Amino acid variants of hladrb1 confer susceptibility to dapsone hypersensitivity syndrome in addition to hlab. Dapsone hypersensitivity syndrome overlaps reversal. Druginduced hypersensitivity syndrome dihs is a rare systemic autoimmune disorder that can cause mild to severe mucosal and cutaneous reactions. General medicine, maharaja agrasen institute of medical education and research, agroha, haryana, india corresponding author.
Reactions classically begin 1 week to 8 weeks after starting drug therapy. Symptoms range from mild to severe and include rash, anaphylaxis, and serum sickness. Hypersensitivity reactions to non betalactam antimicrobial. An unusual case of dapsone hypersensitivity syndrome in a. Drug hypersensitivity syndrome kumari r, timshina dk, thappa. Gout treatment dosage affects risk for hypersensitivity syndrome. Druginduced hypersensitivity syndrome dihs, or drug rash with eosinophilia and systemic symptoms dress syndrome, is a serious acute drug reaction that is characterized by fever, cutaneous eruption, and involvement of several internal organs in the form of enlarged lymph nodes, renal impairment, pneumonitis, carditis, and hematologic. Tissue damage is due to cytotoxic t cells and cytokine release. May, 2016 while sobbing at the drop of a hat is known to be a key indicator of someone who is highly emotional, there are other notsosubtle signs that youre a hypersensitive person. Dapsone hypersensitivity syndrome that occurred during. Drug hypersensitivity syndrome usually occurs on first exposure to the associated medication, with a delayed onset.
The clinical presentation consisted of shortness of breath, occasional hemoptysis, odynophagia, skin rash. This syndrome is characterised by high fever, maculopapular rashes, hepatitis, lymphadenopathy and lymphocytosis. It generally appears within first 6 weeks of treatment. 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 blood. Dapsone is available in tablets of 25 or 100 milligrams mg. The pathogenesis of the dapsone syndrome is as yet unknown. Dhs is characterized by exfoliative dermatitis, fever, internal organ involvement lungs, liver, spleen, and kidney. The dapsone hypersensitivity syndrome is usually manifested 4 to 6 weeks after the initiation of therapy. Jan 06, 2011 drug hypersensitivity syndrome usually occurs 1 to 8 weeks into the first course of therapy with the associated medication.
Dapsone hypersensitivity syndrome in a leprosy patient. Jun 23, 2012 druginduced hypersensitivity syndrome dihs is a rare systemic autoimmune disorder that can cause mild to severe mucosal and cutaneous reactions. Immune system disorder immune system disorder type iii hypersensitivity. A 50yearold caucasian male with a history of aids was admitted with suspicion of pneumonia. Download fulltext pdf download fulltext pdf severe dapsone hypersensitivity syndrome in a child article pdf available in korean journal of pediatrics 566. However, the use of dapsone may be associated with a plethora of adverse effects, some of which may involve. Furthermore, tomecki and catalano suggested that the hypersensitivity reaction of dapsone was not dose related.
Dapsone 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of skin diseases, bacteria, and fungi. There are challenges in the clinical diagnosis of druginduced injury and in obtaining information on the reactivation of human herpes viruses hhv during idiosyncratic adverse drug reactions. Is the druginduced hypersensitivity syndrome dihs due to. Amino acid variants of hladrb1 confer susceptibility to. Dapsone avlosulfon aids information, education, action.
Presumed dapsoneinduced drug hypersensitivity syndrome. For pht, the mean interval to onset is 17 to 21 days. She had fever and cervical lymphadenopathy, and later a widespread erythematous eruption studded with pustules developed. Reactivation of human herpesvirus 6 hhv6, the cause of roseola or. A hypersensitivity reaction to lowdose 50mg, shortterm daspone administration developed in a 16yearold girl. Risk factors for fatalities from dapsone hypersensitivity syndrome. It is an immunemediated reaction involving macrophage and tlymphocyte activation.
