RESUMEN
Introduction: Actinic prurigo is a chronic photodermatosis. It affects the Latin American population more frequently, predominantly women, and involves the sun-exposed areas of the skin, conjunctiva, and lips. Objective: To update the information on the clinical-epidemiological characteristics and treatment of patients with actinic prurigo in Colombia. Materials and methods: We conducted a cross-sectional study including the medical records of patients with actinic prurigo treated in the Photodermatology Service of Hospital Universitario Centro Dermatológico Federico Lleras Acosta between 2011 and 2016. We described the demographic, clinical, histopathological, and treatment characteristics of the patients. Results: We included 108 patients, 77 (71.3%) were women and 31 (28.7%) men, mainly with phototypes III-IV (70%). The disease had begun during the first decade of life in 66.4% of the cases and 25% of the patients had a family history with the condition. The lesions predominated on the face (93.5%), forearms (79.6%), and back of the hands (70.4%). Ocular (87.9%) and lip (88.8%) involvement was also documented. A photo-provocation test with UVA was performed in 25% of the cases and skin biopsies in 19.4%. Physical and chemical photoprotection was indicated in all patients. Mild to moderate cases were treated with topical corticosteroids (91.7%) and calcineurin inhibitors (65.7%) while severe cases received thalidomide (33.3%) and pentoxifylline (14.8%). Conclusion: The characteristics of actinic prurigo patients in Colombia are similar to those reported in other Latin American countries: early onset of the disease, predominance in women, frequent involvement of conjunctiva and lips, and adequate response to topical and systemic treatment.
Introducción. El prurigo actínico es una fotodermatosis crónica. Afecta con mayor frecuencia a la población latinoamericana, predomina en mujeres y compromete la piel expuesta al sol, las conjuntivas y los labios. Objetivo. Actualizar la información sobre las características clínico-epidemiológicas y el tratamiento de pacientes con prurigo actínico en Colombia. Materiales y métodos. Se hizo un estudio de corte transversal que incluyó los registros clínicos de pacientes con prurigo actínico atendidos en el Servicio de Fotodermatología del Hospital Universitario Centro Dermatológico Federico Lleras Acosta entre el 2011 y el 2016, y se describieron sus características demográficas, clínicas e histopatológicas, así como su tratamiento. Resultados. Se incluyeron 108 pacientes, el 71,3 % de ellos mujeres y el 28,7% hombres, con predominio de los fototipos III-IV (70 %). La enfermedad se había iniciado durante la primera década de vida en el 66,4% de los casos y el 25 % de los pacientes tenía antecedentes familiares de la enfermedad. Las lesiones predominaban en el rostro (93,5 %), los antebrazos (79,6 %) y el dorso de las manos (70,4 %). También, se documentó compromiso ocular (87,9 %) y de los labios (88,8 %). Se hizo la prueba de fotoprovocación con radiación ultravioleta A en el 25 % de los casos y biopsia cutánea en el 19,4 %. Todos los pacientes se trataron con protección solar química y física. En los casos leves a moderados, se formularon corticoides tópicos (91,7 %) e inhibidores de la calcineurina (65,7 %), y en los graves, talidomida (33,3 %) y pentoxifilina (14,8 %). Conclusión. Las características de los pacientes colombianos con prurigo actínico son similares a las reportadas en otros países latinoamericanos: inicio temprano de la enfermedad, predominio en mujeres, compromiso frecuente de conjuntivas y labios, y adecuada respuesta al tratamiento tópico y sistémico.
