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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Bibliographic Details
Published in:Dermatopathology (Basel, Switzerland) Switzerland), 2019-06, Vol.6 (2), p.99-104
Main Authors: Koch, Karen, Grayson, Wayne
Format: Article
Language:English
Subjects:
Carcinoid syndrome
Case Report
Scleroderma
Scleroderma-like disorders
Sclerodermoid disease
Skin
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Description
Summary: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.
ISSN:2296-3529
2296-3529
DOI:10.1159/000496388