Dapsone is used in the treatment of infections and inflammatory diseases. The link between hypersensitivity syndrome reaction. Pdf dapsoneinduced hypersensitivity syndrome, hemolytic. Dapsone induced hypersensitivity syndrome, hemolytic anemia, and severe agranulocytosis article pdf available june 2015 with 102 reads how we measure reads. Drug reaction with eosinophilia and systemic symptoms dress drug induced hypersensitivity syndrome dihs. Learn types hypersensitivity disorder with free interactive flashcards. Multidrug therapy mdt which consists of dapsone, rifampicin and clofazimine has been effective to combat this disease. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. Dapsone is one of the commonly implicated drugs in drug induced systemic hypersensitivity syndrome, apart from anticonvulsants, sulfonamides, allopurinol, nonsteroidal antiinflammatory drugs and minocycline. Treatment is drug discontinuation, supportive treatment eg, with antihistamines, and. A defect in the way the liver metabolises drugs may be responsible. Leucocytosis, abnormal erythrocytes with anemia, increase in number of eosinophils also have been reported, associated with this syndrome. Confusing matters is the incidence of hypersensitivity reactions in hivpositive patients. Leprosy is a stigmatizing, chronic infection which degenerates the nervous system and often leads to incapacitation.
Dapsone hypersensitivity syndrome dhs is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy. Dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. 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. Drug reaction with eosinophilia and systemic symptoms. To the best of our knowledge, this is the first time it has been described in the literature. To achieve effective blood levels for 34 weeks, suspensions of large dapsone particles in an aqueous vehicle were made. Sunil k gothwal, surendra khosya and nilesh gharpure. Dapsone hypersensitivity syndrome is a rare yet severe adverse drug reaction caused by dapsone, a principal drug in multidrug therapy for leprosy. In recent years, there have been major advances in the field of cough research. Skin biopsy showing perivascular lymphocytic infiltrates with.
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, sulphacompounds and daposne. Some or all of these signs and symptoms may be present in less than 0. Drug reaction with eosinophilia and systemic symptoms dress, also termed druginduced hypersensitivity syndrome dihs, is a rare reaction to certain medications. Drug reaction with eosinophilia and systemic symptoms wikipedia. Hypersensitivity syndrome definition of hypersensitivity. However, the use of dapsone may be associated with a plethora of. Dapsone is an antibacterial medication used in the treatment of dermatitis herpetiformis a skin condition and leprosy hansens disease. An unusual case of dapsone hypersensitivity syndrome in a leprosy patient. 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. Case report severe dapsone hypersensitivity syndrome. Clinical features of druginduced hypersensitivity syndrome. Druginduced hypersensitivity syndrome dihs, also called drug rash with eosinophilia and systemic symptoms dress, is a severe reaction usually.
A case of borderline tuberculoid leprosy who developed this syndrome is reported. 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. Druginduced hypersensitivity syndrome dihs is a severe and rare systemic reaction triggered by a drug usually an antiepileptic drug. Dapsone hypersensitivity syndrome dhs is a rare, but potentially lifethreatening reaction to dapsone. Dapsone hypersensitivity syndrome not related to g6pd. We describe a patient with disseminated intravascular coagulation dic as an adverse reaction to dapsone. 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. Drug hypersensitivity is an immunemediated reaction to a drug. The reaction included fever, malaise, dermatitis, jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. Druginduced hypersensitivity syndrome dihs is a lifethreatening, multiorgan system reaction. Current concepts on the diagnosis and pathogenesis of drug. It may cause a severe adverse drug reaction with multiorgan involvement known as dapsone hypersensitivity syndrome.
We describe a 22yearold woman with cutaneous polyarteritis nodosa in whom dapsone hypersensitivity syndrome dhs developed 5 weeks after initiation of dapsone therapy. The dose you take depends on the type of infection you are trying to treat or prevent. 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. Agranulocytosis, methemoglobinemia, hemolytic anemia, reticulocytosis. 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. Dhs is a variant of drug rash with eosinophilia and. Dapsone hypersensitivity syndrome dhs is a severe and distinct idiosyncratic adverse reaction with multiorgan involvement. The syndrome is a severe idiosyncratic drug reaction characterized by the. Discussion in the literature tends to focus on identifiable syndromes based on severity of symptoms see table 1. Furthermore, there had been reports of dapsone hypersensitivity syndrome. Use of certain antiretroviral arv drugs may cause hypersensitivity syndrome. The clinical picture of this syndrome is highly variable and not. Druginduced hypersensitivity syndrome dhs, also named drug rash with eosinophilia and systemic symptoms dress is a poten.
