A rare multisystemic, autoinflammatory disorder of unknown etiology characterized by the formation of immune, non-caseating granulomas in any organ(s), leading to variable clinical symptoms and severity. Clinical presentation is typically with persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and Löfgren syndrome.

Synonyms: Besnier-Boeck-Schaumann disease Boeck sarcoid

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Term information

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OMIM:612387

MedDRA:10039486

MeSH:D012507

OMIM:181000

OMIM:612388

ICD-10:D86.8

ICD-10:D86.9

UMLS:C0036202

ICD-10:D86.2

ICD-10:D86.3

ICD-10:D86.0

ICD-10:D86.1

notation

ORPHA:797

Term relations

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