lichen sclerosus et atrophicus

e u r o p e a n u r o l o g y 5 5 ( 2 0 0 9 ) 9 4 5 -9 5 6 a v a i l a b l e a t w w w . s c i e n c e d i r e c t . c o m j o u r n a l h o m e p a g e : w w w . e u r o p e a n u r o l o g y . c o m Abstract Background: Surgical options in male patients with genital lichen sclerosus (LS) involving the anterior urethra still represent a challenging issue. Objective: To review the outcome of surgical treatment in patients with genital and urethral LS. Design, setting, and participants: Multicenter, international, retrospective, observational descriptive study performed in two specialized centers. Two hundred fifteen male patients underwent surgery for histologically proven genital LS involving the foreskin and/or the anterior urethra. Intervention: Circumcision (34 cases), meatotomy (15 cases), circumcision and meatotomy (8 cases), one-stage penile oral mucosal graft urethroplasty (8 cases), two-stage penile oral mucosal graft urethroplasty (15 cases), one-stage bulbar oral mucosal graft urethroplasty (88 cases), and definitive perineal urethrostomy (47 cases). Measurements: Primary outcome was considered a failure when any postoperative instrumentation was needed, including dilation, or when recurrence was diagnosed. Results and limitations: The average follow-up was 56 mo (range: 12-170 mo). Circumcision showed 100% success rate with no recurrence of the disease; meatotomy, 80% success rate; circumcision and meatotomy, 100% success rate; one-stage penile oral mucosal graft urethroplasty, 100% success rate; two-stage penile oral mucosal graft urethroplasty, 73% success rate; one-stage bulbar oral mucosal graft urethroplasty, 91% success rate; and definitive perineal urethrostomy, 72% success rate. Limitations include short follow-up for recording neoplastic degeneration and no instrument to investigate quality of life. Conclusions: Patients with LS disease restricted to the foreskin and/or external urinary meatus showed a high surgery success rate. In patients with penile urethral strictures or panurethral strictures, the use of one-stage oral graft urethroplasty showed greater success than the staged procedures.

Squamous cell carcinoma of the penis arising on lichen sclerosus et atrophicus is rarely reported. A case is described in a 63year-old man who had been diagnosed of lichen sclerosus et atrophicus 3 years before. The previously reported cases, the association of these diseases in women and men, and the possible pathogenesis are discussed.

e u r o p e a n u r o l o g y 5 5 ( 2 0 0 9 ) 9 4 5 -9 5 6 a v a i l a b l e a t w w w . s c i e n c e d i r e c t . c o m j o u r n a l h o m e p a g e : w w w . e u r o p e a n u r o l o g y . c o m Abstract Background: Surgical options in male patients with genital lichen sclerosus (LS) involving the anterior urethra still represent a challenging issue. Objective: To review the outcome of surgical treatment in patients with genital and urethral LS. Design, setting, and participants: Multicenter, international, retrospective, observational descriptive study performed in two specialized centers. Two hundred fifteen male patients underwent surgery for histologically proven genital LS involving the foreskin and/or the anterior urethra. Intervention: Circumcision (34 cases), meatotomy (15 cases), circumcision and meatotomy (8 cases), one-stage penile oral mucosal graft urethroplasty (8 cases), two-stage penile oral mucosal graft urethroplasty (15 cases), one-stage bulbar oral mucosal graft urethroplasty (88 cases), and definitive perineal urethrostomy (47 cases). Measurements: Primary outcome was considered a failure when any postoperative instrumentation was needed, including dilation, or when recurrence was diagnosed. Results and limitations: The average follow-up was 56 mo (range: 12-170 mo). Circumcision showed 100% success rate with no recurrence of the disease; meatotomy, 80% success rate; circumcision and meatotomy, 100% success rate; one-stage penile oral mucosal graft urethroplasty, 100% success rate; two-stage penile oral mucosal graft urethroplasty, 73% success rate; one-stage bulbar oral mucosal graft urethroplasty, 91% success rate; and definitive perineal urethrostomy, 72% success rate. Limitations include short follow-up for recording neoplastic degeneration and no instrument to investigate quality of life. Conclusions: Patients with LS disease restricted to the foreskin and/or external urinary meatus showed a high surgery success rate. In patients with penile urethral strictures or panurethral strictures, the use of one-stage oral graft urethroplasty showed greater success than the staged procedures.

