Peutz Jeghers: Case Report
Peutz Jeghers syndrome, an autosomal dominant disorder that presents polyposis and pigmentation of the skin, and a chance at up to 93% of cancer. Men and women are equally affected with no racial preference, is considered a rare disease. The diagnosis is clinical, supported by a genetic test. There...
| Autores: | , , , , , |
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| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2013 |
| País: | Brasil |
| Institución: | Faculdade de Medicina de Campos (FMC) |
| Repositorio: | Revista Científica da Faculdade de Medicina de Campos |
| Idioma: | portugués |
| OAI Identifier: | oai:ojs.www.fmc.br:article/68 |
| Acceso en línea: | https://www.fmc.br/ojs/index.php/RCFMC/article/view/68 |
| Access Level: | acceso abierto |
| Palabra clave: | Lesão hiperpigmentar mucocutânea Pólipos intestinais SPJ Hiperpigmentar mucocutaneous lesion Intestinal polyps |
| Sumario: | Peutz Jeghers syndrome, an autosomal dominant disorder that presents polyposis and pigmentation of the skin, and a chance at up to 93% of cancer. Men and women are equally affected with no racial preference, is considered a rare disease. The diagnosis is clinical, supported by a genetic test. There is variability of symptoms, from mild to severe with hospitalization and surgery. Objective: Report a case to emphasize the importance of valuing the patient’s complaint, can lead to early diagnosis and institute appropriate monitoring and treatment. Case Report: VRM, 14, female, at the dermatology had complaint of hyperpigmented lesions on the lower lip that hindered socially. The mother reports having your first memory on the stains before 2 years of age. Denied people in the family with similar injuries or any other clinical symptom related. Was referred to gastroenterology, physical examination with no change. Being asked for an endoscopy and colonoscopy, which resulted: multiple sessile polyps microscopy hamartomatous Peutz Jeghers type with no signs of malignancy. The patient was advised to clinical monitoring, repeating these tests periodically. Conclusion: This is a rare case, not only by his family polypoid syndrome itself, but also for its presentation is exclusively dermatology. Clinical manifestations of Peutz Jeghers usually more specific and forceful as well as the presence of family history. Presents a dermatological lesion can point future possible neoplasms, being instiuido proper treatment. |
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