2 resultados para primary sjogrens-syndrome

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Introduction. Familial Atypical Multiple Mole-Melanoma Syndrome (FAMMM) is an autosomal dominant genodermatosis characterized by the presence of a high number of dysplastic nevi and family history of melanoma or pancreatic cancer. Melanomas in FAMMM patients tend to occur at a younger age, although they are clinically similar to sporadic melanomas in terms of overall survival. Case report. A 45 year-old woman with a family history of melanoma, a type II phototype and numerous (>100) nevi was admitted to our Department of Dermatology and Plastic Surgery. Over the past years, the patient underwent several surgical operations to remove pigmented lesions and two are dysplastic nevi. Since 1995, she underwent surgery to remove four melanomas. She is followed for skin examinations including dermoscopy. Conclusion. Identifying high-risk patients for melanoma represents a primary objective for the specialists that are involved in the management of this disease, especially in order to enact all the necessary surveillance and follow-up strategies.

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Objectives: To highlight systemic involvement features in Sjögren Syndrome (SS). Materials and methods: A case of a 32-year-old woman presenting with flaccid tetraparesis, in the setting of severe hypokalaemia, is described. Results: Additional evaluation confirmed primary SS with type 1 renal tubular acidosis (RTA1) and gammaglobulin-mediated vasculitis. A significant clinical improvement was achieved following adequate treatment. Conclusion: Extra-glandular involvement in SS is usually due to autoimmune lymphocytic infiltration and severe complications can be avoided if there is a prompt diagnosis.