Objective: The aim of this study was to assess the effectiveness of using topical steroid-based creams in the management of clinically diagnosed balanitis xerotica obliterans (BXO) in boys. Methods: The response in 56 boys to locally applied steroid-based creams was studied retrospectively. All had been referred by their general practitioners with symptoms of penile pathology and then subsequently had a clinical diagnosis of BXO made. The boys were initially assessed after 3 months of treatment and then at various intervals depending on their response. Results: The mean age at presentation of the boys was 8.9 (4 to 15) years. After the initial 3-month assessment, 10 (17.9%) boys had complete resolution. When the treatment period was prolonged by an average of 14 months, the total number of boys showing resolution increased to 17 (30.4%). All boys showing resolution had clinically mild BXO limited to the prepuce only. Those patients with established scar formation on the foreskin showed no significant improvement. Conclusions: Topical steroids seem to offer a reliable option only in the management of mild BXO limited to the prepuce in boys with minimal scar formation. Steroid-based creams can be offered to such patients provided they and their families are realistic as to the likelihood of success. This treatment is ineffective in the face of established scarring. D

Anogenital malignancies and premalignancies are an important personal/public health problem due to their effects on individuals' physical, mental, and sexual health. Also, due to their etiological association with human papillomavirus (HPV) infection, anogenital malignancies and premalignancies constitute an immense public health burden. In addition to HPV infection, immunosuppression, HIV infection, chronic dermatoses, such as lichen sclerosis, previous radiotherapy and chemotherapy treatments, and smoking, are the other important etiopathologic factors in the development of anogenital malignancies and premalignancies. The incidence of anal squamous cell carcinoma (SCC) has increased considerably in the past decade, mainly due to the growing number of cases in high-risk groups, such as men who have sex with men, immunosuppressed individuals, and patients with HIV infection. Also, an increase in vulvar intraepithelial neoplasia (VIN) and VIN-related invasive vulvar cancer has been noted in women younger than age 50 years due to its association with HPV infections over the past decade. SCC of the scrotum seems to be the first cancer linked to occupational exposure. Bowen's disease, Bowenoid papulosis, and erythroplasia of Queyrat are the most widely seen premalignancies of anogenital region and are all forms of squamous intraepithelial neoplasia. Histopathologically, these conditions share identical histologic features of SCC in situ, but their clinical features differ. Early diagnosis is vital to improve prognosis, especially in anogenital malignancies. Also, if a delay occurs in diagnosis, treatment options used will be associated with significant negative effects on the patient's psychological well-being and quality of life; hence, management of anogenital malignancies and premalignancies should be organized in a multidisciplinary fashion.

Vulvar lichen sclerosus (LS), a lymphocyte-mediated chronic skin disease, begins with uncharacteristic symptoms and progresses undiagnosed to atrophy and destructive scarring. Some patients with longstanding advanced LS have an increased risk of vulvar carcinoma. Early LS is treatable, although not curable, if diagnosed early. Therefore, patients with persistent vulvar symptoms should be biopsied to establish the diagnosis. In contrast to advanced LS, the histological features in early LS are quite subtle and often more prominent in adnexal structures than in interfollicular skin. Adnexal structures show acanthosis, luminal hyperkeratosis and hypergranulosis with ⁄ without dystrophic hair and basement membrane thickening. The epidermis ⁄ mucosa shows mild irregular, occasionally psoriasiform acanthosis and focal basement membrane thickening. Early dermal changes are homogenized collagen and wide ectatic capillaries in dermal papillae immediately beneath the basement membrane. The lymphocytic infiltrate can be sparse or dense, lichenoid or interstitial with epidermal lymphocyte exocytosis and lymphocytic ⁄ lymphohistiocytic vasculitis. Dermal melanophages indicate preceding keratinocyte ⁄ melanocyte destruction. Biopsy specimens of early LS rarely display all features. Therefore, serial sections and periodic acid-Schiff reactions are necessary for their identification. Recognition and treatment of these early stages of LS may result in longstanding remission. Progression to atrophic stages with their associated morbidity and even to squamous cell carcinoma may be prevented.

