and matched normal tissue for each individual was performed on an Illumina HiSeq4000. Short variant discovery was carried out using BWA mem and FreeBayes. Variants were annotated using ANNOVAR.FIGO stages were: IB (n = 7), II (n = 11), III (n = 8), and IVA (n = 3), (n = 5 unknown). TP53 missense mutations were most commonly detected with 56% (19/34). 12/34 (35.3%) samples were HPV positive (all HPV16), HPV positivity and TP53 mutations were mutually exclusive (p .0001). Additionally, we observed mutations in known cancer relevant genes, like NBPF1 (n = 7), MACF1 (n = 5), SYNE2 (n = 5), DOCK2 (n = 4), KMT2D (n = 4), MAP2 (n = 4), NACA (n = 4), PIK3CA (n = 4), SYNE1 (n = 4), FBWX7 (n = 3), MSH6 (n = 3), NSD1 (n = 3), POLE (n = 3), TSC2, (n = 3) and CDKN2A (n = 2), but at considerably lower frequencies. For the total cohort 1848 cancer related mutations were detected (median of 54.4 per sample).The key mutation in HPV negative vulvar carcinoma affects TP53. While a multitude of cancer related mutations was detected in various samples, only few mutations recur and/or affect concurrent signaling pathways.

Penile intra-epithelial neoplasia (PeIN) is a known precursor for penile cancer. It may be undifferentiated or differentiated. The former is related to high-risk Human Papilloma Virus (HPV) and associated with p16 over-expression. Patients may present with red patches or lesions on the penis which on occasion may affect sexual activity.This study will assess associations between p16 status, patient parameters, treatment choice and outcomes. Data were collected on patients diagnosed with PeIN, who were referred to a single European Network, between 2008 and 2018. The following parameters were collected utilising patient records: demographics, smoking status, performance status, comorbidities, HPV/p16 status, lichen sclerosus (LS) status, treatment and clinical response. Log rank, Kaplan–Meier, Pearson Chi-Squared and Fishers Exact test were utilised to determine significance. One hundred thirty-seven patients were identified with PeIN and no invasive cancer. Staining for p16 was available in 91 patients and 74 patients were p16+. There were no significant differences in disease-free survival (DFS) for smoking status, performance status, comorbidities and lichen sclerosus, although patients with lichen sclerosus tended to recur sooner. Overall, p16+ patients showed significantly better DFS over p16− patients (n = 67; 10.4 vs 7.4 months; p = 0.023). In p16+ patients receiving treatment with imiquimod alone or with surgery, response rates were 100% vs 54% without imiquimod (n = 56; p = 0.017). In p16− patients receiving treatment with imiquimod alone or with surgery, response rates were 100% vs 56% without imiquimod (n = 17; p = 0.99). Overall 13.6% of patients progressed to cancer. The results indicate treatment combinations with immunotherapy tend to provide better responses despite p16 status. Patients with p16+ disease have a longer disease-free survival. Approximately 14% of patients progress to invasive disease. However, given the limitations in this study, further research is required to confirm these findings.

Lichen sclerosus (LS) is a chronic relapsing disease with a predilection for the anogenital areas of patients of both sexes and at all ages. In males, LS is usually a late diagnosis, increasing the...

BACKGROUND/OBJECTIVES Vulvar lichen planus is a debilitating skin condition usually complicated by delayed diagnosis due to its highly variable clinical appearance and inconsistent histopathological characteristics. This study aims to devise a clinical diagnostic tool for the disease and to correlate this with histopathology findings. METHODS The retrospective single-centre chart review was conducted for patients presenting between January 2010 and December 2019. Clinical features were compared between 243 women with clinically suspected vulvar lichen planus with available histopathology, 50 patients with biopsy-proven vulvar lichen sclerosus and 50 patients with culture-proven chronic vulvovaginal candidiasis. Features which significantly differentiated between conditions were further studied using multivariate nonlinear regression analyses to formulate a score-based diagnostic criteria. Criteria was then applied to the remaining patients with inconclusive biopsies (classified as 'normal', 'non-specific' or 'suggestive or lichenoid') to determine sensitivity and specificity. RESULTS The clinical features that significantly differentiated the conditions were the presence of erosions (P

