Druginduced hypersensitivity syndrome dihs is a severe and rare systemic reaction triggered by a drug usually an antiepileptic drug. A case of borderline tuberculoid leprosy who developed this syndrome is reported. 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. Case report, clinical report by national journal of physiology, pharmacy and pharmacology. Drug hypersensitivity syndrome is a delayed t cell mediated reaction. Learn types hypersensitivity disorder with free interactive flashcards.
There is a genetic predisposition to drug hypersensitivity syndrome. Furthermore, there had been reports of dapsone hypersensitivity syndrome. 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. Diaminodiphenyl sulphone dapsone is a drug of choice in the treatment of leprosy.
It may occur in 56 weeks to 6 months after initial treatment in leprosy patients. With the introduction of multidrug therapy for leprosy worldwide and with the use of dapsone in chemoprophylaxis for p. Dapsone induced hypersensitivity syndrome, hemolytic anemia, and severe agranulocytosis article pdf available june 2015 with 102 reads how we measure reads. Dapsone hypersensitivity syndrome ronald prussick, md, frcpc, and nell h. The dose you take depends on the type of infection you are trying to treat or prevent. To achieve effective blood levels for 34 weeks, suspensions of large dapsone particles in an aqueous vehicle were made. 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 syndrome is an uncommon hypersensitivity reaction to diamino diphenyl sulfone. Clinical features of druginduced hypersensitivity syndrome. Dapsone is one of the commonly implicated drugs in drug induced systemic hypersensitivity syndrome, apart from anticonvulsants, sulfonamides, allopurinol, nonsteroidal antiinflammatory drugs and minocycline. Amino acid variants of hladrb1 confer susceptibility to. Drug hypersensitivity syndrome kumari r, timshina dk, thappa. However, the use of dapsone may be associated with a plethora of.
This entity is also termed drug reaction with eosinophilia and systemic symptoms dress 5, and dhs has been considered a manifestation of dress syndrome. We describe a 22yearold woman with cutaneous polyarteritis nodosa in whom dapsone hypersensitivity syndrome dhs developed 5 weeks after initiation of dapsone therapy. Drug reaction with eosinophilia and systemic symptoms. This druginduced hypersensitivity syndrome also known as sulfone syndrome consists of exfoliative dermatitis andor other skin rashes, generalised lymphadenopathy, hepatosplenomegaly, fever and hepatitis. 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. Leukocytoclastic vasculitis resolution with topical dapsone. Dapsone syndrome is one of the rare hypersensitivity reactions.
Drug reaction with eosinophilia and systemic symptoms dress, also termed druginduced hypersensitivity syndrome dihs, is a rare reaction to certain medications. The classes of antibody involved are the same ones that participate in type ii reactionsigg and igmbut the mechanism. Reactivation of human herpesvirus 6 hhv6, the cause of roseola or. 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. Treatment is drug discontinuation, supportive treatment eg, with antihistamines, and sometimes desensitization. Dapsone hypersensitivity syndrome causing disseminated.
Use of certain antiretroviral arv drugs may cause hypersensitivity syndrome. Dapsone is available in tablets of 25 or 100 milligrams mg. Drug reaction with eosinophilia and systemic symptoms wikipedia. Dapsone hypersensitivity syndrome that occurred during. Some or all of these signs and symptoms may be present in less than 0. Cramping bronchospasm hypotension hypersensitivity. Pdf hypersensitivity syndrome associated with dapsone.
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. Dapsone syndrome first malaysian paediatric case report. Dapsone hypersensitivity syndrome dhs is one of the most serious adverse reaction of dapsone. The most serious hypersensitivity reaction to dapsone is known as the dapsone hypersensitivity syndrome which is likely a form of dihsdress. Druginduced hypersensitivity syndrome dihs, also termed as drug reaction with eosinophilia and systemic symptoms dress, is a multiorgan systemic. Drug hypersensitivity msd manual professional edition. Studies in the rat revealed that dapsone dependent methaemoglobinaemia could be greatly diminished. Drug hypersensitivity syndrome kumari r, timshina dk. Hypersensitivity reactions to non betalactam antimicrobial. In recent years, there have been major advances in the field of cough research. An unusual case of dapsone hypersensitivity syndrome in a.
