RESUMEN

Objective: To report the prevalence of malignant transformation of vulvar lichen sclerosus (VLS) and possible risk factors. Methods: This is a cohort study with data analysis from medical records of 138 patients with histological diagnosis of VLS registered at the Vulvar Pathology Outpatient Clinic of the University Hospital, between 2007 and 2017. Predominance of risk factors was performed using logistic regression analysis. The variables studied were the length of follow-up, age, regular or irregular follow up; presence of symptoms (dyspareunia, pruritus and/or vulvar burning); histology characteristics, the presence of epithelial hyperplasia; and the presence of autoimmune diseases. Results: There were 138 patients included in the study, and among them five progressed to malignant transformation. The patients had a median age of 59 years and 83% were symptomatic. The most frequent symptom was itching with 72%. Autoimmune diseases were present in 11.6%, the most prevalent being thyroid disease. All five case of malignant transformation (0.6%) had an irregular follow up. The logistic regression analysis was used among the studied variables, and no statistical significance was found among them (p ≥ 0.05). The relationship between hyperplasia and the clinical outcome of malignant transformation, in which non-significant but acceptable p value close to 0.05 was observed. Conclusion: The prevalence of malignant transformation in patients with VLS was 0.6%, and common factors were the lack of adherence to medical treatments and the loss of follow-up.

Asunto(s)

Transformación Celular Neoplásica , Liquen Escleroso Vulvar , Humanos , Femenino , Persona de Mediana Edad , Liquen Escleroso Vulvar/epidemiología , Liquen Escleroso Vulvar/complicaciones , Factores de Riesgo , Adulto , Anciano , Estudios de Cohortes , Neoplasias de la Vulva/epidemiología , Neoplasias de la Vulva/patología , Prevalencia , Estudios Retrospectivos , Anciano de 80 o más Años , Adulto Joven

RESUMEN

Abstract Objective To report the prevalence of malignant transformation of vulvar lichen sclerosus (VLS) and possible risk factors. Methods This is a cohort study with data analysis from medical records of 138 patients with histological diagnosis of VLS registered at the Vulvar Pathology Outpatient Clinic of the University Hospital, between 2007 and 2017. Predominance of risk factors was performed using logistic regression analysis. The variables studied were the length of follow-up, age, regular or irregular follow up; presence of symptoms (dyspareunia, pruritus and/or vulvar burning); histology characteristics, the presence of epithelial hyperplasia; and the presence of autoimmune diseases. Results There were 138 patients included in the study, and among them five progressed to malignant transformation. The patients had a median age of 59 years and 83% were symptomatic. The most frequent symptom was itching with 72%. Autoimmune diseases were present in 11.6%, the most prevalent being thyroid disease. All five case of malignant transformation (0.6%) had an irregular follow up. The logistic regression analysis was used among the studied variables, and no statistical significance was found among them (p ≥ 0.05). The relationship between hyperplasia and the clinical outcome of malignant transformation, in which non-significant but acceptable p value close to 0.05 was observed. Conclusion The prevalence of malignant transformation in patients with VLS was 0.6%, and common factors were the lack of adherence to medical treatments and the loss of follow-up.