Histopathology 62, 161-175 HPV-independent. Although large, multicentre studies are needed to definitively assess the involvement of HPV in the prognosis of VSCC, most studies have not found clear differences in survival between HPV-associated and HPV-independent tumours.

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.

Background
Lichen sclerosus is an inflammatory dermatosis of unknown etiology which currently has no cure. Most treatment guidelines recommend the use of ultrapotent topical corticosteroids. However, the relapse rate is usually high. Through a retrospective study we evaluated the efficacy and safety of stromal vascular fraction of adipose tissue as therapy for lichen sclerosus.
Material and methods
For this retrospective review, we obtained data on patients with vulvar lichen sclerosus treated with autologous fat grafting enriched with adipose derived stromal vascular fraction cells. Data collected through a modified vulvo-vaginal symptoms questionnaire were analyzed before treatment, six months and 24 months after treatment. The 19 items questionnaire was subdivided in four categories: symptoms, signs, social functioning and sexual functioning. Global scores and partial scores for each category were analyzed using paired t-test. For all statistical analyses, a value of p ≤ 0.05 was considered statistically significant. All data are presented as mean ± SD.
Results
Thirty nine patients were included in the study. Thirty seven patients (94.87%) experienced a significant decrease in global score at six months and 24 months after treatment (p < 0.05). Decrease in scores were also statistically significant between pretreatment and 24 months after treatment for each of the four questionnaire categories - symptoms, signs, social functioning and sexual functioning (p < 0.05).
Conclusions
This retrospective study showed that the use of autologous fat grafting enriched with adipose derived stromal vascular fraction is safe and leads to significant and long lasting improvement in patients with vulvar lichen sclerosus.

Aims: To analyse 90 vulvar and 72 penile cases of lichen sclerosus (LS) on haematoxylin and eosin sections for vascular changes and the vascular infiltrates immunohistochemically with antibodies to T cells, B cells and antigen-presenting dendritic cells. LS is a skin disease of presumed autoimmune origin. Autoimmune diseases are mediated by lymphocytes which occasionally produce a lymphocytic vasculitis. Methods and results: Three types of lymphocytic infiltrates were identified: (i) perivascular lymphocytic infiltrates without damage to vessel walls; (ii) lymphocytic vasculitis in three forms: (a) concentric lymphohistiocytic infiltrates with lamination of the adventitia by basement membrane material which was typical for penile LS; (b) lymphocytic vasculitis with dense perivascular lymphocytic cuffing with occasional fibrin deposition in vessel walls and subendothelial lymphocyte infiltration, quite common in vulvar LS; and (c) intramural lymphocytic infiltrates in large muscular vessels; (iii) leukocytoclastic vasculitis in LS was exceptionally rare. In lymphocytic vasculitis, CD20+ B cells, CD4+ T cells and dendritic cells were the principal infiltrating cells. Conclusions: Dendritic cells capture (foreign) antigens after entry into the affected tissues and initiate immune responses acting as a matrix on which antigen-specific T and B cells interact. The described vascular features are indicative of antigen-mediated vasculitic changes in LS.

Background 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.

