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Disease definition

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.


Classification level: Disorder
  • Synonym(s):
    • Besnier-Boeck-Schaumann disease
    • Boeck sarcoid
  • Prevalence: 1-5 / 10 000
  • Inheritance: Multigenic/multifactorial 
  • Age of onset: Childhood, Adolescent, Elderly, Adult
  • ICD-10: D86.0  D86.1  D86.2  D86.3  D86.8  D86.9
  • OMIM: 181000  612387  612388
  • UMLS: C0036202
  • MeSH: D012507
  • GARD: -
  • MedDRA: 10039486

Detailed information

Article for general public


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