Article

Risk factors for genital lichen sclerosus in men

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Abstract

Lichen sclerosus (LS) is an inflammatory disease of the skin and mucous membranes. Its aetiology is still unknown. To determine risk factors for genital LS in men. In a case-control study, 73 patients with LS, consecutively diagnosed at the City Dispensary for Skin and Venereal Diseases in Belgrade, were compared with 219 male patients visiting the same institution because of tinea cruris. Univariate and multivariate logistic regression analyses were used for analysis of data collected. According to multivariate logistic regression analysis, risk factors for male LS were as follows: a personal history of genital injury [odds ratio (OR) 28·1, 95% confidence interval (CI) 5·2-150·8], vitiligo (OR 23·1, 95% CI 2·2-240·2), alopecia areata (OR 8·8, 95% CI 1·1-68·5) and hypercholesterolaemia (OR 3·1, 95% CI 1·1-8·2), and a family history of alopecia areata (OR 24·3, 95% CI 2·1-280·7), diseases of the thyroid gland (OR 9·1, 95% CI 2·3-36·2) and other autoimmune diseases (OR 8·6, 95% CI 1·3-58·6). The results of the present study are in line with the hypothesis that trauma of the penis is a possible trigger of symptoms in genetically predisposed individuals and that personal and family histories of autoimmune disorders are risk factors for male LS.

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... Comorbidities with a significant association with LS include chronic hypertension, diabetes mellitus, rheumatoid arthritis/collagen vascular disease and obesity. Diabetes mellitus has a known association with LS. 10,11 In addition to diabetes mellitus, Hofer et al showed LS in men was associated with obesity as well as coronary artery disease and tobacco use. 10 These findings suggest that direct microvascular compromise or metabolic disturbances may contribute to the development of LS. ...
... Lastly, a previous study showed that a family history of diabetes mellitus, vitiligo, alopecia areata and thyroid diseases were independent risk factors for LS in men. 11 These autoimmune diseases were not available in the NIS data set. A higher percentage of patients with LS had a complication (7.5%) than those without LS (6.5%) in the immediate postoperative period, but this was not statistically significant. ...
Article
Introduction: We characterize comorbidities and inpatient complications of patients with lichen sclerosus who underwent urethroplasty from a large national patient data source. Methods: We queried the Nationwide Inpatient Sample for patients who underwent urethroplasty between 2000 and 2010. We compared demographics, comorbidities, complications, length of hospital stay and hospital charges for patients with and without the diagnosis of lichen sclerosus. Results: An estimated 13,700 urethroplasties were performed in the United States during the study period. Patients with lichen sclerosus comprised an estimated 3.8% of the urethroplasty population. The majority of patients with urethral stricture with lichen sclerosus were Caucasian (84%) and older, with 63% age 45 or older. Chronic hypertension, diabetes mellitus, rheumatoid arthritis/collagen vascular disease and obesity were associated with increased odds of having a lichen sclerosus diagnosis. The central East Coast (7.2%) and the Pacific Northwest (6.3%) had the highest percentage of patients treated with urethroplasty with lichen sclerosus. Patients with lichen sclerosus had longer hospital stays than those without lichen sclerosus (3.5 vs 2.6 days, p <0.0001). Patients with lichen sclerosus had more complications and hospital charges than those without lichen sclerosus but these differences did not reach statistical significance. Conclusions: A higher percentage of patients with lichen sclerosus had comorbidities, increased complications and longer hospital stays compared to patients treated with urethroplasty without lichen sclerosus. Our findings demonstrate the increased complexity that providers face when treating men with lichen sclerosus related urethral stricture disease. © 2015 American Urological Association Education and Research, Inc.
... Male genital lichen sclerosus (LS) is a chronic inflammatory dermatosis of undetermined etiology, which occurs in all age groups. The exact pathogenesis is unknown, but an autoimmune etiology have been proposed (1)(2)(3)(4). The prevalence of genital LS is difficult to estimate, but it is usually underestimated. ...
... Sport activities are often associated with injuries. Koebner phenomenon which occurs on sites of injured or traumatized skin is a well-known manifestation of LS, so trauma, injury, and sexual abuse have been suggested as possible triggers of symptoms in genetically predisposed people (4,6). ...
Article
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The aim of the paper was to evaluate the clinical presentation, demographic and lifestyle characteristics of men with genital lichen sclerosus (LS). This study examined the cases of 73 men with genital LS who appeared at the City Institute for Skin and Venereal Diseases in Belgrade between January 2007 and December 2008. The diagnosis of LS was established by history and physical examination. Data about demographic and lifestyle characteristics were obtained by the use of a questionnaire. Nearly 60% of men with LS were older than 45 years. The most frequent complaint was a tight foreskin which was detected in 70% of men. White atrophic lesions, fissures and cracking were recorded in the majority of the patients. The most frequently affected site was prepuce in 76% of cases. More than 70% of participants had frequent sexual activities and 57.5% were exposed to stress. LS is disease with a wide spectrum of clinical manifestations, such as prepuce lesions, which increases the risk for acquiring some sexually transmitted pathogens. That fact stresses the importance of LS patients counseling on consistent condom use and importance of early diagnosis and early treatment which may prevent further penile lesions, complications and diminish vulnerability to sexually transmitted infections and HIV.
... Lichen sclerosus (LS) je hronično inflamatorno oboljenje koje se javlja u anogenitalnoj regiji kod muškaraca svih uzrasnih grupa. Iako je uzrok bolesti nepoznat, smatra se da je oboljenje autoimune etiologije (1)(2)(3)(4). Prevalenciju genitalnog LS kod muškaraca je teško utvrditi, jer bolesnici često nemaju izražene simptome, te se ne javljaju lekaru, a sa druge strane, upućuju se lekarima različitih specijalnosti (dermatovenerolozima, urolozima, hirurzima, pedijatrima). Kizer i saradnici su u svojoj studiji opisali da je prevalencija genitalnog LS 0,07% (5). ...
... Kada uporedimo naše rezultate sa ovom studijom, vidimo da su bolesnici sa LS ređe konzumirali alkohol (38% prema 70,5%) a češće upražnjavali sportske aktivnosti (28,7% prema 54,2%). Sportske aktivnosti neretko su praćene i povredama genitalne regije, a Koebnerov fenomen koji se javlja na mestima povrede i traumatizacije tkiva opisan je kod LS, te povrede genitalne regije mogu biti okidač za pojavu simptoma kod genetski predisponiranih osoba (4,6). ...
... atitis C virus) and trauma has also been implicated in triggering the disease. 4,5 The most common location is the genital area. The extragenital form occurs in approximately 20% of the patients, and in the majority of these cases there are concomitant genital lesions. ...