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. Beacons chemicals pte ltd, singapore is routinely prescribed for antimalarial chemoprophylaxis in military servicemen in singapore who are not glucose6phosphate dehydrogenasedeficient. In our case, though serum transaminase levels were found normal, serum bilirubin was high indicating involvement of liver. Diaminodiphenyl sulphone dapsone is a drug of choice in the treatment of leprosy. Choose from 500 different sets of types hypersensitivity disorder flashcards on quizlet. This contrasts with other hypersensitivity reactions such as igemediated anaphylaxis to penicillin, which can occur following multiple courses of drug therapy. There is a genetic predisposition to drug hypersensitivity syndrome. Dapsone hypersensitivity syndrome in a patient with.
Dapsoneinduced hypersensitivity syndrome, hemolytic anemia, and severe agranulocytosis article pdf available june 2015 with 102 reads how we measure reads. Drug hypersensitivity syndrome is a delayed t cell mediated reaction. Type iii, or immunecomplex, reactions are characterized by tissue damage caused by the activation of complement in response to antigenantibody immune complexes that are deposited in tissues. This druginduced hypersensitivity syndrome also known as sulfone syndrome consists of exfoliative dermatitis andor other skin rashes, generalised lymphadenopathy, hepatosplenomegaly, fever and hepatitis.
Dapsone hypersensitivity syndrome dhs was first described in the 1930s as involving several types of adverse reactions caused by the drug, including skin rashes, fever and eosinophilia 2. Treatment is drug discontinuation, supportive treatment eg, with antihistamines, and sometimes desensitization. Comparison of reporting of stevensjohnson syndrome and toxic epidermal necrolysis in association with selective cox2 inhibitors. The most serious hypersensitivity reaction to dapsone is known as the dapsone hypersensitivity syndrome which is likely a form of dihsdress. Dapsone hypersensitivity syndrome causing disseminated. From the japanese association of medical sciences current concepts on the diagnosis and pathogenesis of druginduced hypersensitivity syndrome jmaj 525. Druginduced hypersensitivity syndrome dress by phenobarbital. It may occur in 56 weeks to 6 months after initial treatment in leprosy patients. Dapsone hypersensitivity syndrome a fatal adverse drug reaction. Recent advances in druginduced hypersensitivity syndromedrug. Dapsone, a potent antiparasitic and antiinflammatory compound, is mainly used in the treatment of leprosy and a variety of blistering skin diseases.
Stevensjohnson syndrome, exfoliative dermatitis, hypersensitivity reactions including erythema nodosum leprosum, photosensitivity, systemic lupus erythematosus. Druginduced hypersensitivity syndrome dihs, also termed as drug reaction with eosinophilia and systemic symptoms dress, is a multiorgan systemic. It is also useful for the treatment of many neutrophilic and other dermatoses. Increase in the incidence of dapsone hypersensitivity. Drug hypersensitivity msd manual professional edition. Sunil kumar gothwal, assistant professor, department. It is normally taken once a day or three times a week.
Importance dapsone hypersensitivity syndrome dhs is the most serious adverse reaction associated with dapsone administration and one of the major causes of death in patients with leprosy, whose standard treatment includes multidrug therapy mdt with dapsone, rifampicin, and clofazimine. Systemic dapsone also is an effective therapy for chronic andor symptomatic lcv. Pdf hypersensitivity syndrome associated with dapsone. Druginduced hypersensitivity syndrome dihs, also called drug rash with eosinophilia and systemic symptoms dress, is a severe reaction usually characterized by fever, rash, and multiorgan failure, occurring 1y8 weeks after drug introduction. Dapsone sulfone syndrome cme thappa d m, sethuraman g. Dapsone has rare, but lifethreatening side effects, known as dapsone hypersensitivity syndrome dhs or sulfon syndrome. Background dapsone is used in the treatment of infections and inflammatory diseases. This hypersensitivity reaction occurs in patients who are unable to detoxify reactive metabolites produced by thyroid peroxidase.