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BACKGROUND: The incidence of preputial lichen sclerosus (PLS) among children presenting with phimosis varies from 10 to 95%, depending on the age, the protocol for the treatment of pediatric phimosis, the method of diagnosis (clinical versus histological), and case mix (congenital versus acquired phimosis). OBJECTIVE: PLS may not be clinically obvious. Our aim is to show that a systematic histological examination of the prepuce may diagnose PLS in clinically unsuspected cases. METHODS: Prospective observational study of the histology of all prepuces resected from boys undergoing circumcision for phimosis but not clinically suspected to have PLS. RESULTS: PLS was diagnosed histologically in 22 boys (32%). Boys with PLS were significantly older (mean 8.4 versus 4.7 years old). Diagnosis of PLS was not related to the degree of phimosis (summary figure). In three patients (grade 4 phimosis) glans discoloration was observed during surgery, and all had PLS. Follow up for boys found to have PLS ranged from 1 to 10 years. One patient developed recurrent phimosis, attributed to inappropriate conservative resection, and required further surgery. There were no cases of meatal stenosis. DISCUSSION: Subtle cases of PLS may be difficult to detect clinically. Children are frequently asymptomatic, except for being unable to retract the prepuce. Physical examination has a low negative predictive value for the diagnosis of PLS. Complete removal of the prepuce with permanent glans exposure is regarded as essential to cure PLS and to avoid recurrent phimosis, but our patients were treated with partial circumcisions for cultural reasons. Only one needed reoperation for recurrent phimosis. CONCLUSION: Histological PLS was present in approximately 1/3 of boys with phimosis, frequently without typical manifestations. Those patients may be cured with partial circumcisions.

Asunto(s)

Circuncisión Masculina , Liquen Escleroso y Atrófico , Fimosis , Estrechez Uretral , Masculino , Niño , Humanos , Preescolar , Liquen Escleroso y Atrófico/complicaciones , Liquen Escleroso y Atrófico/diagnóstico , Liquen Escleroso y Atrófico/epidemiología , Fimosis/diagnóstico , Fimosis/cirugía , Circuncisión Masculina/efectos adversos , Estrechez Uretral/cirugía , Estudios Prospectivos

RESUMEN

The association between penile lichen sclerosus and striking accumulation of elastic fibers in deep dermis has been described in rare reports, mostly in vulvar lesions. We describe one case of severe balanopreputial adhesions related to lichen sclerosus and this form of elastosis, with no concomitant neoplasia. Aggregates of elastic fibers were seen in deep dermis and in blood vessels. The lesion mirrors nevus elasticus and nevus elasticus vascularis - a well described cutaneous lesion with no known association with lichen sclerosus.

Asunto(s)

Tejido Elástico/patología , Liquen Escleroso y Atrófico/patología , Enfermedades del Pene/patología , Anciano de 80 o más Años , Humanos , Masculino

RESUMEN

Labial adhesions in postmenopausal women are caused by various inflammatory processes in the context of estrogen deficiency. Lichen sclerosus (LS) is a chronic, progressive condition characterized by idiopathic epithelial thinning of the anogenital region with symptoms of significant pruritus, discomfort, and dyspareunia. Early diagnosis and treatment of LS can prevent disease progression, leading to labial adhesions and malignancy. We present an 84-year-old woman with long-standing vulvar Hailey-Hailey disease who developed labial adhesions. Clinical examination with vulvar biopsy revealed histopathological findings consistent with LS. Surgical separation of the labia by blunt dissection was then performed, and clobetasol ointment and vaginal dilatators were postoperatively prescribed. This resulted in a significant improvement in her symptoms. This is the first reported case of LS in a patient with Hailey-Hailey disease.

RESUMEN

La morfea ampollar es un tipo enfrecente de esclerodermia localizada que se caracteriza por presentar ampollas sobre placas escleróticas. La presencia de este tipo de lesiones obliga a descartar la variante extraenital de liquen esclerodemias localizadas, es posible hallar ambas afecciones. Se describe el caso de una paciente de 19 años con diagnóstico de morfea panesclerótica y liquen escleroso ampollar.

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Bollous morphea is an infreqent type of morphea characterized for developing bullae on sclerodermiformic plaques. The presence of bullae forces to discard lichen sclerosus, a disease that usually develops in the genital zone, the extragenital variant could belong to the same spectrum that localized sclerodermiformic diseases. We present a 19 year old female patient with the diagnosis of panesclerotic morphea and bullous lichen sclerosus.

