Dapsone hypersensitivity syndrome in a leprosy patient. Dapsone hypersensitivity syndrome dhs is a rare potentially fatal systemic idiosyncratic adverse reaction, with multiorgan involvement also known as sulphone syndrome which is particularly seen in leprosy patients who are on world health organization. Unsupervised tsne plot generated from a merged dataset of the two time. We describe a 55yearold caucasian woman with normal glucose6phosphate dehydrogenase levels presenting with an extensive skin eruption, highgrade fever, pneumonitis and hepatitis, which occurred within 3 weeks after initiation of dapsone. Uncommonly, weakness of the foot and hand muscles can occur, particularly with long term dapsone therapy with doses greater than 100 mg per. Dapsone hypersensitivity reaction was reported as early as 1950 by lowe and was so named by allday and barnes. Hypersensitivity to dapsone is thought to be the major cause of this syndrome. Although good results may be obtained for some patients with a glutenfree diet, the difficulty of dietary adherence makes dapsone the drug of choice for many. Dapsone hypersensitivity syndrome dhs is an idiosyncratic adverse effect caused by dapsone. It is rarely associated with potentially fatal severe drug hypersensitivity syndrome, in which there is prolonged rash, fever, swollen lymph glands and internal organ failure liver, lungs, heart, kidneys. A case of dapsone hypersensitivity syndrome dhs in an aids patient receiving dapsone for pneumocystis pneumonia pcp prophylaxis is discussed. Dapsone hypersensitivity syndrome is characterized by an exfoliative dermatitis, fever and hepatitis which may be lifethreatening. Dapsone is one treatment for sweet syndrome both as monotherapy or adjunctive to corticosteroid therapy.
Hematologic side effects have been the most pronounced. Amino acid variants of hladrb1 confer susceptibility to. Amino acid variants of hladrb1 confer susceptibility to dapsone. Case report severe dapsone hypersensitivity syndrome o sener,1 l doganci,2 m safali,3 b besirbellioglu,4 f bulucu,5 a pahsa 4 1 division of allergy, 2 department of microbiology and clinical microbiology, 3 department of pathology, 4 department of infectious diseases, and 5 department of internal medicine gulhane military medical academy. Drug reaction with eosinophilia and systemic symptoms dress syndrome formed by the acronym derived from the term drug rash with eosinophilia and systemic symptoms, coined by bocquet et al, also known as druginduced hypersensitivity syndrome dihs, was first recognized in 1950 by chaiken, in a patient using anticonvulsant. Sethuraman, dapsone sulfone syndrome, indian journal of dermatology, venerology and leprology, 66 iii, 2000, 117 120. An unusual case of dapsone hypersensitivity syndrome in a leprosy patient. Dapsone syndrome is a manifestation of the dress drug rash with eosinophilia and systemic symptoms syndrome which is a serious condition that has been reported in association with various drugs. The pathogenesis of the dapsone syndrome is as yet unknown. Studies in the rat revealed that dapsone dependent methaemoglobinaemia could be greatly diminished. Sep 10, 2011 hypersensitivity reactions to dapsone are potentially fatal adverse drug reactions with unknown prevalence and risk factors. Here we report a case of severe, life threatening dapsone systemic hypersensitivity syndrome in a 17yearold male who. Dec 24, 2018 dosage should be reduced to a minimum maintenance level as soon as possible.
The clinical presentation consisted of shortness of breath, occasional hemoptysis. Aug 25, 2016 yeah, im trying to read up, and from what i can tell about 1% of patients experience dapsone hypersensitivity syndrome, and about 10% of those cases it is fatal. Prompt withdrawal of dapsone is an important aspect in management of dapsone hypersensitivity syndrome. Targeted therapy guided by singlecell transcriptomic. Large doses of corticosteroids should be given if severe reactions or neuritisoccurs. Journal of the association of physicians of india vol 62 published on 1st of every month 1st august, 2014 dapsone hypersensitivity syndrome with myocarditis carmen m pereira, marina vaz, sindhoora kotha, nh santosh abstract drug hypersensitivity with myocarditis is known to occur with many drugs especially with antiepileptics. Dapsone dosing, indications, interactions, adverse effects. Dapsone hypersensitivity syndrome is a rare yet severe adverse drug reaction. Dapsone hypersensitivity syndrome that occurred during.
Skin biopsy showing perivascular lymphocytic infiltrates with. Dapsone syndrome first malaysian paediatric case report. The need for a more reliable route of administration led to investigation the possibility of an i. Dapsone hypersensitivity syndrome background dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. Dapsone hypersensitivity syndrome dhs is a rare, but potentially lifethreatening reaction to dapsone. Deaths associated with the administration of dapsone have been reported from agranulocytosis, aplastic anemia and other blood dyscrasias. Dapsone definition of dapsone by the free dictionary. Table table1 1 lists the multiple adverse effects of dapsone, including the dapsone hypersensitivity syndrome dhs and dress syndrome. The descriptive content of the publications was studied and merged, and the. Case study by journal of pakistan association of dermatologists. Anticonvulsant hypersensitivity syndrome ahs is a rare and lifethreatening delayed drug hypersensitivity reaction characterized by skin eruption, fever, lymphadenopathies, and. Detachment of the skin affects less than 10 % of bsa. Download fulltext pdf download fulltext pdf severe dapsone hypersensitivity syndrome in a child article pdf available in korean journal of pediatrics 566. Itching and blister formation can be controlled with 100 to 200 mgd of dapsone in most 1.