Asunto(s)
Trastornos por Fotosensibilidad , Enfermedades Cutáneas Genéticas , Adolescente , Corticoesteroides/uso terapéutico , Adulto , Edad de Inicio , Altitud , Inhibidores de la Calcineurina/uso terapéutico , Niño , Colombia/epidemiología , Estudios Transversales , Dermatología , Femenino , Hospitales Universitarios , Humanos , Masculino , Pentoxifilina/uso terapéutico , Trastornos por Fotosensibilidad/epidemiología , Trastornos por Fotosensibilidad/patología , Trastornos por Fotosensibilidad/terapia , Protectores contra Radiación/uso terapéutico , Distribución por Sexo , Enfermedades Cutáneas Genéticas/epidemiología , Enfermedades Cutáneas Genéticas/patología , Enfermedades Cutáneas Genéticas/terapia , Luz Solar/efectos adversos , Adulto JovenRESUMEN
Introducción. El prurigo actínico es una fotodermatosis crónica. Afecta con mayor frecuencia a la población latinoamericana, predomina en mujeres y compromete la piel expuesta al sol, las conjuntivas y los labios. Objetivo. Actualizar la información sobre las características clínico-epidemiológicas y el tratamiento de pacientes con prurigo actínico en Colombia. Materiales y métodos. Se hizo un estudio de corte transversal que incluyó los registros clínicos de pacientes con prurigo actínico atendidos en el Servicio de Fotodermatología del Hospital Universitario Centro Dermatológico Federico Lleras Acosta entre el 2011 y el 2016, y se describieron sus características demográficas, clínicas e histopatológicas, así como su tratamiento. Resultados. Se incluyeron 108 pacientes, el 71,3 % de ellos mujeres y el 28,7% hombres, con predominio de los fototipos III-IV (70 %). La enfermedad se había iniciado durante la primera década de vida en el 66,4% de los casos y el 25 % de los pacientes tenía antecedentes familiares de la enfermedad. Las lesiones predominaban en el rostro (93,5 %), los antebrazos (79,6 %) y el dorso de las manos (70,4 %). También, se documentó compromiso ocular (87,9 %) y de los labios (88,8 %). Se hizo la prueba de fotoprovocación con radiación ultravioleta A en el 25 % de los casos y biopsia cutánea en el 19,4 %. Todos los pacientes se trataron con protección solar química y física. En los casos leves a moderados, se formularon corticoides tópicos (91,7 %) e inhibidores de la calcineurina (65,7 %), y en los graves, talidomida (33,3 %) y pentoxifilina (14,8 %). Conclusión. Las características de los pacientes colombianos con prurigo actínico son similares a las reportadas en otros países latinoamericanos: inicio temprano de la enfermedad, predominio en mujeres, compromiso frecuente de conjuntivas y labios, y adecuada respuesta al tratamiento tópico y sistémico.
Introduction: Actinic prurigo is a chronic photodermatosis. It affects the Latin American population more frequently, predominantly women, and involves the sun-exposed areas of the skin, conjunctiva, and lips. Objective: To update the information on the clinical-epidemiological characteristics and treatment of patients with actinic prurigo in Colombia. Materials and methods: We conducted a cross-sectional study including the medical records of patients with actinic prurigo treated in the Photodermatology Service of Hospital Universitario Centro Dermatológico Federico Lleras Acosta between 2011 and 2016. We described the demographic, clinical, histopathological, and treatment characteristics of the patients. Results: We included 108 patients, 77 (71.3%) were women and 31 (28.7%) men, mainly with phototypes III-IV (70%). The disease had begun during the first decade of life in 66.4% of the cases and 25% of the patients had a family history with the condition. The lesions predominated on the face (93.5%), forearms (79.6%), and back of the hands (70.4%). Ocular (87.9%) and lip (88.8%) involvement was also documented. A photo-provocation test with UVA was performed in 25% of the cases and skin biopsies in 19.4%. Physical and chemical photoprotection was indicated in all patients. Mild to moderate cases were treated with topical corticosteroids (91.7%) and calcineurin inhibitors (65.7%) while severe cases received thalidomide (33.3%) and pentoxifylline (14.8%). Conclusion: The characteristics of actinic prurigo patients in Colombia are similar to those reported in other Latin American countries: early onset of the disease, predominance in women, frequent involvement of conjunctiva and lips, and adequate response to topical and systemic treatment.