A case of dapsone hypersensitivity syndrome dhs in an aids patient receiving dapsone for pneumocystis pneumonia pcp prophylaxis is discussed. Fringe medicine a hypothetical polysymptomatic condition attributed by socalled clinical ecologists to immune dysregulation induced by contaminants e. Case study by journal of pakistan association of dermatologists. With the introduction of multidrug therapy for leprosy worldwide and with the use of dapsone in chemoprophylaxis for p. Dapsone hypersensitivity syndromerelated lung injury.
Immune system disorder type iii hypersensitivity britannica. This entity is also termed drug reaction with eosinophilia and systemic symptoms dress 5, and dhs has been considered a manifestation of dress syndrome. Health, general science and technology, general allergic reaction care and treatment complications and side effects allergy anemia dosage and administration usage drug allergy case studies causes of drug hypersensitivity. The classes of antibody involved are the same ones that participate in type ii reactionsigg and igmbut the mechanism. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and.
Leukocytoclastic vasculitis resolution with topical dapsone. Allopurinol hypersensitivity syndrome ahs, a rare but potentially fatal adverse reaction in patients beginning allopurinol therapy for gout, might be largely avoided by keying the starting dose to kidney function and slowly increasing it to therapeutic levels once tolerance is certain, according to a retrospective casecontrol study. Dapsone hypersensitivity syndrome dhs is one of the most serious adverse reaction of dapsone. Dapsone syndrome first malaysian paediatric case report. May 06, 2020 hypersensitivity syndrome is characterized by fever, rash, organ involvement most frequently the liver, and high blood levels of eosinophils a type of white blood cell. The need for a more reliable route of administration led to investigation the possibility of an i. Studies in the rat revealed that dapsone dependent methaemoglobinaemia could be greatly diminished. Dapsone syndrome is one of the rare hypersensitivity reactions.
Antiviral immune responses intertwined with drug hypersensitivity make the disease more complicated and protracted. The dapsone hypersensitivity syndrome, which is associated with a reported mortality of 9. Following its initial description in 1989 by sullivan et al. Table table1 1 lists the multiple adverse effects of dapsone, including the dapsone hypersensitivity syndrome dhs and dress syndrome. Feb 20, 2019 the dapsone syndrome is manifested by viral illnesslike symptoms fever, chills, myalgias, arthralgias, exanthema, lymphadenopathy, edema, lymphocytosis, hepatomegaly, elevated liver function tests, methemoglobinemia, and anemia. Dress druginduced hypersensitivity syndrome scielo. Manifestations of hypersensitivity site of exposure syndrome common allergens symptoms skin contact urticaria hives pruritis contact dermatitis rash pruritis blood systemic allergy hivesedema abd. 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. Case report a 48yearold female presented with highgrade fever, breathlessness, and cough with mucus expectoration for 3 days. Dapsone hypersensitivity syndrome is an idiosyncratic reaction to this drug and can present with different clinical manifestations of varying severity. Case report, clinical report by national journal of physiology, pharmacy and pharmacology. Dapsone syndrome, drug hypersensitivity reaction, drug reaction with eosinophilia and systemic 3symptoms dress, leprosy introduction dapsone syndrome is a potentially fatal hypersensitivity reaction to sulphone characterised by high fever associated. 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. Multiple drug hypersensitivity proof of multiple drug hypersensitivity by patch and lymphocyte transformation tests.
Dhs is a rare, but still one of a serious adverse drug reaction adr of dapsone. Dapsone hypersensitivity syndrome ronald prussick, md, frcpc, and nell h. Dapsone 4, 4 diaminodiphenyl sulphone induced hypersensitivity is. 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. Dapsone syndrome is an uncommon hypersensitivity reaction to diamino diphenyl sulfone. Drug hypersensitivity syndrome kumari r, timshina dk. Dapsoneinduced hypersensitivity syndrome, hemolytic.
1312 1153 295 716 23 1366 1555 532 54 1116 1590 1404 959 1377 1330 1467 1366 1311 1252 465 409 380 1252 1398 641 615 1387 325 111 549 723 99 462 266 1397 578 691 1073