The known spectrum of skin manifestations in cutaneous Lyme disease is continuously expanding and can not be regarded as completed. Besides the classical manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially morphea, lichen sclerosus and cases of cutaneous B-cell lymphoma are causally related to infections with Borrelia. Also granuloma annulare and interstitial granulomatous dermatitis might be partly caused by Borrelia burgdorferi or similar strains. There are also single reports of other skin manifestations to be associated with borrelial infections like cutaneous sarcoidosis, necrobiosis lipoidica and necrobiotic xanthogranuloma. In addition, as the modern chameleon of dermatology, cutaneous borreliosis, especially borrelial lymphocytoma, mimics other skin conditions, as has been shown for erythema annulare centri...

Background: Narrowing of the prepuce in men is poorly documented, and the causes are often unknown, except in the case of clinical infections or skin diseases such as lichen sclerosus (LS). Objective: We conducted a histologic study of circumcision specimens with phimosis or paraphimosis. Methods: This prospective study included 43 men with contraction referred for circumcision. Results: LS was present in 32% of cases, but only 12% of these cases of LS had not been diagnosed before circumcision. In 31% of cases the histologic findings were normal. Subacute nonspecific inflammatory changes were diagnosed in 37% of all cases, and secondary narrowing of the prepuce in 62% of cases. It is probable that this histologic modification of the preputial mucosa is involved in narrowing of the prepuce. Conclusion: Phimosis in young adults is usually not associated with LS (only 14%). In contrast, most older patients had secondary phimosis caused by progressive LS (40%) or subacute nonspecific inflammatory changes (40%). Although all cases of phimosis in men should be treated by complete circumcision to prevent penile cancer, paraphimosis associated with preputial dyspareunia, with the exception of cases associated with LS, can be treated by corrective surgery.

To assess the effectiveness of treating premenarchal vulvar lichen sclerosus with clobetasol propionate. METHODS: A retrospective chart review was performed of girls presenting to the University of Michigan Pediatric and Adolescent Gynecology Clinic from January, 1995, to July, 2000, with premenarchal lichen sclerosus. Subjects in the study were treated with topical clobetasol propionate ointment 0.05% for 2-4 weeks, and then tapered to a less potent steroid. Information was extracted concerning age at onset, symptoms, vulvar examination, previous treatments, effectiveness of clobetasol, follow-up, and complications. The parents were contacted for a follow-up telephone survey. RESULTS: Fifteen girls averaging 5.7 years at the start of symptoms met criteria. The diagnosis of lichen sclerosus was made visually in 11 and by biopsy in four. Follow-up ranged from 2 months to 6 years. Fourteen girls had good improvement within 4-7 weeks. One girl developed a yeast superinfection and one developed transient erythema. At least 1 year of follow-up by clinic visit or telephone interview was available in 11 girls. Of these 11, two girls had no further vulvar symptoms after the initial treatment, five had one or two total flares, three reported three to eight flares per year, and one girl continues to be unresponsive to therapy. CONCLUSION: Clobetasol propionate was an effective treatment of premenarchal vulvar lichen sclerosus in this small group; however, recurrences were common and required additional steroid treatment. Furthermore, complications of treatment were infrequent, minor, and easily treatable.

To determine the practice patterns of urologists who treat Male Genitourinary Lichen Sclerosus (MGU-LS) via a national web-based survey distributed to AUA members. A twenty-question survey was collected from a random sample of AUA members. Respondents answered questions on their practice patterns for MGU-LS diagnosis, treatment of symptomatic urethral stricture disease, surveillance and follow-up. In total, 309 urologists completed the survey. The majority of respondents reported practicing more than 20+ years (37.5%) within an academic (31.7%) or group practice (31.1%) setting. The majority of respondents saw 3-5 men with MGU-LS per year (32.7%). The most common locations of MGU-LS involvement included the glans penis (66.2%), foreskin (26.3%) and/or the urethra (5.8%). Respondent first line treatment for urethral stricture disease was direct visual internal urethrotomy (26.6%) and second line was referral to subspecialist (38.4%). After controlling for the number of patients evalu...