Asunto(s)
Prurigo , Trastornos por Fotosensibilidad , Talidomida , Rayos Ultravioleta , FotobiologíaRESUMEN
INTRODUCTION: Oculocutaneous albinism is an autosomal recessive disease caused by complete absence of or decrease in melanin biosynthesis in melanocytes. Due to the reduction or absence of melanin, albinos are highly susceptible to the harmful effects of ultraviolet radiation and are at greater risk of actinic damage and skin cancer. There are no epidemiological data on the incidence of albinism in Brazil. OBJECTIVE: To analyze the clinical and epidemiological profile of patients with albinism treated by the Pró-Albino Program of the Dermatology Clinic of Santa Casa de Misericórdia from its beginning in 2010 until 2017. METHODS: In this cross-sectional study, the records of all consecutive albino patients admitted to the service in the study period were reviewed. Sociodemographic data, family history, and dermatological clinical data were collected. RESULTS: Between March 2010 and April 2017, 191 patients were admitted, of whom 109 were female (57.07%) and the age range was 0-92 years, with >30% under the age of 18 years. Consanguinity among the parents was confirmed by 26% of the patients. Unprotected sun exposure was reported by 109 (57.07%), and 138 (72.25%) had a history of sunburn. Of the 146 records with information, 38 had skin cancer (26%), with a mean age of 47.4 (p < 0.0001); the youngest patient diagnosed with a cutaneous tumor was 23 years old. The prevalence of actinic damage was high. There was information on solar elastosis and actinic keratosis in 148 medical records, of which 96 (64.8%) patients had elastosis and 75 (50.67%) keratoses. Elastosis, keratosis, and skin cancer were significantly associated with age, unprotected sun exposure, and sunburn (p < 0.05). Of the 37 (26% of the sample of 146) patients with a previous or current history of skin cancer, it was possible to identify the histological type in 29 (13 men and 16 women); of these, 18 (62%) were basal cell carcinomas (BCC), 15 (51%) were squamous cell carcinomas (SCC), and 2 (7%) were melanomas. Of these, 4 cases (14%) presented the 2 types of carcinoma (BCC and SCC), and the 2 that had a diagnosis of melanoma also had BCC. Some patients had multiple ulcerated tumors. The tumor site was preferentially in the head and neck (43%), trunk (37%) and limbs (20%). CONCLUSIONS: Albinos represent a risk group for skin cancer and other actinic lesions. These lesions were found to be prevalent in the albinos seen by the program and probably reflect the characteristics found in the Brazilian albino population. Access to health care, especially through multidisciplinary programs that enable the diagnosis and early treatment of these lesions, health education, and the use of photoprotective measures can reduce morbidity and mortality and improve the quality of life of patients with this rare genetic condition.
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Albinismo/epidemiología , Neoplasias Cutáneas/epidemiología , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Albinismo/complicaciones , Brasil/epidemiología , Niño , Preescolar , Estudios Transversales , Femenino , Humanos , Incidencia , Lactante , Recién Nacido , Masculino , Persona de Mediana Edad , Prevalencia , Neoplasias Cutáneas/etiología , Adulto JovenRESUMEN
Abstract: Solar urticaria is a rare form of physical urticaria mediated by immunoglobulin E. The lesions appear immediately after the sun exposure, interfering with the patient's normal daily life. Omalizumab, a monoclonal anti-IgE antibody, has been recently approved for the treatment of chronic spontaneous urticaria, and the latest reports support its role also in the treatment of solar urticaria. Hereby, we report a case of solar urticaria refractory to conventional treatment strategies, with an excellent response to treatment with omalizumab and phototesting normalization.
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Humanos , Masculino , Persona de Mediana Edad , Luz Solar/efectos adversos , Urticaria/tratamiento farmacológico , Antialérgicos/uso terapéutico , Omalizumab/uso terapéutico , Trastornos por Fotosensibilidad/diagnóstico , Trastornos por Fotosensibilidad/etiología , Trastornos por Fotosensibilidad/tratamiento farmacológico , Urticaria/diagnóstico , Urticaria/etiologíaRESUMEN
Abstract Lupus erythematosus tumidus is a rare dermatosis. It is considered a subtype of chronic cutaneous lupus erythematosus of uncertain pathogenesis, favorable prognosis and rare association with systemic lupus erythematosus. Clinically, it manifests as urticarial-like plaques in photo exposed areas, mainly affecting adults, being extremely rare in pediatric age. Herein, we present two cases of six and nine-year-old male patients with clinical and histological characteristics typical of lupus erythematosus tumidus and poor response to first-line treatment (topical, intralesional steroids and topical calcineurin inhibitors); therefore, it was decided to start systemic therapy with antimalarials, obtaining a very good response.
Resumen El lupus eritematoso tumidus es una dermatosis poco frecuente. Es considerada una variante del lupus eritematoso cutáneo crónico, de patogénesis incierta, pronóstico favorable y rara asociación con lupus eritematoso sistémico. Clínicamente, se manifiesta como placas de aspecto urticarial en zonas fotoexpuestas, que principalmente afectan a los adultos, siendo extremadamente rara en edad pediátrica. A continuación presentamos dos casos de pacientes de sexo masculino de seis y nueve años, con características clínicas e histológicas típicas de lupus eritematoso tumidus y poca respuesta al tratamiento de primera línea (esteroides tópicos, intralesionales e inhibidores de calcineurina tópica), por lo que se decidió iniciar manejo sistémico con antimalárico, obteniendo muy buena respuesta terapéutica.