Article
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Lichen sclerosus is a chronic inflammatory disease, usually located in the genital area. The etiology of lichen sclerosus is multifactorial, with participation of genetic, autoimmune, infectious and hormonal factors. Bullous clinical form stems from hydropic degeneration of the basal membrane, constituting a less frequent variant of the disease. In this work, we report the case of a female patient, 55 years old, who in the last three years presented whitish plaques, with horny spikes, located on back and arms. Some of these lesions evolved with hemorrhagic blisters, which after histopathological examination confirmed the diagnosis of bullous and hemorrhagic lichen sclerosus. The patient was treated with high-potency topical corticosteroid for two months, resulting in remission of bullous and hemorrhagic lesions.
... Estimates of the prevalence of LS vary from 0.0014% to over 0.01% in adult men, but it may be underreported [4,5]. While in practice it is primarily diagnosed clinically, the degree to which clinical diagnosis is consistent with pathologic findings is not well established. ...
Article
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Purpose Lichen sclerosus (LS) in men is poorly understood. Though uncommon, it is often severe and leads to repeated surgical interventions and deterioration in quality of life. We highlight variability in disease presentation, diagnosis, and patient factors in male LS patients evaluated at a tertiary care center. Materials and Methods We retrospectively reviewed charts of male patients presenting to our reconstructive urology clinic with clinical or pathologic diagnosis of LS between 2004 and 2014. Relevant clinical and demographic information was abstracted and descriptive statistics calculated. Subgroup comparisons were made based on body mass index (BMI), urethral stricture, and pathologic confirmation of disease. Results We identified 94 patients with clinical diagnosis of LS. Seventy percent (70%) of patients in this cohort had BMI >30 kg/m², and average age was 51.5 years. Lower BMI patients were more likely to suffer from urethral stricture disease compared to overweight counterparts (p=0.037). Patients presenting with stricture disease were more likely to be younger (p=0.003). Thirty percent (30%) of this cohort had a pathologic diagnosis of LS. Conclusions Urethral stricture is the most common presentation for men with LS. Many patients endure skin scarring and have numerous comorbidities. Patient profile is diverse, raising the concern that not all patients with clinical diagnosis of LS are suffering from identical disease processes. The rate of pathologic confirmation at a tertiary care institution is alarmingly low. Our findings support a role for increased focus on pathologic confirmation and further delineation of the subtype of disease based on location and clinical manifestations.
... In our case, the patient has a LSA that has been associated either to FFA and vitiligo. [1,2] A dermoscopic clue, for suspect a primary cicatricial alopecia, is the loss of follicular ostia reflecting the cicatricial phenomenon that were absent. In this case, the first diagnostic hypothesis was of FFA, because of the clinical characteristics and the LSA background. ...
... LS'nin, birliktelik gösterdiği otoimmun hastalıklar sıklıkla Hashimoto tiroiditi, alopesi areata, vitiligo, diabetes mellitus ve pernisyöz anemidir. Bu hastalıklar açısından ailesel ve kişisel otoimmun hastalık öyküsü LS için risk faktörü kabul edilir (1,5,6). LS'nin otoimmun hastalık olduğunu düşündüren bir diğer bulgu, genital LS'li hastalarda dolaşımda ekstraselüler matriks protein (EMP) -1'e karşı Ig G yapısında antikor varlığının gösterilmesi olmuştur (1,7). ...
Article
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... Surgically constructed vulvo-vaginal area in transgender women meant to resemble vaginas in cis-gendered women; usually constructed from penile, scrotal, and/or colonic tissue; also refers to construction of vaginas in congenital abnormalities, vaginal malignancies, and intersex disorders could be unique to transgender women in the context of their hormone use, chronic irritation, and trauma/scarring from surgery (Bjekić et al., 2011;Friedrich and Kalra, 1984). ...
Article
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Many previous reviews and studies on transgender dermatology have highlighted the expected dermatologic manifestations of hormone affirmation therapy in transgender patients. Others have highlighted attitudes and practices of both transgender patients and medical professionals taking care of these patients. This review compiles data from other, lesser known aspects of transgender dermatology, including neovaginal concerns, neoplastic concerns (both neovaginal and cutaneous), autoimmune conditions, and the sequelae of injectable substances that have not been approved by the U.S. Food and Drug Administration. This review, like others, will be a stepping-stone and serve as an impetus for future research in transgender dermatology.
... The etiopathogenesis of LS is not fully understood, however it seems that the genetic and autoimmune background may have the strongest influence on its development. The relationship with injuries, hormonal disorders, and chronic irritation of the genitals is also suggested [4][5][6][7][8][9]. ...
... However, the sequence of events leading to the altered fibroblast function, microvascular changes and hyaluronic acid accumulation in the upper dermis is still being researched. The prevalence of vulvar LS in elderly nursing home women in one study was found to be 3% (1 in 30) [10]. ...
... Vitiligo (12 % vs. 0 %) and alopecia areata (12 % vs. 1 %) were significantly more common in patients than in controls. A family history of diabetes mellitus, vitiligo, alopecia areata, or thyroid disease and the presence of other autoimmune diseases were identified as risk factors for male LS [18]. ...
Article
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Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.
... Гистологические изменения, похожие на ЛС, нередко наблюдаются после повторных уретроплас тик бульбарного отдела, но при этом изменения не распро страняются на проксимальный или дистальный отделы мо чеиспускательного канала и ЛС невозможно подтвердить по гистологическому материалу, несмотря на картину протя женной стриктуры по данным уретрограмм [59,83] [32,60,74,105,114,121]. Выявлены аутоантитела других типов (42%), а также связь с другими ау тоиммунными болезнями, включая витилиго, алопецию и за болевания щитовидной железы у пациентов с ЛС [21, 37,71]. ...
... [19][20][21] Koebnerization is frequent and LS with koebnerization may be accompanied by plaque type morphea. 22,23 Some authors claim that LS and morphea have a common pathogenesis. 11,22,24 Involvement of the genital area might lead to functional problems and skin involvement may cause cosmetic problems. ...
Article
Background: Lichen sclerosus (LS) is a chronic disease of the skin, for which the pathogenesis is not known. It can lead to various changes of the skin and the genital area, potentially leading to both functional as well as cosmetic problems for the patient, thus disrupting the quality of life. In this study; the purpose was to review the clinical characteristics and the treatments of the 15 pediatric patients under the age of 18 followed up in our out-patient clinic with a diagnosis of LS and to compare the findings with literature data. Methods: Between 2011 and 2017, the files of 15 patients diagnosed clinically and/or histologically with LS in our clinic were retrospectively examined. The demographic characteristics, clinic and laboratory findings, treatment options of the patients are reported. Results: Of the patients included in the study 14 were girls and one was a boy. The average age was 11.6 years (5-17 years), the average age for the initial disease was 7.8 years (2-13 years). The average duration of the disease at the diagnosis was 3.9 years. The most common form was genital vulvar type (8/14 girls) without anal and cutaneous involvement, and each of them suffered from itching. One of the cases had genital LS as well as extragenital morphea lesions. Two of the 15 patients were ANA positive. The other antibodies were negative. In two cases with extragenital involvement, lesions were widespread and they were in blachkoid form. Conclusion: LS is a chronic disease that progresses with recurrences and regressions. In our study, the most common LS type was genital type (60%). There was extragenital involvement in 6 patients (40%). Extragenital involvement was the most common on the trunk. Diagnosis, treatment and follow-up during childhood is highly important to prevent any possible future anatomical or psychological damage and genital malignancies.