Lichen sclerosus et atrophicus (LSA) is a chronic mucocutaneous inflammatory disorder of uncertain aetiology that is clinically characterized by the appearance of well delimited white plaques or papules, preferentially affecting the skin and genitals, and more rarely the oral cavity. We present the case of a woman with LSA limited to the oral cavity in the form of a well delimited, flat whitish lesion affecting the vestibular gingiva of the right upper incisors and left central incisor, and extending towards the vestibular fundus and frenulum of the upper lip. Widening of the periodontal space was observed, with gingival recession and attachment loss limited to these teeth. Local corticosteroid injections caused the mucosal lesion but not the periodontal alterations to resolve. Emphasis is placed on the importance of knowledge of this condition in relation to establishing a diagnosis, and on its periodontal repercussions.

In the present study, Borrelia spirochetes were found in four (26.6%) out of 15 patients with Atrophoderma of Pasini and Pierini (IAPP) and lichen sclerosis et atrophicans (LSA) from the Brazilian Amazon Region. Borreliosis was investigated by immunohistochemistry and focus floating microscopy for Borrelia burgdorferi in skin biopsy samples from 15 patients with both clinical and histopathology evidences compatible with Morphea, LSA, and IAPP. Spirochetes were detected by specific immunohistochemistry and focus floating microscopy for B. burgdorferi in samples from three patients. A limitation of our study was the fact that we were not able to isolate and culture these organisms. Our data confirm the presence of borreliosis cases in the Amazon.

Background: The association between lichen sclerosus and vulvar squamous cell carcinoma (VSCC) has long been recognized, but large epidemiologic studies are lacking. Methods: Data of women diagnosed with vulvar pathology in the Netherlands were retrieved from the Dutch Pathology Registry. All vulvar pathology reports of this historical cohort were reviewed to construct a research database, including 3,038 women with lichen sclerosus diagnosed between 1991 and 2011. The incidence rate of lichen sclerosus and the cumulative incidence of VSCC among women with lichen sclerosus were estimated. Results: Between 1991 and 2011, the incidence rate of lichen sclerosus increased from 7.4 to 14.6 per 100,000 woman-years. The median age at time of lichen sclerosus diagnosis was 59.8 years and the cumulative VSCC incidence was 6.7%. The 10-year VSCC incidence in women with lichen sclerosus was associated with concurrent vulvar intraepithelial neoplasia (VIN; 18.8% in women with VIN and 2.8% in women without VIN) and age at time of lichen sclerosus diagnosis (5.9% in women of !70 years, 3% in women between 50 and 70 years, and 1.8% in women <50 years). The effects of presence of VIN and age remained significant in adjusted Cox regression analysis. Conclusion: This historical cohort showed a nearly 100% increase in incidence of lichen sclerosus between 1991 and 2011. Concurrent VIN and age !70 years at time of lichen sclerosus diagnosis are important risk factors for vulvar cancer development. Impact: The incidence of lichen sclerosus is rising and special attention is needed in particular in women with concurrent VIN because of their high risk of cancer. Cancer Epidemiol Biomarkers Prev; 25(8); 1224-30. Ó2016 AACR.