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Abstract: Background: Actinic prurigo (AP) is an idiopathic photodermatosis. Although its initial manifestations can appear in 6 to 8-year-old children, cases are diagnosed later, between the second and fourth decades of life, when the injuries are exacerbated. Objective: To identify risk factors associated with clinical manifestations of AP such as skin and mucosal lesions. Methods: Thirty patients with AP and 60 controls were included in the study, the dependent variable was the presence of skin or labial mucosal lesions, the independent variables were age, sex, solar exposure, living with pets or farm animals, exposure to wood smoke, smoking habit, years smoking, and hours spent per day and per week in contact with people who smoke. Results: Of the 30 diagnosed AP patients, 66.7% were female. Patients age ranged from 7 to 71 years and the mean age was 35.77 ± 14.55 years. We found significant differences with the age and cohabitation with farm animals. Those who lived with farm animals presented 14.31 times higher probability of developing AP (95% CI 3-78.06). Study limitations: This is a case-control study; therefore, a causal relationship cannot be proven, and these results cannot be generalized to every population. Conclusions: The identification of factors related to the development of AP increases our knowledge of its physiopathology. Moreover, identifying antigens that possibly trigger the allergic reaction will have preventive and therapeutic applications in populations at risk of AP.
Asunto(s)
Humanos , Animales , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Adulto Joven , Trastornos por Fotosensibilidad/etiología , Enfermedades Cutáneas Genéticas/etiología , Exposición a Riesgos Ambientales/efectos adversos , Trastornos por Fotosensibilidad/fisiopatología , Enfermedades Cutáneas Genéticas/fisiopatología , Luz Solar/efectos adversos , Factores de Tiempo , Estudios de Casos y Controles , Modelos Logísticos , Factores de Riesgo , Factores de Edad , Estadísticas no Paramétricas , Hipersensibilidad/etiología , Hipersensibilidad/fisiopatología , Animales DomésticosRESUMEN
Introducción: la destrucción de la capa de ozono ha provocado un aumento en la incidencia de lesiones de la piel, a la que se suma la queilitis actínica. Objetivo: describir los aspectos histológicos, clínicos y epidemiológicos de la queilitis actínica a partir de la literatura reciente. Métodos: se revisaron las bases electrónicas PubMed, SciELO y Google Scholar con los términos claves en inglés y español: queilitis, queratosis, actínica, solar. Se incluyeron artículos originales, de revisión, reportes de casos, tesis y libros de la especialidad publicados preferentemente en el período 2005-2014. Resultados: La queilitis actínica es un trastorno potencialmente maligno inducido por la exposición solar y caracterizado por alteraciones micro y macroestructurales del labio. Factores de riesgo que interaccionan con la exposición solar son el fototipo (piel clara), hábito tabáquico, sexo (hombres), edad y ocupación (aire libre). Entre las alteraciones histológicas se encuentran la displasia epitelial y la elastosis solar; sin embargo, la severidad de estas no correlacionan con la gravedad clínica. Los pacientes con queilitis actínica presentan alteraciones de color, descamación, ulceraciones, difuminación del bermellón, entre otras. En muchas ocasiones la consulta y el diagnóstico son tardíos; se realizan cuando el cuadro ha evolucionado a cáncer. El diagnóstico es principalmente clínico, sumado a la biopsia de las lesiones con presentaciones moderadas y severas. Actualmente la terapia incluye métodos quirúrgicos y farmacológicos, y métodos innovadores como la fototerapia. Sin duda, la estrategia de prevención más importante es aumentar el uso de protectores solares, especialmente en la población de alto riesgo ocupacional. Conclusiones: la queilitis actínica es una patología relevante para los países sudamericanos, debido a que los factores de riesgo están presentes diariamente en las actividades de millones de trabajadores de nuestra región, por eso es necesario potenciar la investigación que permita mejorar la prevención, tratamiento y rehabilitación de esta patología(AU)
Introduction: depletion of the ozone layer has brought about an increase in the incidence of skin lesions, including actinic cheilitis. Objective: describe the histological, clinical and epidemiological characteristics of actinic cheilitis based on a review of recent literature. Methods: a search was conducted in the databases PubMed, SciELO and Google Scholar using the descriptors cheilitis, keratosis, actinic, solar, and their counterparts in Spanish. The search included original papers, review papers, case reports, theses and books about the specialty preferably published from 2005 to 2014. Results: actinic cheilitis is a potentially malignant condition induced by sun exposure and characterized by micro- and macrostructural alterations of the lip. The risk factors interacting