Tresoldi1 summary a 12 y old girl was admitted 24 days after start a who multidrug therapy scheme for multibacillary leprosy dapsone. Risk factors for fatalities from dapsone hypersensitivity syndrome. 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. Drug reaction with eosinophilia and systemic symptoms. Dress syndrome is characterized by fever, cutaneous eruption, internal organ involvement, and hematologic abnormalities 18 weeks after the administration of the culprit drug, 1 with an incidence ranging from 1 in 1,000 to 1 in 10,000 exposures in patients taking the drug. Dapsone induced cholangitis as a part of dapsone syndrome. It is included with each meddra release, and is also accessible on the meddra website under support documentation. Dapsone hypersensitivity syndrome dhs is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy.
Dapsone hypersensitivity syndrome dhs is a rare yet potentially fatal adverse drug reaction caused by dapsone 4,4. It may cause a severe adverse drug reaction with multiorgan involvement known as dapsone hypersensitivity syndrome. The sulfone syndrome in a patient receiving dapsone. Drug reaction with eosinophilia and systemic symptoms dress, also termed druginduced hypersensitivity syndrome dihs, is a rare reaction to certain medications. It also was called hypersensitivity syndrome earlier, since first described by salzestein in 1959. The dapsone hypersensitivity syndrome revisited journal of. Early withdrawal of dapsone and appropriate treatment reduced the fatality rate. Dapsonetrimethoprim for pneumocystis carinii pneumonia in. The reaction included fever, malaise, dermatitis, jaundice with hepatic dysfunction, lymphadenopathy, and hemolytic anemia. Introductory guide for standardised meddra queries smqs.
If the inline pdf is not rendering correctly, you can download the pdf file here. Antibiotic hypersensitivity reactions and approaches to. Dapsone has been part of the world health organization multidrug therapy for the treatment of leprosy. Delayed type drug hypersensitivity boonthorn 2 august 2010. Not only has it been the drug of choice for the treatment of leprosy hansens disease since the middle of the 20th century, but it has also been. Genetic and immune predictors for hypersensitivity syndrome to antiepileptic drugs. The dapsone hypersensitivity syndrome, which is associated with a reported mortality of 9. Aug 11, 2003 dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. We encourage immediate discontinuation of the drug in a patient in whom a fever or flulike illness. Dapsone hypersensitivity syndrome ronald prussick, md, frcpc, and nell h. Uncommonly, weakness of the foot and hand muscles can occur, particularly with long term dapsone therapy with doses greater than 100 mg per. An unusual case of dapsone hypersensitivity syndrome in a. Dapsone is used in the treatment of infections and inflammatory diseases.
An unusual case of dapsone syndrome grace m indian dermatol. 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. Dapsone 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of skin diseases, bacteria, and fungi. Dapsone is occasionally used in the treatment of the autoimmune bullous diseases, a group of disorders resulting from autoimmunity directed against basement membrane andor intercellular adhesion molecules on cutaneous and mucosal surfaces. Dapsone hypersensitivity syndrome overlaps reversal reaction. 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. Dapsone hypersensitivity syndrome a fatal adverse drug reaction. Hypersensitivity is confined to the first 6 weeks of treatment.
Dapsone hypersensitivity syndrome is characterized by an exfoliative dermatitis, fever and hepatitis which may be lifethreatening. In the literature, the effect of dapsone on the heart has only been described twice. Dapsone hypersensitivity syndrome in a leprosy patient. Sep 10, 2011 hypersensitivity reactions to dapsone are potentially fatal adverse drug reactions with unknown prevalence and risk factors. The patients first presentation was superior vena cava syndrome due.
D r ug aller g y abdul waheed, md, tiffany hill, md, nidhi dhawan, md keywords drug reaction drug allergy hypersensitivity presentation diagnosis treatment prevention key points adverse drug reactions can be classified as type a reactions and type b reactions. The introductory guide for standardised meddra queries smqs, prepared in english, is intended only for use with the english version of meddra and its versionspecific smqs. Dapsone hypersensitivity syndrome overlaps reversal. 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 hypersensitivity syndrome with myocarditis. Currently, no tests are available to predict the risk of the dapsone hypersensitivity syndrome. Dapsone is also combined with pyrimethamine as maloprim beacons chemicals pte ltd, singapore, a fairly effective chemoprophylactic agent in the management of malaria.
