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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.
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Tecido Elástico/patologia , Líquen Escleroso e Atrófico/patologia , Doenças do Pênis/patologia , Idoso de 80 Anos ou mais , Humanos , MasculinoRESUMO
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.
Assuntos
Circuncisão Masculina , Dermatologia , Adulto , Genitália , Hispânico ou Latino , Humanos , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
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.
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.
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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.
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Circuncisão Masculina , Líquen Escleroso e Atrófico , Plasma Rico em Plaquetas , Humanos , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/terapia , Masculino , Estudos Prospectivos , Qualidade de VidaRESUMO
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.
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.
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Doenças Raras , Esclerodermia Localizada , Interação Gene-Ambiente , Predisposição Genética para Doença , Humanos , Doenças Raras/classificação , Doenças Raras/diagnóstico , Doenças Raras/patologia , Doenças Raras/terapia , Esclerodermia Localizada/classificação , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapiaRESUMO
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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Humanos , Masculino , Feminino , Esclerodermia Localizada/classificação , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Fototerapia/métodos , Prognóstico , Índice de Gravidade de Doença , Fatores Sexuais , Terapia por Exercício , ImunossupressoresRESUMO
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.
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Líquen Escleroso e Atrófico/tratamento farmacológico , Líquen Escleroso e Atrófico/patologia , Doenças da Boca/tratamento farmacológico , Doenças da Boca/patologia , Criança , Feminino , Glucocorticoides/uso terapêutico , Humanos , Injeções Intralesionais , Triancinolona Acetonida/uso terapêuticoRESUMO
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.
Assuntos
Esclerodermia Localizada , Terapia por Exercício , Feminino , Humanos , Imunossupressores , Masculino , Fototerapia/métodos , Prognóstico , Esclerodermia Localizada/classificação , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/etiologia , Esclerodermia Localizada/terapia , Índice de Gravidade de Doença , Fatores SexuaisRESUMO
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.
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.
RESUMO
El liquen escleroso y atrófico o la craurosis de la vulva es una afección caracterizada por la atrofia progresiva crónica de la piel y la mucosa vulvar, asociada con cambios involutivos en los genitales externos. El que sea muy poco usual en nuestro medio fue la causa que motivó la presentación de este caso, de una paciente de 49 años, de color de piel blanca, con antecedentes patológicos personales de hipertensión arterial, que asistió a consulta de Dermatología remitida por su médico de familia. Refirió que desde hacía más o menos ocho meses padecía de prurito intenso en la región vulvar y cambios en la textura de la misma que señaló como ¨sensación de endurecimiento¨ que se había intensificado, así como irregularidad en el período menstrual, todo lo cual no se resolvía con tratamiento habitual pues siempre fue interpretado como parásitos vaginales. Explicó que sufría de dolor intenso durante las relaciones sexuales, con mayor frecuencia en los últimos meses, así como trastornos del sueño y nerviosismo. Se realizó biopsia que arrojó como diagnosticó un liquen escleroso. Se le puso tratamiento con esteroides locales de alta potencia, estrógenos conjugados y apoyo psicológico. La paciente tuvo una evidente mejoría del cuadro sintomático. Se le realiza evaluación periódica para evitar recaídas y/o complicaciones.
Sclerosus and atrophic genital Lichen or vulvar craurosis is an affection characterized by a progressive chronic atrophy of the vulvar skin and mucosa, associated to involutive changes in external genitalia. The presentation of this case was motivated by the unusual appearance of it in our context. A 49 year old white patient with history of hypertension, came to the Dermatology consultation referred by her Family Doctor. She reported that she had had an intense pruritus since approximately eight months in the vulvar region and changes in texture which she stated as ¨hardening sensation¨ which had had intensified, so as irregularities in her menstrual period, all of which was not relieved with the usual treatment as it was interpreted as vaginal parasites. She explained that she suffered from an intense pain during intercourse, more frequently in the last months so as sleeping problems and nervousness. A biopsy was performed and it showed lichen sclerosus. It was treated with local high potency steroids, conjugated estrogens and psychological support. The patient had an evident symptom improvement. She has a periodic evaluation to avoid relapse and /or complications.