with sun exposure are the skin phototype (light skin), smoking, gender (male), age and occupation (outdoor jobs). Histological alterations include epithelial dysplasia and solar elastosis, though their severity does not correlate with the degree of clinical seriousness. Patients with actinic cheilitis present color alterations, desquamation, ulceration and blurring of the vermillion border, among other signs and symptoms. On many occasions patients do not seek care during the early stages of the disease. As a result, diagnosis is made when the condition has already evolved into cancer. The diagnosis is basically clinic, with the support of the biopsy of lesions with moderate to severe characteristics. Current therapy includes surgery and medication, as well as innovative techniques like phototherapy. The most important strategy is no doubt the use of sunscreens, especially by the population at high occupational risk. Conclusions: actinic cheilitis is a condition relevant to South American countries, since its risk factors are present in the daily activities of millions of workers from our region. It is therefore necessary to foster research aimed at improving its prevention, treatment and rehabilitation(AU)
Asunto(s)
Humanos , Queilitis/epidemiología , Bases de Datos Bibliográficas/estadística & datos numéricos , Queratosis Actínica/patología , Trastornos por Fotosensibilidad/prevención & control , Neoplasias de la Boca/terapia , RevisiónRESUMEN
Abstract BACKGROUND: Actinic prurigo is an idiopathic photodermatosis, the pathophysiology of which has been hypothesized to involve subtype IV type b (Th2) hypersensitive response, whereby IL4, IL5, and IL13 are secreted and mediate the production of B cells, IgE, and IgG4. OBJECTIVES: To examine the association of serum IgE levels and the clinical severity of injuries. METHODS: This case-control study comprised patients with a clinical and histopathological diagnosis of actinic prurigo, as well as clinically healthy subjects, from whom 3cc of peripheral blood was taken for immunoassay. Cases were classified by lesion severity as mild, moderate, and severe. Descriptive statistics were analyzed, and chi-square test was performed. RESULTS: We included 21 actinic prurigo patients and 21 subjects without disease; 11 patients with actinic prurigo had elevated serum IgE levels, and 10 had low serum levels. Six actinic prurigo (AP) patients with elevated serum levels of IgE had moderate injuries, 4 had severe injuries, and 1 had minor injuries. Eight out of 10 patients with normal IgE levels presented with minor injuries in the clinical evaluation. The 21 controls did not have increased serum IgE levels. CONCLUSIONS: Elevated IgE levels are associated with moderate to severe clinical lesions, suggesting that actinic prurigo entails a type IV subtype b hypersensitivity response in which Th2 cells predominate.
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Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Inmunoglobulina E/sangre , Trastornos por Fotosensibilidad/sangre , Trastornos por Fotosensibilidad/fisiopatología , Enfermedades Cutáneas Genéticas/sangre , Enfermedades Cutáneas Genéticas/fisiopatología , Estudios de Casos y Controles , Inmunoensayo , Inmunosupresores/uso terapéutico , Trastornos por Fotosensibilidad/patología , Valores de Referencia , Índice de Severidad de la Enfermedad , Enfermedades Cutáneas Genéticas/patología , Talidomida/uso terapéuticoRESUMEN
The actinic comedonal plaque is characterized by papules, cysts and comedones forming a yellowish plaque in areas of chronic sun exposure skin. There are few reports in literature about this entity, considered a rare and ectopic form of Favré-Racouchot Syndrome. We report two cases of lesions located on forearms and thorax. Favré-Racouchot Syndrome is a condition usually restricted to the periorbital area; however, there are reports of similar findings in atypical locations, such as forearms and chest, which are known as actinic comedonal plaque. Ultraviolet radiation exposure is the main factor involved in its pathogenesis. The objective of this study was to provide accurate knowledge of this dermatosis and stimulate dermatologists to provide a correct diagnosis of the condition.
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Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Eritema/patología , Dermatosis Facial/patología , Piel/patología , Biopsia , Luz Solar/efectos adversosRESUMEN
We report the case of a 28-year-old woman with Kindler syndrome, a rare form of epidermolysis bullosa. Clinically, since childhood, she had widespread pigmentary changes in her skin as well as photosensitivity and fragility of the skin and mucous membranes. The mucosal involvement led to an erosive stomatitis as well as esophageal, anal and vaginal stenoses, requiring surgical intervention. The diagnosis of Kindler syndrome was confirmed by DNA sequencing with compound heterozygosity for a nonsense/frameshift combination of mutations (p.Arg110X; p.Ala289GlyfsX7) in the FERMT1 gene.