BACKGROUND Chronic inflammatory vulvar diseases can have a huge detrimental impact on patient welfare. A few studies have addressed this issue so far, mainly measuring patients' quality of life or sexual dysfunction. OBJECTIVE To assess the burden of suffering in patients with chronic inflammatory vulvar diseases using the Pictorial Representation of Illness and Self-Measure (PRISM), a visual, non-verbal instrument. We also assessed (i) the concordance between PRISM and the Dermatology Life Quality Index (DLQI), (ii) whether some variables, both patient-related and disease-related, affect the patient's suffering and quality-of-life impairment. METHODS We evaluated for inclusion in this cross-sectional study all patients who attended our Vulva Unit over a 9-month period with histologically proven lichen sclerosus (LS), lichen planus (LP), lichen simplex chronicus (LSC), eczema, plasma cell vulvitis and psoriasis (at least five for each disease). Demographics and disease-related subjective and objective scores were recorded. The PRISM and DLQI were administered. RESULTS We included 87 patients affected with LS, 13 with LSC and seven with LP. Median PRISM values (0-273 mm) ranged from 95 to 120 mm, depending on the disease, and median DLQI scores (0-30) were five for all three groups. Neither PRISM nor DLQI scores differed significantly among the three groups. Moderate coherence was found between PRISM and DLQI (ρ = 0.5455, P

A 62-year-old female, with previous history of asthma and hypertension, presented with generalized hyperpigmented skin lesion, found a year ago. Physical examination revealed brown colored lichenified and sclerotic patches on the lower abdomen and flexural areas of extremities. Punch biopsy was performed and histopathological examination revealed hyperkeratosis, follicular plugging and thinning in epidermis. In dermoepidermal junction, cleft like space separating atrophic epidermis and dermis was seen. Also, lichenoid lymphocytic infiltration was observed in mid-dermis. Based on clinical and histopathological findings, a diagnosis of generlaized lichen sclerosus et atrophicus (LSA) was made. Other laboratory examinations were unremarkable. As there is no standard treatment for LSA, the patient received various treatments including topical steroid, tacrolimus and narrow- band ultraviolet B therapy. The skin lesion has softened and its color improved after treatment. LSA is defined as infrequent chronic inflammatory dermatosis with anogenital and extragenital manifestations. Generalized type is rare and genital involvement is the most frequent and often the only site of involvement. We report this case as it is an uncommon type of LSA with generalized hyperpigmented and sclerotic skin lesion in a postmenopausal female patient. (Ann Dermatol 32(4) 327∼330, 2020)

Lichen sclerosus (LS) was first described by Hallopeau in 1887. It is a chronic inflammatory condition most commonly involving the anogenital region with a relapsing course and a potential for destruction, functional impairment, atrophy, and malignant changes. LS affects both sexes with a female preponderance of 5:1. The exact prevalence of the disease is difficult to predict as the lesions are asymptomatic in the initial phase and later when the complications arise patients might visit the surgeon, pediatrician, gynecologist, or urologist. The etiology of LS has a complex interplay of genetic factors, autoimmunity, infections, and trauma. Physical examination to assess the extent of the disease and decide the line of management is the most crucial step in the management. Corticosteroids, calcineurin inhibitor, retinoids, phototherapy, and surgery can be helpful. Self-examination and long-term follow-up are necessary.

The most problematic vulvovaginal conditions are familiar to dermatologists but may exhibit distinct clinical features or medication management because of the anatomic location. The second article in this continuing medical education series focuses on management pearls for treating vulvar diseases. We highlight key conditions, such as lichen sclerosus, erosive lichen planus, and vulvodynia. In addition, we review conditions that dermatologists may be less familiar with, such as plasma cell vulvitis, desquamative inflammatory vaginitis, vulvar aphthae, and low estrogen states. Nearly 1 in 6 women experience undiagnosed and untreated vulvovaginal discomfort at some point in their lives. Physicians who treat vulvar disorders will improve the quality of life of countless women.

We describe the association of balanitis xerotica obliterans and differentiated penile intraepithelial neoplasia (PeIN) with dermal elastosis and "bramble-bush" elastic fibers in a diabetic patient with no history of penicillamine intake. An 84-year-old man presented with urethral obstruction syndrome that required postectomy, meatotomy, and foreskin excision. Histopathological examination revealed changes of lichen sclerosus and differentiated PeIN. Orcein staining showed striking dermal elastosis and transepidermal elimination of elastic fibers. At higher magnification, multiple serrations and buds arising from the borders of the elastic fibers were observed giving the so-called bramble-bush appearance. Balanitis xerotica obliterans is considered a premalignant disease, and 14% of patients have evidence of PeIN. The differentiated subtype can be difficult to diagnose because of the minimal basal cell atypia, so a striking dermal elastosis, which may even occur before the neoplasm becomes invasive, may facilitate its diagnosis. The term "acquired perforating dermatosis" seems appropriate to describe those cases of perforating elastosis that occurs in adults with systemic diseases. The bramble-bush appearance of elastic fibers is not specific for penicillamine-induced elastopathy, and it may occur in other diseases, such as diabetes mellitus. This peculiar morphology of elastic fibers may be related to the enzymatic imbalance between matrix metalloproteinases and lysyl oxidase, an enzyme required for the cross-linking of elastic fibers.