... Morphea and lichen sclerosus are immune diseases that may be associated with other autoimmune disorders. The coexistence of two autoimmune diseases may not occur by chance, but could be attributed to autoimmune reactions elicited by recognition of common antigens [4][5][6][7] . ...
Article
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Thyroid gland is one of the key organs regulating the metabolism of carbohydrates, proteins and fats. Its primary function is connected with increase of the metabolic conversion of the body. Skin lesions are often one of the first symptoms of hypothyroidism. In a 71-year-old patient, skin lesions in the form of thickened areas with severe hyperkeratosis first appeared 12 years before. Eight years before, the patient was diagnosed with hypothyroidism of unknown cause. Upon admission, lesions were observed in the trunk area, left arm and vulva. These lesions had the appearance of brownish spots with hyperkeratosis. Initially, they were localized on the trunk, then involving upper limbs and neck area with time. Significant progression was present in the back area. In the anogenital area, porcelain-white discolorations were observed. Laboratory examinations were normal. During hospital stay, iv. ceftriaxone at a dose of 2.0 g/day for 10 days and intramuscular injection of vitamin B6 were administered, along with 10% urea ointment for hyperkeratosis lesions as topical therapy. The patient had lesions in the form of skin induration and discoloration, with visible, very severe hyperkeratosis, which is not characteristic of changes of the scleroderma and lichen sclerosus type. These lesions caused diagnostic problems due to the atypical clinical appearance.
Article
Introduction Lichen sclerosus et atrophicus, or lichen sclerosus, is an acquired, chronic inflammatory disease, resulting in a loss of quality of life and increased morbidity. The causes of the appearance of lichen are unknown and it is important to study the relationship of this disease with different pathologies and lifestyle and demographic factors. Objective The objective of this study was to obtain and present some preliminary results from a small series of patients with lichen sclerosus. Material and methods We report 20 patients undergoing circumcision due to histologically confirmed lichen sclerosus, in whom we analysed demographic, clinical and analytical characteristics to complete an epidemiological profile of patients with this disease. Results The mean patient age was 54.8 years. It is notable that 25% of patients had diabetes mellitus, 50%, hypercholesterolemia and 75% were overweight. Overall lipid and carbohydrate profile was altered in 50% of patients with this disease. Patients with lichen present, in a significant percentage of cases, alterations of the urethral meatus, so the physical examination is very important. Conclusion Although this study had a limited number of patients, we have to bear in mind the possible association of lichen sclerosus with dyslipidemia and other endocrine and metabolic diseases.
Article
Male genital lichen sclerosus (MGLSc) is a chronic inflammatory skin disease responsible for male sexual dyspareunia and urological morbidity. An afeared complication is squamous cell carcinoma (SCC) of the penis. The precise etiopathogenesis of MGLSc remains controversial although genetic, autoimmune and infective (such as human papillomavirus (HPV) hepatitis C (HCV), Epstein-Barr virus (EBV) and Borrelia) factors have been implicated: Consideration of all the evidence suggests that chronic exposure of susceptible epithelium to urinary occlusion by the foreskin seems the most likely pathomechanism. The mainstay of treatment is topical ultrapotent corticosteroid therapy. Surgery is indicated for cases unresponsive to topical corticosteroid therapy, phimosis, meatal stenosis, urethral stricture, carcinoma in situ (CIS) and squamous cell carcinoma.
Article
Lichen sclerosus (LS) is an inflammatory skin disease that usually involves the anogenital area. All patients with symptoms or signs suspicious of lichen sclerosus should be seen at least once initially by a physician with a special interest in the disease in order to avoid delay in diagnosis, as early treatment may cure the disease in some and reduce or prevent scarring. The diagnosis is made clinically in most cases. Biopsies should only be performed under certain circumstances. The gold standard for treatment remains potent to very potent topical steroids; however, mild and moderate disease in boys and men may be cured by circumcision. Certain triggers should be avoided. http://www.euroderm.org/images/stories/guidelines/2014/S3-Guideline-on-Lichen-sclerosus.pdf http://www.awmf.org/fachgesellschaften/mitgliedsgesellschaften/visitenkarte/fg/deutsche-gesellschaft-fuer-gynaekologie-und-geburtshilfe-dggg.html. © 2015 European Academy of Dermatology and Venereology.
Chapter
Morphea and lichen sclerosis et atrophicus (LSA) are two distinct immune-mediated diseases with a dominant presentation of dermal fibrosis and sclerosis. The two diseases have many similar clinical and histological features and tend to co-occur. Both diseases are thought to result from a derailment of the normal response to environmental triggers. Positive family history is more common in LSA than morphea but individuals with morphea have a higher frequency of concomitant and familial autoimmunity. These findings hint at the involvement of inheritance in susceptibility to LSA and morphea and thus provide a rationale for exploring the disease genetics. This chapter contains a comprehensive review of the pathogenesis of the two diseases and their known genetic associations including HLA class I and II genes.
Article
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Background: Limited data are available on risk factors associated with lichen sclerosus and no data are available on gender differences in genital lichen sclerosus (GLS). Objective: This multicentre study aimed at identifying potential risk factors for GLS, through data collection from a large, mixed-sex sample of patients comparing gender-related differences in relation to data from the general population. Methods: This was a cross-sectional study on 729 subjects (53.8% females, 46.2% males) affected with GLS, consecutively observed within a network of 15 Italian dermatology units. The following information was collected: demographic data, anthropometric measures, comorbidities, family history of LS, clinical features and symptoms related to GLS. Results: Overweight and obesity, blood hypertension, hypothyroidism and an educational attainment equal or above upper secondary school level were more frequent among the study patients than among the general Italian population. Moreover, a family history of GLS was reported more frequently than expected among GLS patients. These factors were similar in males and females. The disease tended to occur later in females than in males. Conclusions: Our findings suggest that metabolic factors, and possibly a sedentary lifestyle, may play a role in GLS pathogenesis in genetically predisposed patients, and that risk profile is similar in males and females despite some difference in the onset of symptoms.
Article
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Lichen sclerosus (LS) is a chronic, inflammatory disease primarily involving the genital skin and urethra in males. Historically, the treatment of this common condition was a challenge due to its uncertain etiology, variable response to therapy, and predilection to recur. The etiology of LS is still debated and has been linked to autoimmune disease, infection, trauma, and genetics. Today, topical steroids are a mainstay of therapy for patients, even in the presence of advanced disease, and can induce regression of the disease. In advanced cases, surgery may be required and range from circumcision, meatoplasty, or, in the case of advanced stricture disease, urethroplasty or perineal urethrostomy. When urethroplasty is required, the use of genital skin as a graft or flap is to be avoided due to the predilection for recurrence. Surgical management should be approached only after failure of more conservative measures due to the high risk of recurrenceof LS in the repaired site despite the use of buccal grafting. LS may be associated with the development of squamous cell carcinoma and for this reason, patients should undergo biopsy when LS is suspected and longterm surveillance is recommended.