Nós relatamos uma paciente feminina de 28 anos com Síndrome de Kindler, uma forma rara de Epidermólise Bolhosa. Clinicamente, ela apresentava alterações cutâneas pigmentares disseminadas, fotossensibilidade e fragilidade da pele e das mucosas desde a infância. O envolvimento mucoso levou à estomatite erosiva e a estenoses esofágica, anal e vaginal, as quais necessitaram de intervenções cirúrgicas. O diagnóstico de Síndrome de Kindler foi confirmado por sequenciamento de DNA, que demonstrou heterozigose composta uma combinação de mutações uma nonsense e outra frameshift (p.Arg110X; p.Ala289GlyfsX7) no gene FERMT1.
Asunto(s)
Adulto , Femenino , Humanos , Vesícula/genética , Codón sin Sentido , Epidermólisis Ampollosa/genética , Mutación del Sistema de Lectura , Enfermedades Periodontales/genética , Trastornos por Fotosensibilidad/genética , Vesícula/patología , Análisis Mutacional de ADN , Epidermólisis Ampollosa/patología , Proteínas de la Membrana/genética , Proteínas de Neoplasias/genética , Enfermedades Periodontales/patología , Trastornos por Fotosensibilidad/patología , Piel/patologíaRESUMEN
Hydroa Vaciniforme is a very rare photodermatosis that is mainly seen in childhood. An 18 year old female student reported that since the age of 5 she has been suffering necrotic lesions and vesicles lesions in exposed areas, leaving asymptomatic varioliform scars, which worsened in summer. Light microscopy showed epidermal necrosis with lymphocytic infiltration . Sunscreens were prescribed with light improvement.
O Hidroa Vaciniforme é uma fotodermatose muito rara vista geralmente na infância. Uma paciente de 18 anos foi examinada, a qual apresenta lesões vesiculosas e necróticas varioliformes nas áreas fotoexpostas, que evoluem para cicatrizes atróficas, piorando no verão. A microscopia óptica mostrou necrose epidérmica com infiltrado linfocítico. Houve pouca melhora com uso de filtros solares.
Asunto(s)
Adolescente , Femenino , Humanos , Hidroa Vacciniforme/patología , Necrosis , Piel/patologíaRESUMEN
Kindler syndrome is a rare autosomal recessive genodermatosis characterized by trauma-induced blisters, progressive poikiloderma and varying degrees of photosensitivity. In 2003, loss-of-function mutations were identified in the gene KIND1 mapped to chromosome 20p12.3. In this paper, we report Kindler syndrome in two children born to consanguineous parents presenting acral blistering, photosensitivity, poikiloderma, cutaneous atrophy and periodontitis.
A síndrome de Kindler é uma genodermatose rara, autossômica recessiva, caracterizada pela presença de bolhas induzidas por traumas, fotossensibilidade, atrofia cutânea e poiquilodermia progressiva. A alteração genética da síndrome foi descrita em 2003, com a identificação de mutação no gene KINDIN1, localizado no cromossomo 20p12.3. Nesse trabalho relata-se a presença da síndrome de Kindler em irmãos, filhos de pais consangüíneos, que apresentavam, desde a infância, fotossensibilidade, bolhas após pequenos traumas, poiquilodermia, atrofia cutânea e periodontite.
