Summary

The authors present an atypical case of multisystem sarcoidosis presenting at a late stage with severe lupus pernio sarcoidosis skin lesions and stage IV pulmonary sarcoidosis complicated by semi-invasive chronic necrotising aspergillosis and aspergillomas. Lepromatous leprosy, tuberculosis and active atypical mycobacterial infection had to be ruled out en route to reaching the final diagnoses. His case presented us with a management dilemma, specifically concerning treatment of his sarcoidosis with corticosteroid and other immunosuppressive agents, as these risked aggravating his active invasive fungal disease. The patient’s semi-invasive aspergillosis was treated first with antifungal agents for 6 months before treatment with corticosteroids and hydroxychloroquine was started. The patient has tolerated his treatments well, and over a 3-year follow-up period, has had a significant improvement in his respiratory and systemic symptoms, with some improvement in his lupus pernio sarcoidosis skin lesions.