This syndrome is an idiosyncratic drug reaction which occurs in 0. Terms such as mononucleosislike syndrome and febrile mucocutaneous syndrome are also used. The authors report a case of dapsone induced methemoglobinemia observed in the emergency department during routine workup for contact dermatitis in a patient with celiac disease. Dapsone hypersensitivity syndrome dhs is a rare potentially fatal systemic idiosyncratic adverse reaction, with multiorgan involvement also known as sulphone syndrome which is particularly seen in leprosy patients who are on world health organization recommended multidrug therapy whomdt regimen.
Tell your doctor all prescription or overthecounter medicines and supplements you use. Dapsone hypersensitivity syndrome dhs is a rare yet serious adverse. A 50yearold caucasian male with a history of aids was admitted with suspicion of pneumonia. Sep, 2012 it is also used as an adjuvant in the treatment of pemphigus and pemphigoid. The patient should be warned to respond to the presence of clinical signs such as sore throat, fever, pallor, purpura or jaundice. Dapsone avlosulfon aids information, education, action. An unusual case of dapsone hypersensitivity syndrome in a. Finally, we diagnosed the patient with dapsone hypersensitivity syndrome dhs, a rare adverse event of this drug. The course of dapsone syndrome is variable, but it may last for four weeks or more. We present a case report of a 52 years old female with a recent history of antecedent dapsone exposure of 100 mg daily for 2 weeks.
Hla screening for dapsone hypersensitivity in indiaa long. Generation and characterization of dapsone and nitroso. Dec 24, 2018 dapsone 100 mg daily is continued 3 to10 years until all signs of clinical activity are controlled with skin scrapings and biopsies negative for one year. Efficacy of dapsone in the treatment of pemphigus and pemphigoid. Dapsone hypersensitivity syndrome dhs can be considered a manifestation of dress drug rash with eosinophilia and systemic symptoms syndrome and is an idiosyncratic multiorgan disease.
These have included methemoglobinemia, aplastic anemia, leukopenia, agranulocytosis, eosinophilia, macrocytic anemia, and heinz bodies. Case report dapsone hypersensitivity syndrome in an adolescent during treatment of leprosy fabio bucaretchi1,2, danielly c. Jul 11, 2011 dapsone 100 mg daily is continued 310 years until all signs of clinical activity are controlled with skin scrapings and biopsies negative for one year. It involves primarily a widespread skin rash, fever, swollen lymph nodes, and characteristic blood abnormalities such as an abnormally high level of eosinophils, low number of platelets, and increased number of atypical white. However, allday and barnes reported that it was probably due to hypersensitivity, because there was an interval of 5 to 6 weeks from the beginning of therapy in every case.
Drug reaction with eosinophilia and systemic symptoms. Over 10 million scientific documents at your fingertips. Hypersensitivity reactions to dapsone are potentially fatal adverse drug reactions with unknown prevalence and risk factors. A 50yearold male with dapsone hypersensitivity syndrome. Dapsone hypersensitivity syndrome with myocarditis. Hemolysis more likely to occur with deficiency of glucose 6phosphate dehydrogenase or g6pd, bone marrow aplasia, renal disease, peripheral neuropathy, methemoglobinemia, nausea, dizziness, fatigue and other systemic manifestations may.
Proper monitoring of patients initiated on dapsone therapy is essential. Dapsone may induce a doserelated hemolytic anemia, which is more likely with doses greater than 200 mgday or in patients with glucose6phosphate. Other drugs can interact with dapsone, especially those that may also affect the blood. Dapsone is used in the treatment of infections and inflammatory diseases. Dapsone hypersensitivity syndrome in an adolescent during. Lung injury unaccompanied by eosinophilia in the bronchoalveolar lavage fluid is even more rare as a dhsrelated lung manifestation. With the advent of who multidrug therapy for leprosy, more cases of dapsone syndrome have been reported. Assess patient for infection vital signs, sputum,wbcandmonitorrespiratorystatusrate,character,lungsounds,dysp. Dapsone induced cholangitis as a part of dapsone syndrome.
Anticonvulsant hypersensitivity syndrome request pdf. Health, general dapsone dosage and administration research drug allergy drug hypersensitivity leprosy complications and side effects. The term hypersensitivity syndrome hss has long been used in different ways to. Sep 05, 2011 the need for a more reliable route of administration led to investigation the possibility of an i. The present report describes a near fatal case of dhs in an adolescent. Dapsone hypersensitivity syndrome dhs is one of the most serious adverse reaction of dapsone. Dapsone is widely used in the treatment of leprosy and several chronic. Dapsone hypersensitivity syndrome in a patient with.
