- Chronic multisystem disease of unknown aetiology, commonly affecting the lungs, skin, and eyes, characterised by formation of non-caseating granulomas.
- Has a bimodal age distribution with 2 peaks in the third and fifth decades.
- Lungs and lymph nodes affected in >90% of patients.
- Treated with topical corticosteroids for mild local cutaneous disease. Systemic corticosteroids are the mainstay of treatment for severe disease.
- Carries a mortality of 1% to 6%.
- Poorer prognosis if black ancestry, chronic pulmonary involvement, lupus pernio, or chronic hypercalcaemia.
- Spontaneous remissions occur in 55% to 90% of patients with stage I, 40% to 70% of patients with stage II, and about 20% of patients with stage III disease, but no remissions are expected in stage IV.
Last updated: Apr 10, 2013