The patient should be warned to respond to the presence of clinical signs such as sore throat, fever, pallor, purpura or jaundice. Full text adverse reactions in leprosy patients who. Sener o doqanci l safa li m besirbellioglu b bulucu f pahsa a. Dapsone is one of the commonly implicated drugs in drug induced systemic hypersensitivity syndrome, apart from anticonvulsants, sulfonamides, allopurinol, nonsteroidal antiinflammatory drugs and minocycline. Hypocomplementemic urticarial vasculitis syndrome huvs is a type iii hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Deaths associated with the administration of dapsone have been reported from agranulocytosis, aplastic anemia and other blood dyscrasias. 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. 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 andor hemolytic anemia, peripheral neuropathy and the potentially fatal dapsone hypersensitivity syndrome dhs. We report a 42yearold man who is a known case of behcets disease. Background dapsone is used in the treatment of infections and inflammatory diseases. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. Antimalarials, dapsone, exanthema introduction dapsone hypersensitivity syndrome dhs is a rare condition mostly occurring in leprosy patients on multidrug therapy. Dapsone hypersensitivity syndrome masquerading as a viral.
We report an unusual case of dapsone hypersensitivity, manifesting as bone marrow suppression and peripheral pancytopenia in addition to fever, rash, and hepatosplenomegaly. Neuman manuela g, cohen lawrence, nanau radu m, hwang paul. 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 occur singly or in combination in patients on dapsone therapy. Dapsone induced hypersensitivity syndrome a case report. The pathogenesis of the dapsone syndrome is as yet unknown. Download fulltext pdf download fulltext pdf severe dapsone hypersensitivity syndrome in a child article pdf available in korean journal of pediatrics 566. We report this rare case of dapsone hypersensitivity syndrome in a girl on dapsone who presented with fever, anaemia. The skin manifestations of hypersensitivity reaction to dapsone are exanthematous eruptions, exfoliative dermatitis, toxic epidermal necrolysis and stevensjohnson syndrome erythema multiforme bullosum. Learn about side effects, drug interactions, dosages, warnings, and more. 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. It is also useful for the treatment of many neutrophilic and other dermatoses. In 1950, lowe reported the first case of dapsone hypersensity, later on allday.
This article is from korean journal of pathology, volume 46. Although huvs is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often lifethreatening angioedema. Diaminodiphenyl sulphone dapsone is a drug of choice in the treatment of leprosy. Side effects, dosages, treatment, interactions, warnings. Drug reaction with eosinophilia and systemic symptoms wikipedia. 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. A 47yearold female complained of a spreading red patches on the skin of her trunk and.
Fatal dapsone hypersensitivity syndrome with hypothyroidism and. The case is being reported to emphasize the need for timely diagnosis and prompt treatment of this rare complication for successful outcomes. Dapsone hypersensitivity syndrome is a rare but well recognized. Dapsone hypersensitivity syndrome journal of the american.
An updated practice parameter these parameters were developed by the joint task force on practice parameters, representing the american academy of allergy, asthma and immunology, the american college of allergy, asthma and immunology, and the joint council of allergy, asthma and immunology. 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. Dapsone avlosulfon aids information, education, action. Dapsone sulfone syndrome cme thappa d m, sethuraman g. Dapsone hypersensitivity syndrome among leprosy patients.
Marshall 0 edwin swiatlo 3 0 department of medicine, division of clinical immunology and allergy 1 division of infectious diseases, university of alabama at birmingham 2 pharmacy. The most serious hypersensitivity reaction to dapsone is known as the dapsone hypersensitivity syndrome which is likely a form of dihsdress. All patients with the acquired immunodeficiency syndrome and a first episode of pneumocystis carinii pneumonia seen at the san francisco general hospital between november 1984 and april 1985 were evaluated for oral treatment with dapsone 100 mgd plus trimethoprim 20 mgkg body weight d. Pathophysiologic mechanisms, diagnosis, and management of. Cervical lymphadenopathy mimicking angioimmunoblastic t. Hla pharmacogenetic markers of drug hypersensitivity in a. Dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. Cid antibiotic hypersensitivity reactions and approaches to desensitization ellie j.
