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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 L÷fgren 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
  • ICD-11: 4B20  4B20.1  4B20.2  4B20.3  4B20.4  4B20.5
  • OMIM: 181000  612387  612388
  • UMLS: C0036202
  • MeSH: D012507
  • GARD: -
  • MedDRA: 10039486

Detailed information

General public


Disease review articles

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.