Article
Lichen sclerosus (LS) is considered to be a disease resulting of local immune dysregulation. A delayed-type hypersensitivity reaction and cell-mediated immune response are hypothesized to be the pathogenesis of granuloma annulare (GA). The case is presented of an uncircumcised 45-year-old man who developed lesions of LS and GA in his foreskin. Both processes were treated with circumcision. GA is rarely located in the foreskin. As far as we are aware, only one previous case presented this location. We describe herein for the first time LS associated with GA in the foreskin. Both processes have a common autoimmune pattern suggesting a link between them. Although we cannot exclude that comorbidity may be a coincidence, we think this combination of lesions is not just coincidental but significantly associated through immunopathological mechanisms.
Article
Resumen Objetivo Comparar los datos epidemiológicos de una muestra de pacientes con liquen escleroso genital versus un grupo control sin liquen escleroso genital. Material y métodos Estudio caso-control que incluye 50 pacientes divididos en 2 grupos; grupo 1: 30 pacientes con liquen escleroatrófico genital y grupo 2: 20 pacientes sin liquen escleroatrófico. Se han estudiado variables clínicas, analíticas, antecedentes personales y hábitos de vida. El análisis estadístico se ha llevado a cabo mediante el programa SPSS 20.0, siendo la significación estadística p ≤ 0,05. Resultados La edad media de los pacientes del grupo 1 fue de 54,7 años versus 52,5 años en el grupo 2, sin que se observaran diferencias estadísticamente significativas. La principal diferencia observada entre los pacientes del grupo 1 y los del grupo 2 fue el índice de masa corporal, que fue más elevado en el grupo 1, 28,4 kg/m², con respecto al grupo 2, 23,4 kg/m² (p = 0,0001), y el estado civil. No se observó una mayor prevalencia significativa en enfermedades autoinmunes en el grupo 1 respecto al grupo 2. Conclusión Los pacientes con liquen escleroatrófico genital parecen tener mayor índice de masa corporal que los pacientes sin liquen, sin que exista una clara asociación con enfermedades autoinmunes, según los resultados de nuestro estudio.
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Lichen sclerosus (LS) is a chronic inflammatory dermatosis that mostly affects the genital and anal skin areas. Symptoms may vary from pruritis and pain to sexual dysfunction, however, LS can also be asymptomatic. LS occurs at all ages and in both sexes. Approximately 5% of all women affected by vulvar LS will develop vulvar squamous cell carcinoma. Topical treatment is safe but less effective resulting in chronic course in most patients, who suffer from persistent itching and pain. In severe cases of therapy‐resistant LS, there is no adequate treatment. Fat grafting is a novel regenerative therapy to reduce dermal fibrosis. The therapeutic effect of adipose tissue grafts for LS is already investigated in various pioneering studies. This review provides an overview of these studies and the putative mechanisms‐of‐action of fat grafting to treat LS.
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Lichen sclerosis (LS) is a chronic, relapsing disease with a variable presentation. In men, genitourinary LS may affect the penile foreskin, glans, meatus, and urethra. Treatment is multifaceted, ranging from pharmacotherapy to surgery. Urethral reconstruction due to stricture disease from LS is frequently plagued by a high recurrence rate. At the authors’ institution, the high recurrence rate has shifted their practice toward potent steroids and minimally invasive surgical techniques. Management of recurrence includes dilation, meatotomy/meatoplasty, 1-stage and 2-stage repairs. Recalcitrant cases may necessitate abandonment of most of the urethra resulting in a perineal urethrostomy.
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Background: Alopecia areata (AA) is a common autoimmune alopecia with heterogeneous severity and distribution. Previous studies found conflicting results about AA epidemiology. Objective: To determine the prevalence, incidence, and predictors of AA, alopecia totalis, alopecia ophiasis, and alopecia universalis. Methods: A systematic review of all published cohort and cross-sectional studies that analyzed AA and its subtypes. MEDLINE, Embase, LILACS, Scopus, Cochrane Library, and GREAT were searched. At least 2 reviewers performed study title/abstract review and data extraction. Random-effects meta-analysis was used because of significant heterogeneity (I2 = 99.97%). Results: Ninety-four studies met the inclusion criteria. The pooled prevalence (95% confidence interval, N) of AA overall was 2.11% (1.82-2.42, N = 302,157,365), with differences of population-based (0.75% [0.49-1.06%], N = 301,173,403) and clinic-based (3.47% [3.01-3.96], N = 983,962) studies. The prevalences of alopecia totalis, ophiasis, and universalis were 0.08% (0.04-0.13, N = 1,088,149), 0.02% (0.00-0.06, N = 1,075,203), and 0.03% (0.01-0.06, N = 1,085,444), respectively. AA prevalence (95% confidence interval) increased over time (<2000: 1.02% [0.85-1.22]; 2000-2009: 1.76% [1.51-2.03]; >2009: 3.22% [2.59-3.92]; P < .0001) and differed by region. AA prevalence was significantly lower in adults (1.47% [1.18-1.80]) than children (1.92% [1.31-2.65]; P < .0001). Conclusions: AA affects 2% of the global population. AA prevalence is lower in adults than children, is increasing over time, and significantly differs by region.
Article
Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians, pediatricians, and dermatologists. It affects both sexes with a lower incidence in men than in women. Autoimmune factors, infections, and genetic disposition are implicated in its pathogenesis. The classic clinical picture is of atrophic white plaques in the anogenital region. Pruritus, soreness, difficulty retracting the foreskin, and obstructive urinary symptoms are frequent complaints. Early histological confirmation by biopsy is recommended. The disorder is usually highly responsive to ultrapotent topical steroids. This may help arrest or delay the progressive nature of this disorder. Surgical treatment may be needed in cases of disease progression despite medical therapy. Circumcision is a successful treatment, if LS is limited to the glans and foreskin. In cases of meatal stenosis meatoplasty and in cases of urethral involvement one- or two-stage oral graft urethroplasty is necessary.
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To test some hypotheses about risk factors for Peyronie's disease (PD). In a case-control study, 82 patients with PD, consecutively diagnosed at the first author's institution, were compared with 246 men visiting the same institution for dermatological diseases. Univariate and multivariate logistic regression analyses were used to assess the data. From the multivariate logistic regression analysis the risk factors for PD were: a history of genital and/or perineal injuries, transurethral prostatectomy, cystoscopy, diabetes mellitus, hypertension, lipoma, propranolol in therapy, Dupuytren's contracture in the medical history, ever having smoked, alcohol consumption, fibromatous lesions of the genital tract of the partner, and surgical intervention on the genital tract of the partner. The results of the present study are in line with the hypothesis that, in addition to genetic predisposition, trauma of the penis and systemic vascular diseases are risk factors for PD. Smoking and alcohol consumption also seem to have some role in the development of the disease.