Objectives. Strictures due to lichen sclerosus (LS) may affect the urethra as far proximally as the mid-bulb. For such strictures, a staged full-length repair is required and should use a nonpenile graft source such as buccal mucosa. Many cases occur in a population already accustomed to seated voiding, leading us to re-evaluate this approach and, in some circumstances, recommend definitive perineal urethrostomy alone. Methods. We reviewed the medical records and retrograde urethrograms of all patients undergoing surgery for LS at our facilities between January 1991 and June 2002. Results. A total of 63 patients, with an average age of 54.2 years, underwent surgery for LS stricture with an average follow-up of 38.5 months (range 4 to 117). Of the 63 patients, 19 underwent grafting in preparation for future reconstruction. Of these, 11 completed the second-stage repair, and 8 patients elected not to undergo the second stage of the repair, leaving a functional perineal urethrostomy. This led us to look more critically at definitive perineal urethrostomy alone for some patients. Parallel with the staged repairs, and subsequent to them, 44 patients underwent perineal urethrostomy alone. Conclusions. The often extensive nature of LS, the prevailing philosophy that urethroplasty must use nonpenile skin, the limited availability of such sources, and the acceptance of many patients for seated voiding makes definitive perineal urethrostomy alone a viable treatment option. In all our cases, this satisfied patients' quality of life concerns, leaving the anterior urethra dry and amenable to future repair. Younger men desirous of penile voiding should still be considered for staged repair using current techniques. UROLOGY 64: 565-568, 2004.

Objectives. Strictures due to lichen sclerosus (LS) may affect the urethra as far proximally as the mid-bulb. For such strictures, a staged full-length repair is required and should use a nonpenile graft source such as buccal mucosa. Many cases occur in a population already accustomed to seated voiding, leading us to re-evaluate this approach and, in some circumstances, recommend definitive perineal urethrostomy alone. Methods. We reviewed the medical records and retrograde urethrograms of all patients undergoing surgery for LS at our facilities between January 1991 and June 2002. Results. A total of 63 patients, with an average age of 54.2 years, underwent surgery for LS stricture with an average follow-up of 38.5 months (range 4 to 117). Of the 63 patients, 19 underwent grafting in preparation for future reconstruction. Of these, 11 completed the second-stage repair, and 8 patients elected not to undergo the second stage of the repair, leaving a functional perineal urethrostomy. This led us to look more critically at definitive perineal urethrostomy alone for some patients. Parallel with the staged repairs, and subsequent to them, 44 patients underwent perineal urethrostomy alone. Conclusions. The often extensive nature of LS, the prevailing philosophy that urethroplasty must use nonpenile skin, the limited availability of such sources, and the acceptance of many patients for seated voiding makes definitive perineal urethrostomy alone a viable treatment option. In all our cases, this satisfied patients' quality of life concerns, leaving the anterior urethra dry and amenable to future repair. Younger men desirous of penile voiding should still be considered for staged repair using current techniques. UROLOGY 64: 565-568, 2004.

Lichen sclerosus (LS) is a chronic inflammatory skin condition usually located in the anogenital area. Topical corticosteroid therapy is the first choice treatment which may arrest or delay the progression of the disorder. We report the case of a 74-year-old man presented with a 6-month history of nodular lesions localized on penis. The man had a previous history of genital lesions that had been diagnosed as LS and treated with long-term topical corticosteroid therapy. After 3 months of corticosteroid therapy, the patient observed the appearance of several nodular erythematous lesions on the penis with progressive disappearance of the clinical symptoms of LS. These purple to red asymptomatic angiomatoid nodules resembled the clinical features of Kaposi sarcoma.

Lichen sclerosus is a chronic inflammatory disease of unknown origin, which affects mostly women in the fifth and sixth decades of life, but can also occur in men and children. The involvement of the oral mucosa alone or together with other forms of presentation is extremely rare, requiring a differential diagnosis with other diseases of the oral cavity, particularly lichen planus. There are less than 30 cases of lichen sclerosus in the oral mucosa described in the literature and there are no reports on malignant transformation so far. We describe a patient with skin, oral and genital lesions of lichen sclerosus.

Background: The histogenesis of extramammary Paget's disease Ya-Hui Chen, Tak-Wah Wong and (EMPD) is still controversial. Benign pagetoid cells of the nipple first J. Yu-Yun Lee described by Toker and the similar clear cells found in white maculo-Department of Dermatology, National Chengpapules of clear cell papulosis (CCP) have been proposed to be po