Asunto(s)

Humanos , Femenino , Adulto , Adulto Joven , Esclerodermia Localizada/diagnóstico , Liquen Escleroso y Atrófico/diagnóstico , Esclerodermia Localizada/patología , Piel/patología , Enfermedades Cutáneas Vesiculoampollosas/diagnóstico , Tacrolimus/administración & dosificación , Liquen Escleroso y Atrófico/tratamiento farmacológico , Ácido Micofenólico/administración & dosificación

RESUMEN

The role of circumcision in partially protecting against sexually transmitted infections (STIs) and other dermatoses has been documented. Neonatal circumcision is not routinely practiced in South America. Although it is logical to assume that male genital dermatoses are more prevalent in Hispanic men, they are underrepresented in the existing literature. Objective: To describe the epidemiological characteristics from our male genital dermatology unit in Montevideo (Uruguay), the diagnoses, and correlate them with circumcision status and comorbidities. Methods: A retrospective observational cohort study was conducted. A dermatologist and urologist evaluated all patients using standard questionnaires. In 3 years and 8 months, 269 patients were seen. Median age was 41, prevalence of neonatal circumcision was 0.7%, HIV was 4.2%, STIs were 24.9%, non-STIs were 63.9%, and both (STI + non-STI) were 11.2%. Most frequent entities: eczema/balanoposthitis (27.1%), condyloma (24.9%), and lichen sclerosus (15.6%). Data correlating circumcision and other diagnoses did not reach statistical significance. HIV was positively associated with other STIs (p < 0.05), and an association with balanoposthitis was seen; however, it did not reach statistical significance (p < 0.1). Main limitation was small sample size. This is the first study of its kind based on Hispanic patients. Collaboration between specialties proved to be fundamental. Further studies are needed in this demographic to find an association between circumcision, comorbidities, and genital dermatoses.

Asunto(s)

Circuncisión Masculina , Dermatología , Adulto , Genitales , Hispánicos o Latinos , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos

RESUMEN

RESUMEN Fundamento: el liquen escleroso es una dermatosis inflamatoria crónica de la piel y semimucosas. Afecta principalmente la vulva y la región perianal, la localización extragenital vista entre un 15 y un 20 % de los casos es rara en la infancia. Objetivo: describir una serie de 8 pacientes en edad pediátrica con padecimiento de liquen escleroso en el Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos. Métodos: se realizó un estudio descriptivo, retrospectivo, de serie de casos de 8 pacientes seguidos en consulta externa de dermatología del Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos. La información se extrajo de las historias clínicas de los pacientes. Se tuvo en cuenta la edad expresada en años, sexo, raza, afectación genital, tiempo de evolución en años, síntomas asociados, otras enfermedades asociadas: vitiligo, dermatomiositis, esclerodermia, lupus eritematoso, tipo de lesiones, morfea, fenómeno de koebner y el tratamiento. Resultados: se describieron 8 casos todos en pacientes pediátricos con edad media de 8 años y de evolución de 3,1 años, predominó el sexo femenino, el 37,5 % de la serie presentaron lesiones extragenitales y vitiligo en el 62,5 %, hubo retraso diagnóstico en un paciente. Conclusiones: el liquen escleroso es una patología que afecta la calidad de vida de los pacientes, su posible repercusión sexual, urinaria y su potencial riesgo de carcinoma epinocelular, por lo que se hace necesario realizar un diagnóstico oportuno de este padecimiento.

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ABSTRACT Background: lichen sclerosus is a chronic inflammatory dermatosis of the skin and semi-mucous membranes. It mainly affects the vulva and the perianal region, the extragenital location seen between 15 and 20 % of cases, is rare in childhood. Objective: to describe 8 pediatric patients with lichen sclerosus at the Paquito González Cueto Cienfuegos University Pediatric Hospital. Methods: a descriptive, retrospective study of 8 patients followed in the dermatology outpatient clinic of the Paquito González Cueto University Pediatric Hospital in Cienfuegos was carried out. The information was extracted from the medical records of the patients. Age expressed in years, sex, race, genital involvement, evolution time in years, associated symptoms, other associated diseases were taken into account: vitiligo, dermatomyositis, scleroderma, lupus erythematosus, type of lesions, morphea, koebner and treatment. Results: 8 cases were described, all in pediatric patients with a mean age of 8 years and an evolution of 3.1 years, predominantly female, 37.5 % of the series had extragenital lesions and vitiligo 62.5 %, there were diagnostic delay in a patient. Conclusions: lichen sclerosus is a pathology that affects the quality of life of patients, its possible sexual and urinary repercussions and its potential risk of epinocellular carcinoma, for which it is necessary to make a timely diagnosis.

