Dapsone hypersensitivity syndrome complicated by Scedosporium apiospermum pneumonia in an immunocompetent patient

Excerpt

Dapsone (4,4′diaminodiphenyl sulphone) has been used since the middle of the 20th century for leprosy treatment, usually in combination with rifampin and clofazimine. Dapsone hypersensitivity syndrome (DHS) occurs in 0.5−3% of patients treated with this drug. The exact immune mechanism behind this idiosyncratic reaction with multi-organ involvement is unclear. Some genetic and environmental factors, by increasing the production of dapsone-reactive metabolites or affecting the ability of the liver to detoxificate them, may increase the risk of developing DHS. Latency before the onset of symptoms can vary from 2−6 h, in previously sensitised patients, to 6 months [1]. Typical DHS manifestations include high fever, skin rash, malaise, methemoglobinaemia, liver toxicity (jaundice, hepatitis and hepatomegaly) and generalised lymphadenopathy. Acute renal failure, hypersensitivity pneumonia, cholangitis, sensory peripheral neuropathy, pancreatitis and pleural effusion have been reported less frequently [2]. The discontinuation of dapsone and the early initiation of high-dosage systemic corticosteroid therapy are crucial to decrease mortality and morbidity. …