Asunto(s)
Preescolar , Femenino , Humanos , Vesícula/patología , Epidermólisis Ampollosa/patología , Enfermedades Periodontales/patología , Trastornos por Fotosensibilidad/patología , FenotipoRESUMEN
A dermatite solar é uma dermatopatia de natureza ambiental que pode acometer cães e gatos, principalmente aqueles que possuam pele branca e parca cobertura pilosa. Devido à exposição contínua aos raios ultravioletas, a dermatite solar pode evoluir para queratose actínica, uma displasia epitelial préneoplásica.O diagnóstico é feito com base no histórico de exposição solar prolongada, sinais clínicos compatíveis e exame histopatológico. Não há tratamento curativo e o controle consiste principalmente em restrição solar e utilização de fotoprotetores Este trabalho tem como objetivo reunir informações sobre dermatite solar em cães e gatos, abrangendo todos os seus aspectos, bem como relatar três casos da dermatopatia em cães da raça Pit Bull Terrier
Solar dermatitis is an environmental skin disease that can affect dogs and cats, especially those who have white skin and scatter hair coverage. Due the continuous exposure to ultraviolet rays, solar dermatitis can develop into actinic keratosis, a pre neoplasic epithelial dysplasia. The diagnosis is made based on history of continuous solar exposure, compatible clinical signs and histopathologic examination. Although there is no treatment, control can be achieved by strict restriction to solar exposure, as well as continuous use of sun block creams. The aim of this study is gather information about solar dermatitis in dogs and cats, covering all aspects and report three cases of the disease in Pit Bull Terrierdogs
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Animales , Gatos , Perros , Perros , Dermatitis/diagnóstico , Dermatitis/etiología , Dermatitis/patología , Dermatitis/tratamiento farmacológicoRESUMEN
A dermatite solar é uma dermatopatia de natureza ambiental que pode acometer cães e gatos, principalmente aqueles que possuam pele branca e parca cobertura pilosa. Devido à exposição contínua aos raios ultravioletas, a dermatite solar pode evoluir para queratose actínica, uma displasia epitelial préneoplásica.O diagnóstico é feito com base no histórico de exposição solar prolongada, sinais clínicos compatíveis e exame histopatológico. Não há tratamento curativo e o controle consiste principalmente em restrição solar e utilização de fotoprotetores Este trabalho tem como objetivo reunir informações sobre dermatite solar em cães e gatos, abrangendo todos os seus aspectos, bem como relatar três casos da dermatopatia em cães da raça Pit Bull Terrier(AU)
Solar dermatitis is an environmental skin disease that can affect dogs and cats, especially those who have white skin and scatter hair coverage. Due the continuous exposure to ultraviolet rays, solar dermatitis can develop into actinic keratosis, a pre neoplasic epithelial dysplasia. The diagnosis is made based on history of continuous solar exposure, compatible clinical signs and histopathologic examination. Although there is no treatment, control can be achieved by strict restriction to solar exposure, as well as continuous use of sun block creams. The aim of this study is gather information about solar dermatitis in dogs and cats, covering all aspects and report three cases of the disease in Pit Bull Terrierdogs(AU)
Asunto(s)
Animales , Gatos , Perros , Dermatitis/diagnóstico , Dermatitis/tratamiento farmacológico , Dermatitis/etiología , Dermatitis/patología , PerrosRESUMEN
FUNDAMENTO: A queilite actínica crônica é a ceratose actínica localizada no vermelhão labial. O tratamento é de crucial importância, devido ao potencial de transformação maligna. OBJETIVO: Avaliar os resultados estéticos e funcionais das vermelhectomias clássica e em W-plastia na queilite actínica. Na técnica clássica, a cicatriz é linear; na W-plastia, em linha quebrada. MÉTODOS: Foram tratados 32 pacientes com diagnóstico clínico e histopatológico de queilite actínica. Quinze deles foram submetidos à técnica em W-plastia e 17, à técnica clássica. Avaliaram-se parâmetros como retração cicatricial e alterações funcionais. RESULTADOS: Houve associação estatisticamente significativa entre a técnica utilizada e a presença de retração cicatricial, sendo a associação positiva com a clássica (p=0,01 com correção de Yates). O risco relativo (odds ratio - OR) calculado foi de 11,25, ou seja, houve maior chance de retração nos pacientes submetidos à técnica clássica. Nenhuma das técnicas apresentou alterações funcionais. Avaliaram-se complicações pós-operatórias como presença de crostas, lábios secos, parestesia e deiscência de sutura. Não houve associação estatisticamente significante entre as complicações e a técnica utilizada (p=0,69). CONCLUSÃO: Concluiu-se que a vermelhectomia em W-plastia oferece melhores resultados estéticos e índices de complicações semelhantes.
BACKGROUND: Chronic actinic cheilitis is actinic keratosis located on the vermilion border. Treatment is essential because of the potential for malignant transformation. OBJECTIVE: To evaluate the aesthetic and functional results of vermilionectomy using the classic and Wplasty techniques in actinic cheilitis. In the classic technique, the scar is linear and in the W-plasty one, it is a broken line. METHODS: 32 patients with clinical and histopathological diagnosis of actinic cheilitis were treated. Out of the 32 patients, 15 underwent the W-plasty technique and 17 underwent the classic one. We evaluated parameters such as scar retraction and functional changes. RESULTS: A statistically significant association between the technique used and scar retraction was found, which was positive when using the classic technique (p = 0.01 with Yates' correction). The odds ratio was calculated at 11.25, i.e., there was a greater chance of retraction in patients undergoing the classic technique. Both techniques revealed no functional changes. We evaluated postoperative complications such as the presence of crusts, dry lips, paresthesia, and suture dehiscence. There was no statistically significant association between complications and the technique used (p = 0.69). CONCLUSION: We concluded that vermilionectomy using the W-plasty technique shows better cosmetic results and similar complication rates.