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To assess the evidence for Borrelia burgdorferi sensu lato infection in patients with lichen sclerosus by focus-floating microscopy. Dermatology department of a university hospital. Tissue sections were stained with a polyclonal B burgdorferi antibody using standard histological equipment and then scanned simultaneously in 2 planes: horizontally in a serpentine-like pattern and vertically by focusing through the thickness of the section, ie, focus-floating microscopy. Part of the material was also investigated by Borrelia-specific polymerase chain reaction. The study population comprised 61 cases of lichen sclerosus and 118 controls (60 negative controls and 68 positive controls). The presence of B burgdorferi sensu lato within tissue specimens. Using focus-floating microscopy, we detected Borrelia species in 38 of 60 cases (63%) of lichen sclerosus and in 61 of 68 (90%) of positive controls of classic borreliosis, but Borrelia species were absent in all negative controls. Borrelia species were detected significantly more often in early inflammatory-rich (31 of 39 [80%]) than in late inflammatory-poor (7 of 21 [33.3%]) cases (P = .001). Polymerase chain reaction findings were positive in 25 of 68 positive controls (37%) and negative in all 11 cases of lichen sclerosus and all 15 negative controls. Focus-floating microscopy is a reliable method to detect Borrelia species in tissue sections. The frequent detection of this microorganism, especially in early lichen sclerosus, points to a specific involvement of B burgdorferi or other similar strains in the development or as a trigger of this disease.
Article
Two patients with lichen sclerosus (et atrophicus) are described in whom there was conspicuous localization of lesions to sites subjected to friction from tight clothing. This was highly suggestive of the Köbner phenomenon. Both patients experienced symptomatic improvement following avoidance of pressure on the skin from tight clothing.
Article
Lichen sclerosus (LS) is a skin disease that may affect both sexes at all ages and at any site. Its etiology remains unknown. The observation of focal koilocytotic-like changes in the stratum malpighii in prepuce samples of LS in children prompted us to investigate the presence of HPV-DNA. Twenty-three paraffin-embedded samples of LS lesions from children aged 4 to 14 years were studied using nested-PCR and in situ hybridization (ISH). Twelve out of 23 cases amplified HPV-DNA (8 cases corresponded to HPV-DNA type 6; 2 cases each to HPV-DNA types 16 and 18). ISH detected HPV sequences in the nuclei of koilocytotic and some parakeratotic cells in 13 cases (9/13 also HPV-DNA positive by PCR). Our results demonstrated the presence of HPV-DNA in roughly 70% of cases of LS of the prepuce in children. We highlight the observation of koilocytotic-like changes in the prepuce and its association with HPV. The possible pathogenetic significance between the virus and the lesion is not settled.
Article
Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases. The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues. The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings. These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.
Article
Lichen sclerosus is a chronic skin condition with a predilection for the genital area. In the present study, 35 male patients with lichen sclerosus were interviewed and examined. Blood screens were performed and histology was requested if not already performed. The findings indicate that lichen sclerosus in males exists as a spectrum of disease, ranging from a mild form with white plaques and few symptoms to a severe form with inflammation, atrophy and scarring with possible urological consequences. In many areas it differs from the condition in females; the association with autoimmune disease is weaker and there is less perianal and extragenital involvement The association with malignancy in males is of lesser significance than initially believed.
Article
Previous reports have demonstrated an association between psoriasis and dyslipidaemia. As lichen planus (LP) is also a chronic inflammatory disorder, we investigated the association between LP and dyslipidaemia in Israel. A case-control study was performed utilizing the database of Clalit Health Services, a large healthcare provider organization in Israel. Patients aged 20-79 years who were diagnosed as having LP were compared with a sample of enrollees without LP regarding the prevalence of dyslipidaemia. Data on other health-related lifestyle factors and comorbidities were collected. The study included 1477 patients with LP and 2856 controls without LP. The prevalence of dyslipidaemia was significantly higher in patients with LP (42.5% vs. 37.8%, P = 0.003; odds ratio, OR 1.21, 95% confidence interval, [CI]: 1.06-1.38). A multivariate logistic regression model demonstrated that LP was significantly associated with dyslipidaemia even after controlling for confounders, including age, sex, smoking, hypothyroidism, diabetes, hypertension, socioeconomic status and obesity (multivariate OR 1.34, 95% CI: 1.14-1.57, P < 0.001). In the present study, LP was found to be associated with dyslipidaemia.
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It is quite clear from our daily experiences that psychosocial stress and emotional factors affects immunity and health. Moreover, from the first pioneer studies in the emerging field of study concerning the relations between emotions and immunity, stress has been commonly associated with immune-suppression. However, several evidence reveal a more complex relationship between emotions and immunity, thus offering new insights for the study of the impact of emotional factors in the onset, development or severity of many disorders, including autoimmune diseases. The present review summarizes background data concerning the crosstalk between brain and immune system and emphasizes the role of cytokines (in addition to the extensive influence of cathecolamines and glucocorticoid hormones) as key-molecules and discusses related patents.
Article
To investigate the prevalence of autoimmune disease and circulating autoantibodies in women with lichen sclerosus (LS) and erosive lichen planus (LP) of the vulva and to compare these with a control population. Age- and sex-matched controlled study. The vulval clinics in Oxfordshire, England, for patients with LS and LP. Healthy controls were recruited from the hospital and community. A total of 190 women with the typical features of adult-onset LS of the vulva, 126 women with adult-onset erosive LP of the vulva, and 922 female controls (of whom 230 were examined). Personal history of autoimmune disorder for patients and controls, family history of autoimmune disorder for vulval LS and LP cohorts, and an autoantibody screen. The presence or absence of a personal or family history of autoimmune disorder, and the presence or absence of 1 or more circulating autoantibodies. The mean ages of patients with LS, patients with erosive LP, and control patients were 63, 61, and 61 years, respectively. The mean age of the 230 controls examined (including those who had serum autoantibodies assayed) was 62 years. Autoimmune disorders were more frequent in patients with erosive LP compared with controls (29% vs 9%; P < .001) and in those with LS compared with controls (28% vs 9%; P < .001). Circulating autoantibodies were more frequent in those with erosive LP compared with controls (41% vs 20%; P < .001). Conclusion This study demonstrates an association of autoimmune disorder and autoantibodies with erosive LP of the vulva and confirms the autoimmune associations of vulval LS.
Article
This article provides a review of empirical evidence linking emotional processes to immune function in humans. Acute stressors have produced mixed effects on immunity, presumably through differential activation of physiological stress systems. Chronic stress has been associated with suppression of immune function, and there is evidence that the immune system may not adapt over time. Effects of stress accompanying social disruption and psychological depression, when demonstrated, have been consistently adverse. Certain personality styles may enhance or degrade immune response. Relationships between psychosocial factors and immunity have been identified for several diseases, including cancer, acquired immune deficiency syndrome, and autoimmune diseases; psychosocial interventions have been tested with variable results. Theoretical and methodological considerations are summarized and directions for future research suggested.
