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Carcinoid Syndrome-Induced Scleroderma-Like Disease

Scleroderma is a rare complication of carcinoid syndrome and is usually encountered in the setting of a metastatic primary neuroendocrine tumour of the distal ileum. Associated endocardial fibrosis is a frequent finding and the condition carries a poor prognosis. We report a case of scleroderma occu...

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Detalles Bibliográficos
Autores principales: Koch, Karen, Grayson, Wayne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6827453/
https://www.ncbi.nlm.nih.gov/pubmed/31700850
http://dx.doi.org/10.1159/000496388
Descripción
Sumario:Scleroderma is a rare complication of carcinoid syndrome and is usually encountered in the setting of a metastatic primary neuroendocrine tumour of the distal ileum. Associated endocardial fibrosis is a frequent finding and the condition carries a poor prognosis. We report a case of scleroderma occurring in a 72-year-old female with metastatic neuroendocrine carcinoma and associated pericardial fibrosis. The use of an alternative nomenclature such as “scleroderma-like” or “sclerodermoid” disease is proposed in order to emphasise its distinction from true idiopathic scleroderma, despite the histopathological similarities on skin biopsy.