Asunto(s)
Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Queilitis/cirugía , Cicatriz/cirugía , Labio/cirugía , Procedimientos de Cirugía Plástica/métodos , Factores de Edad , Distribución de Chi-Cuadrado , Carcinoma de Células Escamosas/prevención & control , Neoplasias de los Labios/prevención & control , Complicaciones Posoperatorias , Lesiones Precancerosas/prevención & control , Factores de Riesgo , Factores Sexuales , Resultado del TratamientoRESUMEN
Paciente do sexo masculino, negro, 13 anos, apresenta há dois anos lesões pruriginosas, pápulonodulares nos antebraços, associadas a edema do lábio inferior, fotofobia, conjuntivite e pterígio. O exame histopatológico do lábio inferior revelou acantose, espongiose e infiltrado inflamatório perivascular superficial, composto por linfócitos, plasmócitos e eosinófilos, compatível com o diagnóstico de prurigo actínico. As lesões regrediram com o uso de talidomida 100 mg/dia.
A 13-year-old black boy had pruritic papular and nodular lesions on his forearms associated to edema of the lower lip, photophobia, conjunctivitis and pterygium. Skin biopsy of the lower lip revealed acanthosis, spongiosis with dermal perivascular mononuclear cell infiltration composed by lymphocytes, plasma cells and eosinophils consistent with actinic prurigo. Lesions improved considerably with the use of thalidomide 100mg/ day.
Asunto(s)
Adolescente , Humanos , Masculino , Trastornos por Fotosensibilidad/patología , Prurigo/patología , Inmunosupresores/uso terapéutico , Trastornos por Fotosensibilidad/tratamiento farmacológico , Prurigo/tratamiento farmacológico , Talidomida/uso terapéuticoRESUMEN
Purpose: Ultraviolet radiation is the main etiological agent associated with the development of actinic cheilitis, a premalignant irreversible disease that frequently affects the vermilion border of the lower lip. This paper aimed to describe the case of a patient with actinic cheilitis and treated with scalpel vermilionectomy. Case Description: A male, Caucasian, 66 year-old subject sought dental treatment complaining of "wounds on the lip that not heal" for approximately 15 years. The patient reported that he had worked in farms since the age of 5 year-old and was a frequent user of alcohol and tobacco. Atrophic and erosive areas associated with many ulcers covered with eschars were observed throughout the length of the lip. Based on the patient history and clinical exam, scalpel vermilionectomy was proposed. The histopathological analysis revealed a superficially invasive squamous cell carcinoma with free borders. The patient was monitored for two years with no relapse. Conclusion: Scapel vermilionectomy was chose to treat this case due to its low cost, relatively short time for repair, and possibility of histopathological analysis of the removed tissue.
Objetivo: A radiação ultravioleta é o principal agente etiológico associado ao desenvolvimento da queilite actínica, uma lesão pré-maligna que afeta o lábio inferior. Este trabalho descreveu o caso clínico de um paciente que apresentava várias ulcerações recobertas por crosta no lábio inferior e foi tratado pela técnica da vermelhectomia. Descrição do caso: Um sujeito do sexo masculino, Caucasiano, de 66 anos de idade, procurou tratamento odontológico com queixa de "feridas no lábio que não cicatrizavam" por 15 anos. O paciente relatou que tinha trabalhado em fazendas desde os 5 anos de idade e fazia uso frequente de tabaco e álcool. Áreas erosivas e atróficas associadas a múltiplas úlceras cobertas com escaras foram observadas em todo o lábio. Com base na história do paciente e exame clínico, foi proposto tratamento por vermelhectomia. O diagnóstico histopatológico foi de carcinoma espinocelular superficialmente invasivo, com margens livres, e o paciente foi acompanhado por um período de 2 anos sem recidiva. Conclusão: A técnica da vermelhectomia foi a escolha preferencial de tratamento por ser de baixo custo, ter um tempo cicatricial relativamente curto e, especialmente, permitir que o tecido removido possa ser examinado histopatologicamente.