The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations

<p>Abstract</p> <p>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 i...

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Main Authors: Chi David S, Nassour Dima N, Guha Bhuvana, Kosseifi Semaan G, Krishnaswamy Guha
Format: Article
Language:English
Published: BMC 2006-06-01
Series:Journal of Occupational Medicine and Toxicology
Online Access:http://www.occup-med.com/content/1/1/9
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author Chi David S
Nassour Dima N
Guha Bhuvana
Kosseifi Semaan G
Krishnaswamy Guha
author_facet Chi David S
Nassour Dima N
Guha Bhuvana
Kosseifi Semaan G
Krishnaswamy Guha
author_sort Chi David S
collection DOAJ
description <p>Abstract</p> <p>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 <b>(DHS)</b>, the focus of this review, may all occur individually or in combination. <b>DHS </b>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 <b>DRESS syndrome </b>(Drug Rash with Eosinophilia and Systemic Symptoms). <b>DHS </b>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 <b>DHS </b>and need to familiarize themselves with the salient features about the syndrome and its management.</p>
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spelling doaj.art-f628d84575fb49abb132a9ebaceb3a732022-12-22T04:23:11ZengBMCJournal of Occupational Medicine and Toxicology1745-66732006-06-0111910.1186/1745-6673-1-9The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestationsChi David SNassour Dima NGuha BhuvanaKosseifi Semaan GKrishnaswamy Guha<p>Abstract</p> <p>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 <b>(DHS)</b>, the focus of this review, may all occur individually or in combination. <b>DHS </b>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 <b>DRESS syndrome </b>(Drug Rash with Eosinophilia and Systemic Symptoms). <b>DHS </b>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 <b>DHS </b>and need to familiarize themselves with the salient features about the syndrome and its management.</p>http://www.occup-med.com/content/1/1/9
spellingShingle Chi David S
Nassour Dima N
Guha Bhuvana
Kosseifi Semaan G
Krishnaswamy Guha
The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
Journal of Occupational Medicine and Toxicology
title The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
title_full The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
title_fullStr The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
title_full_unstemmed The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
title_short The Dapsone Hypersensitivity Syndrome revisited: a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
title_sort dapsone hypersensitivity syndrome revisited a potentially fatal multisystem disorder with prominent hepatopulmonary manifestations
url http://www.occup-med.com/content/1/1/9
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