RESUMEN

OBJECTIVES: To assess the clinical response to and the histomorphometric effects of microablative fractional radiofrequency (MFR) in women with symptomatic vulvar lichen sclerosus (VLS). METHODS: This was a pilot study on the use of MFR for the treatment of VLS. Upon recruitment and at each treatment session, all participants were examined and each of their symptoms were rated on a visual analog scale. After the procedure, the participants completed a satisfaction questionnaire. We compared the morphometric findings of vulvar biopsies performed at enrollment and after the last treatment session. The participants were divided into three groups according to previous treatment with corticosteroids: G1, no previous treatment; G2, treated for up to 5 years; and G3, treated for >5 years. RESULTS: This study included 26 women. After two to three sessions, most participants in all groups became either "asymptomatic" or "much better" than before treatment and were "very satisfied" or "satisfied" with the intervention. Pruritus and burning sensation were the most frequently reported symptoms. Nearly 40% of the participants in all groups reported complete remission of symptoms. The improvement was rated as moderate or higher by 80%, 76%, and 66% of the women in groups 1, 2, and 3, respectively. The improvement of symptoms persisted for 11 months (range, 7-16 months), on average, after the treatment. Type III collagen concentration significantly increased and was associated with important symptom improvement. Tissue trophism and vascularization also increased but did not reach statistical significance, probably because of the small number of cases. CONCLUSIONS: MFR may be an effective and safe treatment for symptomatic VLS.

Asunto(s)

Humanos , Femenino , Liquen Escleroso y Atrófico , Liquen Escleroso Vulvar/terapia , Proyectos Piloto

RESUMEN

Resumen Introducción: los síndromes esclerodermiformes se definen por la presencia de esclerosis cutánea, induración y pérdida de la elasticidad de la piel. Su diagnóstico diferencial incluye casos de escleroderma localizada, dentro de los cuales se describe el liquen escleroso y atrófico que, hasta la fecha, no se ha reconocido en el escenario de síndromes cutáneos paraneoplásicos. Resultados: se presenta el caso de un paciente con adenocarcinoma de próstata avanzado, quién desarrolló lesiones compatibles con liquen escleroso y atrófico extragenital durante el curso de su enfermedad oncológica, confirmadas por histopatología. Conclusiones: Se cuestiona la naturaleza paraneoplásica de esta entidad cutánea en este contexto clínico particular

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Abstract Introduction: Scleroderma syndromes are defined by the presence of skin sclerosis, induration and loss of skin elasticity. Their differential diagnosis includes cases of localized scleroderma, within which lichen sclerosus et atrophicus are described, which, to date, have not been recognized in the setting of paraneoplastic skin syndromes. Results: We present the case of a patient with advanced adenocarcinoma of the prostate, who developed cutaneous lesions compatible with extragenital lichen sclerosus et atrophicus during the course of the cancer, confirmed by histopathology. Conclusions: The paraneoplastic nature of this skin entity is questioned in this particular clinical context.