Dapsone hypersensitivity syndrome that occurred during. Nsaids antipsychotics antiepileptics sulphonamides antibiotics allopurinol dapsone. 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. Dapsone hypersensitivity syndromerelated lung injury. Adverse reactions in leprosy patients who underwent dapsone multidrug therapy. This is a case report whose dev was eradicated by band ligation for the first time.
However, the use of dapsone may be associated with a plethora of adverse effects, some of which may involve. 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. Mar 01, 2019 hypersensitivity to dapsone andor its derivatives. Dapsone hypersensitivity syndrome dhs is an adverse effect from dapsone therapy that may occur in 56 weeks to 6 months after initial treatment. Dapsone hypersensitivity syndrome longdom publishing sl. Bsrbrra event of special interest esi report serious skin reaction patient name. 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.
It is a kind of druginduced hypersensitivity syndrome dihs and is a triad of eruptions, fever. Dapsone fda prescribing information, side effects and uses. A rare life threatening complication of dapsone therapy. 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. Case report dapsone hypersensitivity syndrome in an adolescent during treatment of leprosy fabio bucaretchi1,2, danielly c. Get a printable copy pdf file of the complete article. Amino acid variants of hladrb1 confer susceptibility to. 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. A hypersensitivity reaction to lowdose 50mg, shortterm daspone administration developed in a 16yearold girl. An unusual case of dapsone hypersensitivity syndrome in a leprosy patient. 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. The woman with a history of linear iga disease was initiated on oral dapsone 100mg once daily. Find patient medical information for dapsone oral on webmd including its uses, side effects and safety, interactions, pictures, warnings and user ratings.
The internist approach to lymphocytosis clin assoc prof p kuperan frcp, frcpa, frcpath. Dapsone, a potent antiparasitic and antiinflammatory compound, is mainly used in the treatment of leprosy and a variety of blistering skin diseases. 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. Angioedema in a 47yearold woman with hypocomplementemic. Summary dapsone hypersensitivity syndrome dhs can be classified as a drug. Case report severe dapsone hypersensitivity syndrome. One of the rare presentations of superior vena cava syndrome is bleeding of downhill esophageal varices dev and different approaches have been used to control it. Dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome dhs, which is potentially fatal. Case study by journal of pakistan association of dermatologists. This syndrome is an idiosyncratic drug reaction which occurs in 0. Dapsone dosing, indications, interactions, adverse effects. A case report, pediatric transplantation on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Get a printable copy pdf file of the complete article 1. Article pdf available in journal of pharmacology and.
A 47yearold female complained of a spreading red patches on the skin of her trunk and extremities for three weeks duration. Abstracta 36yearold woman presented with erythematous confluent macules on her whole body with. Dapsone, a potent antiinflammatory and antiparasitic compound, is used worldwide for the treatment of patients with leprosy, malaria, and immunosuppressioninduced infections caused by pneumocystis carinii and toxoplasma gondii. Here we report a case of severe, life threatening dapsone systemic hypersensitivity syndrome in a 17yearold male who. 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. Dapsone in the management of the autoimmune bullous diseases. Check the full list of possible causes and conditions now. Dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. Toxic epidermal necrolysis has a mortality of 3035%, stevensjohnson syndrome 515% and dress syndrome 10%.
Dapsone is an antibacterial medication used in the treatment of dermatitis herpetiformis a skin condition and leprosy hansens disease. Health, general dapsone dosage and administration research drug allergy drug hypersensitivity leprosy complications and side effects. Bsrbrra event of special interest esi report serious. Table table1 1 lists the multiple adverse effects of dapsone, including the dapsone hypersensitivity syndrome dhs and dress syndrome. Pdf severe dapsone hypersensitivity syndrome in a child. Supplementary appendix this appendix has been provided by the authors to give readers additional information about their work. Read fulminant hepatitis linked to dapsone hypersensitivity syndrome requiring urgent living donor liver transplantation. This syndrome is an idiosyncratic drug reaction which occurs in.