Abstract
4,4'-Diaminodiphenylsulphone (Dapsone) is widely used for a variety of infectious, immune and hypersensitivity disorders, with indications ranging from Hansen's disease, inflammatory disease and insect bites, all of which may be seen as manifestations in certain occupational diseases. However, the use of dapsone may be associated with a plethora of adverse effects, some of which may involve the pulmonary parenchyma. Methemoglobinemia with resultant cyanosis, bone marrow aplasia and/or hemolytic anemia, peripheral neuropathy and the potentially fatal dapsone hypersensitivity syndrome (DHS), the focus of this review, may all occur individually or in combination. DHS typically presents with a triad of fever, skin eruption, and internal organ (lung, liver, neurological and other systems) involvement, occurring several weeks to as late as 6 months after the initial administration of the drug. In this sense, it may resemble a DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms). DHS must be promptly identified, as untreated, the disorder could be fatal. Moreover, the pulmonary/systemic manifestations may be mistaken for other disorders. Eosinophilic infiltrates, pneumonitis, pleural effusions and interstitial lung disease may be seen. This syndrome is best approached with the immediate discontinuation of the offending drug and prompt administration of oral or intravenous glucocorticoids. An immunological-inflammatory basis of the syndrome can be envisaged, based on the pathological picture and excellent response to antiinflammatory therapy. Since dapsone is used for various indications, physicians from all specialties may encounter DHS and need to familiarize themselves with the salient features about the syndrome and its management.
Highlights
Dapsone has been used for many indications because of its antibiotic and anti-inflammatory effects [1]
Since the advent of the era of the Acquired Immunodeficiency Syndrome (AIDS), dapsone has been increasingly utilized in the chemoprophylaxis of Pneumocystis carinii infection in combination with Trimethoprim/ Sulfamethoxazole
A high index of suspicion is needed for early diagnosis of dapsone hypersensitivity syndrome (DHS)
Summary
Dapsone has been used for many indications because of its antibiotic and anti-inflammatory effects [1]. DHS is characterized by the onset of fever, skin eruption and internal organ involvement several weeks to as late as 6 months after patients are given this drug. These include diseases such as: DRESS syndrome and its variants, vasculitis (Churg Strauss syndrome), Hypereosinophilic syndrome, TENS (Toxic epidermal necrolysis syndrome), Steven Johnson Syndrome, Still's disease, Hematological disorders (leukemia, lymphoma), paraneoplastic disorders and certain connective tissue disorders. It has been shown that a reduction in either quantity or activity of N-hydroxylation enzyme systems resulted in decreased total clearance of dapsone This information is supported by studies showing an extensive population and individual variation in this ability involving both genetic (increased or decreased P450 activity, decreased reduced glutathione [GSH]) and environmental (drugs or chemicals such as smoking inducing P450, cirrhosis and drugs inhibiting P450, decreased GSH such as in AIDS, deficiency of antioxidants such as Vitamin E, C, selenium) [24]. Since genetic factors are involved in the pathogenesis of DHS, relatives should be instructed about DHS and their enhanced risk of developing similar adverse reactions should they take dapsone [10]
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