RESUMEN

O líquen escleroso vulvar (LEV) é uma doença dermatológica crônica de etiologia incerta, caracterizada por prurido intenso e atrofia progressiva. O corticosteroide tópico de longo prazo é o tratamento de primeira linha para LEV. No entanto, esse tratamento requer a colaboração da paciente, está associado a efeitos colaterais adversos e algumas pacientes não respondem aos corticosteroides. O tratamento com tecnologias térmicas e fototérmicas tem sido estudado como terapia alternativa ou complementar para melhorar os sintomas de LEV e o trofismo cutâneo. A radiofrequência fracionada microablativa é usada em dermatologia para melhorar o trofismo tecidual. Também tem sido usada em pacientes ginecológicas para tratar a atrofia vulvovaginal, estimulando a neocolagênese dérmica e a neoelastogênese. Apresentamos o caso de uma mulher de 39 anos com LEV refratária que foi tratada com aplicações locais de radiofrequência fracionada microablativa. Ela apresentou melhora satisfatória dos sintomas e do trofismo vulvar em longo prazo, sem necessidade do uso de corticosteroides.(AU)

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Vulvar lichen sclerosus (VLS) is a chronic dermatological disease of unclear etiology characterized by severe itching and progressive atrophy. Long-term topical corticosteroid is the first-line treatment for VLS. However, this treatment requires patient compliance, is associated with adverse side effects, and some patients do not respond to corticosteroids. Treatment with thermal and photothermal technologies have been studied as alternative or complementary therapies to improve VLS symptoms and skin trophism. Microablative fractional radiofrequency (MFR) is used in dermatology to improve tissue trophism. It has also been used in gynecological patients to treat vulvovaginal atrophy by stimulating dermal neocollagenesis and neoelastinogenesis. We present the case of a 39-year-old woman with refractory VLS who was treated with local applications of microablative fractional radiofrequency. She had satisfactory, long-term, improvement of symptoms and vulvar trophism, and stopped using corticosteroids.(AU)

Asunto(s)

Humanos , Femenino , Adulto , Terapia por Radiofrecuencia , Liquen Escleroso Vulvar/radioterapia , Prurito Vulvar/radioterapia , Atrofia/radioterapia , Corticoesteroides/uso terapéutico , Liquen Escleroso Vulvar/tratamiento farmacológico

RESUMEN

Ultrapotent topical corticosteroids and circumcision are usually effective for male genital lichen sclerosus (MGLSc); however, refractory cases are often referred to our Male Genital Dermatology Unit. Treatment with autologous platelet-rich plasma (TPRP) has recently been advocated as a safe and effective treatment option, but there have been no prospective studies in men to date. The objective of this study is to assess the safety and efficacy of TPRP for MGLSc resistant to conventional therapy. A prospective, open-label, single-arm, therapeutic study was carried out in this study. Inclusion criteria: resistant to conventional therapy for at least 6 months. Procedure: infiltration of 0.1 mL/cm2 PRP every 8 weeks. Monthly data recording: visual appearance with photographs and external scoring by an expert using Investigator's Global Assessment Scale (IGA scale 0-5), symptoms (scale 0-5), quality of life (QoL; Dermatology Life Quality Index [DLQI]), and complications. No. of patients included was n = 5. No. of patients excluded during treatment was n = 1. Mean initial IGA: 3.6. Mean initial DLQI: 6. TPRP n = 34 (range: 2-9; average: 6.8 per patient). Mean IGA at 18 months: 3.25. Mean DLQI at 18 months: 1.25. All patients reported being completely asymptomatic at 10 months. No. of patients with complications is n = 1 (balanitis). TPRP seems to be safe and effective, regarding symptom control and improvement in QoL; however, visual changes were minimal.

Asunto(s)

Circuncisión Masculina , Liquen Escleroso y Atrófico , Plasma Rico en Plaquetas , Humanos , Liquen Escleroso y Atrófico/diagnóstico , Liquen Escleroso y Atrófico/terapia , Masculino , Estudios Prospectivos , Calidad de Vida

RESUMEN

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

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La morfea o esclerodermia localizada es una enfermedad poco común del tejido conectivo que se manifiesta con esclerosis localizada de la piel y, en algunos casos, con lesiones extracutáneas. Su etiología no se comprende por completo, pero se cree que hay predisposición genética, además de factores ambientales desencadenantes. La clasificación de la enfermedad no es sencilla debido a las múltiples presentaciones, sin embargo, es útil para definir el tratamiento, el cual debe individualizarse e iniciarse tempranamente para evitar complicaciones cosméticas y funcionales. En esta revisión resumimos los aspectos prácticos más importantes de la clasificación, métodos diagnósticos y de evaluación de actividad en morfea, así como las opciones terapéuticas disponibles, con énfasis en la evidencia clínica existente respecto a su eficacia y seguridad.