Article
To assess risk factors for vulvar lichen sclerosus data were collected in a case-control study of 75 cases and 225 age-matched control subjects. Questions concerned personal characteristics and habits, gynecologic and obstetric data, general indicators of sexual habits, and selected dietary habits. The risk of vulvar lichen sclerosus was greater in parous women than nulliparous women, but there was little tendency for the risk to increase with number of births; the finding was not significant when only married women were considered. There was no material difference between cases and control subjects in relation to major indicators of sexual habits (age at first intercourse and number of sexual partners). A smaller proportion of cases was married, but no difference was observed in the distribution of cases and controls with reference to education, smoking habits, body mass index, and previous history of diabetes. There was no association between retinoids and risk of vulvar lichen sclerosus, but intake of carotenoids was inversely and strongly associated with vulvar lichen sclerosus. This apparent protection did not materially change after adjustment for socioeconomic status and other potential confounding factors.
Article
A review of 76 patients with lichen sclerosus et atrophicus reveals a number of cases in which this disorder is associated with glucose intolerance or diabetes mellitus. The performance of an oral glucose tolerance test is therefore recommended for all patients with lichen sclerosus et atrophicus.
Article
A study of autoimmune related phenomena in 350 women with histologically confirmed lichen sclerosus et atrophicus revealed that 21.5% had one or more autoimmune related diseases, 21% had one or more first degree relatives with an autoimmune-related disease, 42% had an autoantibody at a titre greater than 1:20, and 59.5% had one or more of these autoimmune-related phenomena. No statistically significant differences in the natural history of lichen sclerosus et atrophicus were demonstrated between those patients with autoimmune-related phenomena and those without.
Article
The development of vulval lichen sclerosus et atrophicus in monozygotic twins is described. This is the first report of the occurrence of lichen sclerosus et atrophicus in two genetically identical individuals, and is considered to provide further evidence that inherited factors are of relevance in the aetiology of this disorder.
Article
We have investigated the presence of autoimmune-related diseases and autoantibodies in twenty-five men with lichen sclerosus et atrophicus (LSA). These patients had a significantly higher incidence of autoimmune-related disorders than a control population and a higher incidence of autoantibodies than would be expected in the normal male population.
Article
Patients with lichen sclerosus and atrophicus have an increased incidence of organ specific antibodies, and these patients and their relatives have a significantly higher incidence of associated autoimmune diseases than a control population. The incidence of other auto-immune diseases in patients with lichen sclerosus is highest when the onset is between the ages of 41 and 6o years, but is not related to the duration or site of the lesions. These findings suggest an immunological basis for the disease and indicate that the patients should be investigated and followed up to detect the existence of other auto-immune disorders.
Article
Objectives: To determine the prevalence of human papillomavirus (HPV) types 6, 11, 16 and 18 in foreskin biopsies from patients with balanitis xerotica obliterans (BXO) and other penile conditions. Materials and methods: Foreskin biopsy specimens from 24 patients with penile lesions and 5 control patients were analysed by type-specific polymerase chain reaction (PCR). Results: HPV6 or HPV16 were not detected in patients with BXO. HPV6 was detected in 2 controls. Conclusions: Genital papillomaviruses do not have a strong association with BXO.
Article
Borrelia burgdorferi has been linked to the pathogenesis of morphea and lichen sclerosus et atrophicus (LSA). However, considerable controversy still exists as to the actual role, if any, that this spirochete plays in the development of these diseases. Antibody titer determinations have been inconclusive and polymerase chain reaction (PCR) studies have yielded conflicting results. We sought to show whether PCR analysis detected B. burgdorferi in archival tissue specimens from the involved skin of 20 North American patients with morphea, 10 patients with LSA, and four patients with scleroderma. We used two different sets of PCR primers for the B. burgdorferi flagellin gene, one specific for European strains of B. burgdorferi, and another common to both European and American strains. A subset of these samples were further amplified with nested PCR primers. None of the samples showed PCR products with either primer sets, whereas purified B. burgdorferi DNA and lesional erythema chronicum migrans tissues, which were used as positive controls, yielded easily detectable products with all primer sets. These data suggest that B. burgdorferi infection plays no role in the development of morphea, LSA, or scleroderma in North American patients; these findings further support the recent observations that B. burgdorferi strain variability is associated with differential spectra of disease in North America compared with that found in various parts of Europe.
Article
Although frequently linked clinically with autoimmune disease, no immunogenetic basis for lichen sclerosus has ever been established. In this study, we examined in detail the HLA antigens of 84 patients with histologically proven disease, compared with 357 controls. Patients with lichen sclerosus did not have the expected HLA A1, B8, DR3, DQ2 autoimmune profile. Instead, DQ7 was present in 39 of 78 (50%) of patients compared with 89 (25%) controls (P < 0.001). In addition, 61 of 78 patients (78%) had either DQ7, DQ8 or DQ9 antigens, or a combination of these, compared with 142 (40%) controls (P < 0.01). Raised levels of DQ7 correspond to a glutamic acid residue at position 45 of the DQB1 locus. Proline amino acids at position 55 of this DQB1 locus could explain the raised levels of DQ7, 8 and 9, and exert a secondary effect. There is preliminary evidence that the immunogenetic profile of patients with this disease may affect disease expression with regard to site and extent of involvement.
Article
Two patients with lichen sclerosus (et atrophicus) are described in whom there was conspicuous localization of lesions to sites subjected to friction from tight clothing. This was highly suggestive of the Köbner phenomenon. Both patients experienced symptomatic improvement following avoidance of pressure on the skin from tight clothing.
Article
Morphea (localized scleroderma), and lichen sclerosus et atrophicus (LSA) share common features with acrodermatitis chronica atrophicans (ACA), a known chronic form of borreliosis. These include similar histologic findings such as diffuse dermal fibrosis. These observations have led several investigators to consider the possibility of Borrelia burgdorferi (Bb) as a common etiologic factor among all of these diseases. The aim of this study is to investigate the role of Bb in the pathogenesis of morphea and LSA, by assaying for its presence in lesional skin biopsies from patients with these diseases. We utilized the nested polymerase chain reaction (PCR) technique to selectively amplify a longer segment of a Bb-specific somatic gene, on DNA from paraffin-embedded, formalin-fixed tissues. The results revealed no Bb-specific DNA sequence in 28 specimens of morphea/scleroderma and 7 of LSA with varying stages of disease. Furthermore, confirmatory Southern blot of the PCR product, resulted in similar findings. These data seriously question the role played by this spirochete in the pathogenesis of morphea and LSA, at least in the southeastern part of the USA.
Article
The aetiology of lichen sclerosus et atrophicus (LSA) is unknown. A series of 42 cases of this uncommon condition is reported. The aim of this study was to identify associations of LSA and document the association with sexual abuse. Information about the patients was obtained by retrospective case note review and some patients were contacted by telephone for further information. In 12 cases there was evidence of sexual abuse. The abused group were slightly older than the non-abused group but were similar in all other respects. All three patients who presented over the age of 12 years had evidence of sexual abuse. Genital trauma was recalled by the patient or found at examination in 17 cases. Evidence of autoimmunity was present in five cases. Positive microbiological isolates were obtained in 18 cases. In only 11 cases were there no associated factors. The symptoms of LSA started between the ages of 3 and 7 years in most patients. The usual symptoms were related to genital skin involvement, and symptoms related to bladder and bowel function were common (50%). In this large series of paediatric LSA, associations with trauma, autoimmunity, and infection were noted. There was a high rate of coexisting sexual abuse with LSA, possibly due to genital trauma.