To the editor we congratulate liu et al1 for their work involving proactive hla screening to prevent dapsone hypersensitivity syndrome dhs in patients with leprosy. Nephrotic syndrome associated with hypocomplementaemic urticarial vasculitis syndrome. Journal of occupational medicine and toxicology biomed. It is a secondline medication for the treatment and prevention of pneumocystis pneumonia and for the prevention of toxoplasmosis in those who have poor immune function. Case report severe dapsone hypersensitivity syndrome. Shear, md, frcpc toronto, ontario, canada we describe a 22yearold woman with cutaneous polyarteritis nodosa in whom dapsone hy persensitivity syndrome dhs developed 5 weeks after initiation of dapsone therapy. Tresoldi1 summary a 12 y old girl was admitted 24 days after start a who multidrug therapy scheme for multibacillary leprosy dapsone. To achieve effective blood levels for 34 weeks, suspensions of large dapsone particles in an aqueous vehicle were made.
To date, the mdt regimen remains the standard treatment of leprosy. Dapsone definition of dapsone by medical dictionary. Dapsone hypersensitivity syndromerelated lung injury. Physicians should be familiar with this sulfone syndrome, an uncommon, potentially serious, side effect of dapsone therapy. An unusual case of dapsone syndrome grace m indian dermatol. S295, 2011keywords dapsone, drug hypersensitivity syndrome, erythema elevatum diutinum introduction dapsone is indicated for the treatment of leprosy, but it is also used for various skin diseases1. Aug 11, 2003 dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. Alzahrani, aa 2018 generation and characterization of dapsone and nitroso dapsone specific tcells. Dapsone 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of skin diseases, bacteria, and fungi. Dapsone hypersensitivity syndrome ronald prussick, md, frcpc, and nell h. S295, 2011keywordsdapsone, drug hypersensitivity syndrome, erythema elevatum diutinum introduction dapsone is indicated for the treatment of leprosy, but it is also used for various skin diseases1. Lakshmi department of dermatology, osmania medical collegehospital hyderabad.
Dapsone hypersensitivity typically develops after 46 weeks of treatment and patients must be warned to stop the drug and seek urgent medical attention if symptoms develop. The dapsone hypersensitivity syndrome, which is associated with a reported mortality of 9. Presumed dapsoneinduced drug hypersensitivity syndrome. Pathophysiologic mechanisms, diagnosis, and management of. Dapsone hypersensitivity syndrome dhs is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy. Increase in the incidence of dapsone hypersensitivity syndrome an appraisal p. Dapsone fda prescribing information, side effects and uses. Dapsone, also known as diaminodiphenyl sulfone dds, is an antibiotic commonly used in combination with rifampicin and clofazimine for the treatment of leprosy. Dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome dhs, which is potentially fatal. Generation and characterization of dapsone and nitroso dapsone specific tcells. Dapsoneinduced sulfone syndrome kimberly b lee, trisha. During pregnancy, dapsone should be used only when prescribed.
Currently, no tests are available to predict the risk of the dapsone hypersensitivity syndrome. Dapsone 100 mg daily is continued 310 years until all signs of clinical activity are controlled with skin scrapings and biopsies negative for one year. Increase in the incidence of dapsone hypersensitivity. Find patient medical information for dapsone oral on webmd including its uses, side effects and safety, interactions, pictures, warnings and user ratings. Dapsone should then be continued for an additional 10 years for borderline patients and for life for lepromatous patients. It is not doserelated, usually occurs in doses of 50300 mgd, all cases occur within 2 months of starting dapsone, all patients have fever, and most patients will develop rash and evidence of hepatic injury. Dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. Find, read and cite all the research you need on researchgate we use cookies to make interactions with our website easy and meaningful, to better understand the use of our. Dapsone syndrome, drug hypersensitivity reaction, drug reaction with eosinophilia and systemic 3symptoms dress, leprosy introduction dapsone syndrome is a potentially fatal hypersensitivity. The reaction included fever, malaise, dermatitis, jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia.
In the literature, the effect of dapsone on the heart has only been described twice. Mar 01, 2019 hypersensitivity to dapsone andor its derivatives. Dapsone sulfone syndrome cme thappa d m, sethuraman g. The metabolite can combinemodify some immunologicallysignificant chemicals, such as. Pdf severe dapsone hypersensitivity syndrome in a child. Sulfone syndrome is not a wellknown sequela of dapsone therapy. The pathophysiologic mechanisms, diagnosis, and management of dapsone. Herein, we describe the practical difficulties in translating this screening into clinical practice in india. Dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia.
Dapsone hypersensitivity syndrome dhs is a rare yet potentially fatal adverse drug reaction caused by dapsone 4,4 0 diaminodiphenylsulfone dds, a widely used medication in the treatment of. Dapsone 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of. Drug hypersensitivity syndrome usually occurs 1 to 8 weeks into the first course of therapy with the associated medication. Drug reaction with eosinophilia and systemic symptoms dress, also termed druginduced hypersensitivity syndrome dihs, is a rare reaction to certain medications. Druginduced hypersensitivity syndromedrug reaction with eosinophilia and. Dapsone hypersensitivity syndrome not related to g6pd. Drug hypersensitivity syndrome kumari r, timshina dk.
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