Asunto(s)

Enfermedades Raras , Esclerodermia Localizada , Interacción Gen-Ambiente , Predisposición Genética a la Enfermedad , Humanos , Enfermedades Raras/clasificación , Enfermedades Raras/diagnóstico , Enfermedades Raras/patología , Enfermedades Raras/terapia , Esclerodermia Localizada/clasificación , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patología , Esclerodermia Localizada/terapia

RESUMEN

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Asunto(s)

Humanos , Masculino , Femenino , Esclerodermia Localizada/clasificación , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiología , Esclerodermia Localizada/terapia , Fototerapia/métodos , Pronóstico , Índice de Severidad de la Enfermedad , Factores Sexuales , Terapia por Ejercicio , Inmunosupresores

RESUMEN

Lichen sclerosus is an uncommon chronic inflammatory disease, which rarely affects the oral mucosa. Here, we describe the occurrence of oral lichen sclerosus with gingival destruction in a 12-year-old female patient. After diagnostic confirmation by histopathology, intralesional injection of corticosteroid was performed, producing satisfactory remission of the lesion.

Asunto(s)

Liquen Escleroso y Atrófico/tratamiento farmacológico , Liquen Escleroso y Atrófico/patología , Enfermedades de la Boca/tratamiento farmacológico , Enfermedades de la Boca/patología , Niño , Femenino , Glucocorticoides/uso terapéutico , Humanos , Inyecciones Intralesiones , Triamcinolona Acetonida/uso terapéutico

RESUMEN

PURPOSE: Evaluate the main etiologies and clinical characteristics of male urethral stricture disease (USD) in Brazil. METHODS: This multicentric study was performed using retrospective data collected from six Brazilian referral centers of urethral reconstruction. The database comprised data from 899 patients with USD who had undergone surgical treatment from 2008 to 2018. Age, stricture site and primary stricture etiology were identified for each patient. RESULTS: The mean age was 52.13 ± 16.9 years. The most common etiology was iatrogenic (43.4%), followed by idiopathic (21.7%), trauma (21.5%) and inflammatory (13.7%). Of the iatrogenic causes, 59% were secondary to urethral instrumentation (60% by urethral catheterization and 40% by transurethral procedures), 24.8% by other procedures (prostatectomy, radiotherapy, postectomy) and 16.2% by failed hypospadia repairs. Pelvic fracture urethral distraction injuries were responsible for most of the trauma-related strictures (62.7%). When stratified by age, the most common stricture etiology was trauma in the 0-39 years old group (42.8%), idiopathic in the 40-59 years old group (32.4%) and iatrogenic in patients over 60 years old (68%). In regard to the stricture site, 80% presented with an anterior urethral stricture and 20% with a posterior stenosis. In the anterior stenosis group, the most common stricture site was bulbar (39.5%). CONCLUSION: In Brazil, as in many developed countries, the most common cause of urethral stricture diseases is iatrogenic, especially urethral catheterization. These findings emphasize the need of a careful urethral manipulation and a better training of healthcare professionals. Trauma is still responsible for a great proportion of strictures and inflammatory etiologies are now less frequently observed.