Article
Lichen sclerosus is a skin disorder of unknown prevalence affecting both men and women, and several studies have established HLA associations in women with this disease. Autoimmune disease associations in the form of a personal and/or family history of autoimmune disease have also been shown to be related to lichen sclerosus. In this study, we examined 58 men (mean age 38 years) with lichen sclerosus, 39 of whom had histologically proven disease. HLA tissue typing by phototyping was performed on these patients and contrasted with that of 602 control subjects. There was no difference in antigen frequencies of the HLA class I loci. The patient group was found to have an increased frequency of several HLA antigens of the class II loci: DR11, 13 of 58 (22%) patients vs. 75 of 602 (13%) control subjects (P = 0.05); DR12, five of 58 (9%) patients vs. 16 of 602 (3%) control subjects (P = 0.04); DQ7, 26 of 58 (45%) patients vs. 189 of 602 (31%) control subjects (P = 0.05). There were few autoimmune disease associations: two of 58 (3%) patients had a personal history of a different autoimmune disease, two patients were found to have abnormal thyroid function and six of 58 (10%) had a first-degree relative with an autoimmune disease. There was no difference in the frequency of the autoimmune haplotype HLA A1, B8, DR3/17, DQ2 compared with the control population. HLA DQ7 has now been shown to occur more frequently in both male and female patients with lichen sclerosus, which may reflect the immunopathogenesis of the disease. Autoimmune disease associations, however, are less common in men with lichen sclerosus.
Article
Lichen sclerosis is a chronic inflammatory skin disease that causes substantial discomfort and morbidity, most commonly in adult women, but also in men and children. Any skin site may be affected (and, rarely, the oral mucosa) but lichen sclerosus is most common in the anogenital area, where it causes intractable itching and soreness. In children, the disorder may be confused with changes seen in sexual abuse. Progression to destructive scarring is common. There is increased risk of developing vulval cancer, and there are links with penile cancer. Patients should be kept under long-term review. Lichen sclerosus can occur without symptoms, and the exact prevalence is uncertain. It occurs most commonly in women at times of low sex hormone output. The underlying cause is unknown, but there seems to be a genetic susceptibility and a link with autoimmune mechanisms. The wart virus and the spirochaete borrelia have been suggested but not substantiated as infective triggers. The Koebner phenomenon is known to occur (lichen sclerosus occurs in skin already scarred or damaged), so trauma, injury, and sexual abuse have been suggested as possible triggers of symptoms in genetically predisposed people. The treatment of choice for anogenital lichen sclerosus is potent topical corticosteroid ointment for a limited time. Circumcision may be indicated in men, and surgery may be considered in women, to relieve effects of scarring or to treat coexisting carcinoma. Current research aims to identify a treatable cause of lichen sclerosus, to identify patients at risk of scarring and of malignant disorders, and to find target pathways for therapeutic intervention.
Article
Genital lichen sclerosus (LS) has sporadically been reported to be associated with penile squamous cell carcinoma (SCC). The purpose of this study was to assess the risk of malignant degeneration in a series of male patients affected by genital LS. All cases of histologically proven epithelial malignancy associated with penile LS recorded in our pathology files over a 10-year period (1987-1997) were reviewed. Assessment for presence of human papillomavirus (HPV) was performed from paraffin-embedded tissues using polymerase chain reaction (PCR). Five of 86 white and uncircumcised men with genital LS (mean age at diagnosis, 53 years; range, 22-83 years) showed malignant or premalignant histopathologic features: 3 had SCC, one had erythroplasia of Queyrat (unifocal SCC in situ), and one verrucous carcinoma. The average lag time from onset of LS was 17 years (range, 10-23 years). Histologically, transition from LS to frank neoplastic foci was evident in all cases of SCC. In these SCC cases, areas of epithelial dysplasia were well evident at the tumor periphery. In the remaining cases, the histologic findings were consistent with erythroplasia of Queyrat and verrucous carcinoma. PCR detected HPV 16 infection in 4 of the 5 cases; one SCC patient was negative for HPV. Malignant changes were associated with 5.8% of the cases of penile LS in our series. Therefore patients with genital LS are at considerable risk of the development of penile SCC, as well as other epithelial and in situ carcinomas, namely verrucous carcinoma and erythroplasia of Queyrat. HPV infection probably plays a major role because 4 of 5 patients were positive for HPV. Histologically, epithelial dysplasia may represent a precancerous stage before the development of neoplasia in atrophic nonproliferative LS lesions, as its presence at the tumor periphery in our SCC biopsy samples seemed to suggest.
Article
We present the clinical and laboratory findings in 60 women and 42 men with lichen sclerosus.
Article
We analyzed the incidence of balanitis xerotica obliterans (BXO) by both age and ethnicity at an equal-access health care facility. We retrospectively reviewed discharge records from 1997 to 1999 at Brooke Army Medical Center to determine ethnicity and age of patients with BXO. Of 153,432 male patients, 108 (0.070%) had a diagnosis of BXO. The age distribution was similar over a range from 2 to 90 years, with the exception of the third decade, when the incidence almost doubled. Black and Hispanic patients had twice the incidence found in white patients (10.59, 10.67 and 5.07 per 10,000 patients, respectively). At our equal-access health care facility, the incidence of BXO in black and Hispanic patients was double that in whites. This unexpected finding, in concert with the greater incidence in the third decade, may result from greater access to medical attention for these patients in the military setting. Nevertheless, further research into the origin of the disease is warranted.
Article
Lichen sclerosus is a chronic skin condition, which offers many challenges to the clinician. It affects men, women and children, and usually occurs in the anogenital area. The clinical signs can be confused with those seen in sexual abuse in children. The underlying cause is unknown; however, there is a strong association with autoimmune disorders, and immunogenetic studies have demonstrated a link with HLA DQ7. Patients suffer significant morbidity as a consequence of the intractable symptoms, physical scarring and psychosexual damage. Support groups may be helpful for some patients. Potent topical corticosteroids have been shown to be effective. There is a 5% incidence of squamous cell carcinoma, and all suspicious lesions should be biopsied. It is unclear whether the risk of malignancy is changed with the use of topical corticosteroids, as there is a potential risk of triggering a latent infection of human papillomavirus. A multidisciplinary approach to care is required and ideally all patients should attend a dedicated clinic and be offered long-term monitoring.