Leucocytosis, abnormal erythrocytes with anemia, increase in number of eosinophils also have been reported, associated with this syndrome. Dapsone avlosulfon aids information, education, action. Dapsone hypersensitivity syndrome dhs is a rare, but potentially lifethreatening reaction to dapsone. Reactions classically begin 1 week to 8 weeks after starting drug therapy.
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. A 50yearold caucasian male with a history of aids was admitted with suspicion of pneumonia. Increase in the incidence of dapsone hypersensitivity. Dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. Dhs is a variant of drug rash with eosinophilia and. 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. Leprosy is a stigmatizing, chronic infection which degenerates the nervous system and often leads to incapacitation. The syndrome is a severe idiosyncratic drug reaction characterized by the. 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. General medicine, maharaja agrasen institute of medical education and research, agroha, haryana, india corresponding author. Dapsone 4, 4 diaminodiphenyl sulphone induced hypersensitivity is. Dapsone sulfone syndrome cme thappa d m, sethuraman g.
Pdf dapsoneinduced hypersensitivity syndrome, hemolytic. Download fulltext pdf download fulltext pdf severe dapsone hypersensitivity syndrome in a child article pdf available in korean journal of pediatrics 566. Case study by journal of pakistan association of dermatologists. Treatment is drug discontinuation, supportive treatment eg, with antihistamines, and. The link between hypersensitivity syndrome reaction. We describe a patient with disseminated intravascular coagulation dic as an adverse reaction to dapsone. 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. Dress druginduced hypersensitivity syndrome scielo. A defect in the way the liver metabolises drugs may be responsible. The need for a more reliable route of administration led to investigation the possibility of an i. The dapsone hypersensitivity syndrome, which is associated with a reported mortality of 9.
She had fever and cervical lymphadenopathy, and later a widespread erythematous eruption studded with pustules developed. A hypersensitivity reaction to lowdose 50mg, shortterm daspone administration developed in a 16yearold girl. 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 in a leprosy patient. For pht, the mean interval to onset is 17 to 21 days.
Current concepts on the diagnosis and pathogenesis of drug. 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. Agranulocytosis, methemoglobinemia, hemolytic anemia, reticulocytosis. Dapsone 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of skin diseases, bacteria, and fungi. Tissue damage is due to cytotoxic t cells and cytokine release. This contrasts with other hypersensitivity reactions such as igemediated anaphylaxis to penicillin, which can occur following multiple courses of drug therapy. Background dapsone is used in the treatment of infections and inflammatory diseases. Health, general dapsone dosage and administration research drug allergy drug hypersensitivity leprosy complications and side effects. Multidrug therapy mdt which consists of dapsone, rifampicin and clofazimine has been effective to combat this disease.
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. Hypersensitivity syndrome definition of hypersensitivity. Following its initial description in 1989 by sullivan et al. 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. 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. Antiviral immune responses intertwined with drug hypersensitivity make the disease more complicated and protracted. It is normally taken once a day or three times a week. Dapsone hypersensitivity syndrome a fatal adverse drug reaction. It is an immunemediated reaction involving macrophage and tlymphocyte activation. Jan 06, 2011 drug hypersensitivity syndrome usually occurs 1 to 8 weeks into the first course of therapy with the associated medication. Comparison of reporting of stevensjohnson syndrome and toxic epidermal necrolysis in association with selective cox2 inhibitors. Recent advances in druginduced hypersensitivity syndromedrug. Dapsone hypersensitivity syndromerelated lung injury. Gout treatment dosage affects risk for hypersensitivity syndrome.
Immune system disorder immune system disorder type iii hypersensitivity. Drug reaction with eosinophilia and systemic symptoms dress drug induced hypersensitivity syndrome dihs. In our case, though serum transaminase levels were found normal, serum bilirubin was high indicating involvement of liver. Presumed dapsoneinduced drug hypersensitivity syndrome. This syndrome is characterised by high fever, maculopapular rashes, hepatitis, lymphadenopathy and lymphocytosis. Immune system disorder type iii hypersensitivity britannica. The clinical picture of this syndrome is highly variable and not. Pdf severe dapsone hypersensitivity syndrome in a child. Furthermore, tomecki and catalano suggested that the hypersensitivity reaction of dapsone was not dose related. 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. This hypersensitivity reaction occurs in patients who are unable to detoxify reactive metabolites produced by thyroid peroxidase.