Asunto(s)

Países en Desarrollo , Enfermedad Iatrogénica/epidemiología , Complicaciones Posoperatorias/epidemiología , Estrechez Uretral/epidemiología , Adolescente , Adulto , Distribución por Edad , Anciano , Anciano de 80 o más Años , Brasil/epidemiología , Niño , Preescolar , Fracturas Óseas/complicaciones , Humanos , Hipospadias/cirugía , Liquen Escleroso y Atrófico/complicaciones , Liquen Escleroso y Atrófico/epidemiología , Masculino , Persona de Mediana Edad , Huesos Pélvicos/lesiones , Complicaciones Posoperatorias/etiología , Estudios Retrospectivos , Estrechez Uretral/etiología , Uretritis/complicaciones , Uretritis/epidemiología , Cateterismo Urinario/efectos adversos , Procedimientos Quirúrgicos Urológicos Masculinos/efectos adversos , Adulto Joven

RESUMEN

Lichen sclerosus (LSc) with penile cancer is found in about two thirds of specimens. It has been hypothesized that LSc represents a precancerous condition. To qualify as such, in addition to cytological atypia and similarity with the invasive tumor, a spatial correlation between LSc and neoplastic lesions needs to be demonstrated. The purpose of this study was to evaluate such a spatial relationship. Circumcision (28 cases) and penectomy (81 cases) specimens were evaluated. All cases had LSc, penile intraepithelial neoplasia (PeIN), and/or invasive squamous cell carcinomas. We examined LSc in relation to invasive carcinoma, PeIN, and normal epithelia. Invasive squamous cell carcinomas, classified according to the World Health Organization criteria as non-human papillomavirus (HPV)-related and HPV-related PeIN, were present in 100 cases. Non-HPV-related (differentiated) PeIN was the most common subtype associated with LSc (89%). There were 5 spatial patterns identified: (1) LSc adjacent to PeIN (23%), (2) LSc adjacent and comprising PeIN (42%), (3) LSc next to and within invasive carcinomas (8%), (4) LSc throughout the sequence PeIN-invasive carcinoma (24%), and (5) LSc was separate (with normal tissue between the lesions) from PeIN and/or invasive carcinomas in a minority of cases (3%). LSc within the cancer was not previously described. In this series, we found 35 cases with LSc within invasive carcinomas. The striking continuous spatial relationship among LSc, PeIN, and/or invasive carcinoma as shown in this study may be a necessary (but not sufficient) condition for the hypothesis postulating LSc as a penile precancerous lesion.

Asunto(s)

Carcinoma in Situ/patología , Carcinoma de Células Escamosas/patología , Liquen Escleroso y Atrófico/patología , Neoplasias del Pene/patología , Lesiones Precancerosas/patología , Carcinoma in Situ/cirugía , Carcinoma de Células Escamosas/cirugía , Circuncisión Masculina , Epitelio/patología , Humanos , Liquen Escleroso y Atrófico/cirugía , Masculino , Neoplasias del Pene/cirugía , Pene/patología , Pene/cirugía , Lesiones Precancerosas/cirugía

RESUMEN

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Asunto(s)

Esclerodermia Localizada , Terapia por Ejercicio , Femenino , Humanos , Inmunosupresores , Masculino , Fototerapia/métodos , Pronóstico , Esclerodermia Localizada/clasificación , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiología , Esclerodermia Localizada/terapia , Índice de Severidad de la Enfermedad , Factores Sexuales

RESUMEN

RESUMEN El liquen escleroso y atrófico (LEA), es una enfermedad muco-cutánea inflamatoria crónica cuya etiología es desconocida. Se lo ha vinculado con diversos factores: inmunológicos, genéticos, hormonales, infecciosos y traumáticos. Su localización habitual es la genital. Las formas extra genitales son poco frecuentes y suelen afectar tronco, cuello y extremidades. Se presenta un caso de liquen escleroso y atrófico de localización extra genital, en una paciente de 14 años de edad.

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SUMMARY Lichen sclerosus et atrophic (LSA) is a chronic inflammatory mucocutaneous disease of unknown etiology. It has been linked to various immunological, genetic, hormonal, infectious and traumatic factors. Its usual location is the genital area. Extra-genital forms are infrequent and usually involve trunk, neck, and extremities. We present the case of a 14 years old female with lichen sclerosus et atrophic of extragenital localization.