Article
Lichen sclerosus is a common acquired inflammatory disorder of skin and mucous membranes. The aetiology is unknown, although HLA-subtype susceptibility and high rates of other autoimmune disorders suggest that autoantibodies to specific mucocutaneous antigens are involved. The clinicopathological similarities between lichen sclerosus and lipoid proteinosis, which results from mutations in extracellular matrix protein 1 (ECM1), suggest this protein as an autoantigen. We analysed serum autoantibody profiles in 171 individuals (86 with lichen sclerosus, 85 healthy controls) by immunoblotting of extracts from normal human skin and lipoid proteinosis skin (lacking ECM1). We generated a full-length glutathione-S-transferase fusion protein for ECM1 to confirm specific immunoreactivity. We affinity-purified serum from patients with lichen sclerosus and did indirect immunofluorescence microscopy on normal skin with or without preabsorption with recombinant ECM1. By immunoblotting, IgG autoantibodies were found in 20 (67% [95% CI 45-84]) of 30 lichen sclerosus serum samples. The highest titre was 1 in 20. The bands were not detected in ECM1-deficient substrate. These samples, and those from 56 other patients with lichen sclerosus, showed immunoreactivity to the recombinant ECM1 protein (64 of 86 positive; 74% [65-84]). Only six (7% [2-13]) of 85 control serum samples were positive. Affinity-purified IgG from serum of patients with lichen sclerosus labelled skin similarly to a polyclonal antibody to ECM1. The positive staining was blocked by preabsorption with excess recombinant ECM1 protein. These findings provide evidence for a specific humoral immune response to ECM1 in lichen sclerosus and offer insight into disease diagnosis, monitoring, and approaches to treatment.
Article
Although the precise aetiology of lichen sclerosus is unknown, evidence for an autoimmune basis to the disorder is emerging. Indeed, circulating IgG autoantibodies to the glycoprotein extracellular matrix protein 1 (ECM1) have been demonstrated in the sera of about 75% of affected individuals. To assess this humoral immune response further, immunoblotting was performed using bacterial recombinant proteins spanning different domains of the ECM1 protein. The aim was to identify autoantibody-reactive sites recognized by 90 lichen sclerosus sera. The subclass distribution of anti-ECM1 IgG autoantibodies was also determined in 54 lichen sclerosus sera. Immunoblotting showed that the IgG autoantibodies from lichen sclerosus patients recognize multiple antigenic reactive sites on the ECM1 protein within both the amino terminus (50/90, 55.6%) and the protein loop cysteine-rich repeat domains (54/90, 60%), although few sera (7/90, 7.8%) had antibodies to the carboxyl terminus of ECM1. IgG subclass analysis revealed that the anti-ECM1 autoantibodies belong predominantly to the IgG(2) subclass (48/54, 88.9%), either IgG(2) alone (28/54, 51.9%) or in combination with one or more other IgG subclasses. No correlation was found between the site(s) of the ECM1 epitopes or the anti-ECM1 IgG profile and any specific clinical parameters. Nevertheless, characterization of anti-ECM1 antibodies does provide further insight into humoral immune responses and understanding disease mechanisms in lichen sclerosus.
Article
Lichen sclerosus (LS) is a chronic inflammatory disorder of the skin and mucosa, presenting to genitourinary physicians and dermatologists. It affects both sexes and all age groups. Although the exact aetiology is uncertain, genetic predisposition, infections and autoimmune factors have been implicated in its pathogenesis. Symptoms include pruritus and soreness, but asymptomatic presentations are not uncommon. The classical clinical picture is of atrophic white plaques in the anogenital region. Histopathology is specific with basal cell degeneration, upper dermal oedema, homogenization of collagen and a chronic inflammatory infiltrate. Short courses of potent topical corticosteroids form the mainstay of treatment. The condition tends to be remitting and relapsing, with spontaneous regressions reported in a few. In men, the term balanitis xerotica obliterans is sometimes used to describe late and severe LS of the penis. Scarring and progression to squamous cell carcinomas can occur in chronic LS, resulting in significant morbidity. A multidisciplinary approach to care and the need for long-term monitoring cannot be overemphasized.
Article
This prospective study was designed to address the incidence and clinical and histologic characteristics of balanitis xerotica obliterans in a large random pediatric population with phimosis. We investigated 1178 boys who presented consecutively with phimosis between 1991 and 2001. All patients who underwent complete circumcision and surgical specimens were typed histologically as early, intermediate, or late forms of this disorder or as nonspecific chronic inflammation. Patients with balanitis xerotica obliterans were controlled at 1, 6, and 12 months postoperatively, then yearly. Balanitis xerotica obliterans was found in 471 of the 1178 patients (40%), with the highest incidence in boys aged 9 to 11 years (76%). Secondary phimosis occurred in 93% of boys with balanitis xerotica obliterans and in 32% of those without the disorder. In six instances of balanitis xerotica obliterans, meatotomy and in one meatoplasty was performed, as well as circumcision. On histologic evaluation, we found 19% had early, 60% intermediate, and 21% late form of balanitis xerotica obliterans. Glanular lesions disappeared completely within 6 months in 229 out of 231 patients. Our data strongly suggest that the true incidence of childhood balanitis xerotica obliterans is higher than previously assumed. Its incidence peaks in the 9 to 11 years age group, in whom secondary phimosis was almost exclusively caused by balanitis xerotica obliterans.
Article
Two hundred and two patients with clinically typical or biopsy-confirmed vulval lichen sclerosus were reviewed either at consultation (75%) or by retrospectively examining their chart. At diagnosis, 79% were 50 years or older. Ninety-six per cent complained of itching, pain and/or dyspareunia. Lichen sclerosus most often affected the labia minora and perineum but 50% had perianal and 13% had extragenital disease. Thirty-five patients gave a history of psoriasis (17%), which affected the vulval area in 10. Thyroid disease was reported in 39 patients (19%), and 33 gave a family history of thyroid disease. Of those tested (142), 20% had elevated thyroid antibodies. Topical clobetasol propionate was very effective but at least intermittent treatment was required long term in 85%. At follow up, 101 of 185 patients (56%) were asymptomatic but 22 (12%) continued to have moderate-to-severe symptoms. Thyroid disease and psoriasis are common associated conditions.
Article
To investigate the association between lichen sclerosus and thyroid disease in our patient population. This was a retrospective chart review of patients seen between January 1995 and September 2005 with biopsy-proven lichen sclerosus. Charts were reviewed to assess the patients' history of thyroid disease. We identified 211 patients with biopsy-proven lichen sclerosus, 63 (29.9%) of whom had thyroid disease. In women <55 years old, 25 of 74 (33.8%) had thyroid disease; in women > or = 55 years old, 38 of 137 (27.7%) had thyroid disease. The prevalence of thyroid disease in our patients with biopsy-proven lichen sclerosus is almost 30% and is not dependent upon age. This prevalence is 5- to 30-fold greater than in the general population.
Role of Borrelia burgdorferi in the pathogenesis of morphea/scleroderma and lichen sclerosus et atrophicus: a PCR study of thirty-five cases
  • De Vito
Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus
  • Eiselinde
Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus
  • K Eiselinde
  • T Grabner
  • H Kutzner
  • B Zelger
Eiselinde K, Grabner T, Kutzner H, Zelger B. Possible role of Borrelia burgdorferi sensu lato infection in lichen sclerosus. Arch Dermatol 2008; 144:591-8.