Fringe medicine a hypothetical polysymptomatic condition attributed by socalled clinical ecologists to immune dysregulation induced by contaminants e. To the best of our knowledge, this is the first time it has been described in the literature. It is also useful for the treatment of many neutrophilic and other dermatoses. Dapsone is used in the treatment of infections and inflammatory diseases. The dapsone hypersensitivity syndrome is usually manifested 4 to 6 weeks after the initiation of therapy. 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. Case report a 48yearold female presented with highgrade fever, breathlessness, and cough with mucus expectoration for 3 days. Dapsone hypersensitivity syndrome is a rare yet severe adverse drug reaction caused by dapsone, a principal drug in multidrug therapy for leprosy. 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, a potent antiparasitic and antiinflammatory compound, is mainly used in the treatment of leprosy and a variety of blistering skin diseases.
It generally appears within first 6 weeks of treatment. Dapsone hypersensitivity syndrome not related to g6pd. Amino acid variants of hladrb1 confer susceptibility to dapsone hypersensitivity syndrome in addition to hlab. Dapsone hypersensitivity syndrome dhs is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy. Symptoms range from mild to severe and include rash, anaphylaxis, and serum sickness. Risk factors for fatalities from dapsone hypersensitivity syndrome. It may cause a severe adverse drug reaction with multiorgan involvement known as dapsone hypersensitivity syndrome. Discussion in the literature tends to focus on identifiable syndromes based on severity of symptoms see table 1. Dhs is characterized by exfoliative dermatitis, fever, internal organ involvement lungs, liver, spleen, and kidney.
Dapsone is an antibacterial medication used in the treatment of dermatitis herpetiformis a skin condition and leprosy hansens disease. Table table1 1 lists the multiple adverse effects of dapsone, including the dapsone hypersensitivity syndrome dhs and dress syndrome. The clinical presentation consisted of shortness of breath, occasional hemoptysis, odynophagia, skin rash. 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. Dapsone hypersensitivity syndrome overlaps reversal. Dapsone hypersensitivity syndrome is an idiosyncratic reaction to this drug and can present with different clinical manifestations of varying severity. 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. The reaction included fever, malaise, dermatitis, jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. Druginduced hypersensitivity syndrome dhs, also named drug rash with eosinophilia and systemic symptoms dress is a poten. 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. 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. 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. Abstract drug reaction with eosinophilia and systemic symptoms dress, also known as drug. A case of dapsone hypersensitivity syndrome dhs in an aids patient receiving dapsone for pneumocystis pneumonia pcp prophylaxis is discussed.
Drug hypersensitivity is an immunemediated reaction to a drug. 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. Druginduced hypersensitivity syndrome dihs, also called drug rash with eosinophilia and systemic symptoms dress, is a severe reaction usually. Case report severe dapsone hypersensitivity syndrome. Jun 23, 2012 druginduced hypersensitivity syndrome dihs is a rare systemic autoimmune disorder that can cause mild to severe mucosal and cutaneous reactions. Sunil kumar gothwal, assistant professor, department. Dapsone hypersensitivity syndrome dhs is a severe and distinct idiosyncratic adverse reaction with multiorgan involvement. Multiple drug hypersensitivity proof of multiple drug hypersensitivity by patch and lymphocyte transformation tests. 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.
Druginduced hypersensitivity syndrome dihs is a rare systemic autoimmune disorder that can cause mild to severe mucosal and cutaneous reactions. 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. Skin biopsy showing perivascular lymphocytic infiltrates with. Dapsoneinduced hypersensitivity syndrome, hemolytic. Confusing matters is the incidence of hypersensitivity reactions in hivpositive patients. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and. Is the druginduced hypersensitivity syndrome dihs due to. 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. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. Dhs is a rare, but still one of a serious adverse drug reaction adr of dapsone. Sunil k gothwal, surendra khosya and nilesh gharpure. The pathogenesis of the dapsone syndrome is as yet unknown. Choose from 500 different sets of types hypersensitivity disorder flashcards on quizlet.
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