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ORPHA:797Disease
Also called Besnier-Boeck-Schaumann disease · Boeck sarcoid
What it is
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.
Key facts
- Prevalence
- 1-5 / 10 000
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
19Sometimes5–29%
51- Abnormal cardiac ventricular function
- Abnormal conjunctiva morphology
- Abnormality of the cerebrospinal fluid
- Abnormality of the gastrointestinal tract
- Abnormality of the lymph nodes
- Abnormality of the nasal mucosa
- Abnormal liver parenchyma morphology
- Abnormal reproductive system morphology
and 43 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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