This form of vasculitis is a rare form, with approximately 250 cases reported till date. Mortality due to dapsone hypersensitivity syndrome. Feb 20, 2019 hematologic side effects have been the most pronounced. 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 4,4diaminodiphenylsulphone is used mainly as an antiinflammatory and antibacterial agent for the treatment of skin diseases, bacteria, and fungi. Dapsoneinduced hypersensitivity syndrome, hemolytic anemia. Studies in the rat revealed that dapsonedependent methaemoglobinaemia could be greatly diminished. Dhs is characterized by exfoliative dermatitis, fever, internal organ involvement lungs, liver, spleen, and kidney. Jul 22, 2014 dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. Dapsone sulfone syndrome cme thappa d m, sethuraman g. To achieve effective blood levels for 34 weeks, suspensions of large dapsone particles in an aqueous vehicle were made. Dapsone is extensively used for a variety of infectious, immunological, and hypersensitivity disorders.
We describe a 22yearold woman with cutaneous polyarteritis nodosa in whom dapsone hypersensitivity syndrome dhs developed 5 weeks after initiation of dapsone therapy. It is known that dapsone hypersensitivity syndrome dhs complicates the treatment in a proportion of patients. Drug reaction with eosinophilia and systemic symptoms dress, also termed druginduced hypersensitivity syndrome dihs, is a rare reaction to certain medications. She had fever, gross hematuria, and malaise for three weeks after initiation of dapsone therapy. She had fever and cervical lymphadenopathy, and later a widespread erythematous eruption studded with pustules developed. The patient should be warned to respond to the presence of clinical signs such as sore throat, fever, pallor, purpura or jaundice. 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 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. Hla screening for dapsone hypersensitivity in indiaa long. A rare case of dapsone hypersensitivity syndrome and. Drug reaction with eosinophilia and systemic symptoms wikipedia. Dapsone hypersensitivity syndrome ronald prussick, md, frcpc, and nell h.
Mar 01, 2019 hypersensitivity to dapsone andor its derivatives. Dapsone syndrome first malaysian paediatric case report. 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 is one of the commonly implicated drugs in drug induced systemic hypersensitivity syndrome, apart from anticonvulsants, sulfonamides, allopurinol, nonsteroidal antiinflammatory drugs and minocycline. Dhs is characterized by a hypersensitivity response to the drug, dapsone which is a sulfone. Dapsone hypersensitivity syndrome in a leprosy patient mahe. 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. Studies in the rat revealed that dapsone dependent methaemoglobinaemia could be greatly diminished. Dapsone syndrome, drug hypersensitivity reaction, drug reaction with eosinophilia and systemic 3symptoms dress, leprosy introduction dapsone syndrome is a potentially fatal hypersensitivity. 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. 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. Case report severe dapsone hypersensitivity syndrome.
Dapsone hypersensitivity syndrome that occurred during. Deaths associated with the administration of dapsone have been reported from agranulocytosis, aplastic anemia and other blood dyscrasias. Dapsone hypersensitivity syndrome dhs is a rare, but potentially lifethreatening reaction to dapsone. Skin biopsy showing perivascular lymphocytic infiltrates with. Here we report a case of severe, life threatening dapsone systemic hypersensitivity syndrome in a 17yearold male who.
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. Generation and characterization of dapsone and nitroso. This syndrome is an idiosyncratic drug reaction which occurs in. Pathophysiologic mechanisms, diagnosis, and management of. Dapsone hypersensitivity syndrome dhs is one of the most serious adverse reaction of dapsone. Medical college, perinthalmanna, kerala, india 2 specialist, geriatrics, hamad medical corporation, doha, qatar. 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. Dapsone dds is an antibiotic associated with hypersensitivity reactions in 0. Dapsone is a sulfone derived drug used in the treatment of leprosy and several chronic inflammatory dermatological diseases.
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 may induce a doserelated hemolytic anemia, which is more likely with doses greater than 200 mgday or in patients with glucose6phosphate. Amino acid variants of hladrb1 confer susceptibility to. The pathophysiologic mechanisms, diagnosis, and management of. 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.
In the literature, the effect of dapsone on the heart has only been described twice. 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. Presumed dapsoneinduced drug hypersensitivity syndrome. Dapsone is used in the treatment of infections and inflammatory diseases. Dapsone hypersensitivity syndrome is a rare yet severe adverse drug reaction caused by dapsone, a principal drug in multidrug therapy for leprosy. Our patient presented with fever, skin eruption, jaundice, and anaemia. Dapsone is a component of multidrug therapy mdt for the treatment of all types of leprosy. Dapsone hypersensitivity syndrome dhs is characterized by fever, hepatitis, generalized exfoliative dermatitis and lymphadenopathy. Case report dapsone hypersensitivity syndrome not related. The dapsone hypersensitivity syndrome, which is associated with a reported mortality of 9.
These have included methemoglobinemia, aplastic anemia, leukopenia, agranulocytosis, eosinophilia, macrocytic anemia, and heinz bodies. Herein, we report a case of an 8yearold girl with dapsone hypersensitivity syndrome dhs that occurred during the treatment of erythema elevatum diutinum. Increase in the incidence of dapsone hypersensitivity syndrome an appraisal p. Herein, we describe the practical difficulties in translating this screening into clinical practice in india. Dapsone hypersensitivity syndrome is a rare but wellrecognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepatosplenomegaly and oral.
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. Here we are reporting a rare case so called dapsone syndrome or dhs dapsone hypersensitivity syndrome, which include skin eruption rash, fever and internal organ involvement like hepatobiliary, pulmonary, hematological and neurological and others. However, the use of dapsone may be associated with a plethora of adverse effects, some of which may involve. Dapsone hypersensitivity syndrome is a rare but well recognized serious adverse effect characterized by fever, skin rashes, generalized lymphadenopathy, hepatitis, and hepato. 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. We performed a retrospective study of patients commenced on mdt between 1990 and 2006. 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 in a leprosy patient. Dapsone fda prescribing information, side effects and uses. Dapsone should then be continued for an additional 10 years for borderline patients and for life for lepromatous patients. Dapsone induced cholangitis as a part of dapsone syndrome. Although clinical diagnosis is indicative of a drug hypersensitivity reaction, studies have not been performed to define whether dapsone or a metabolite activates specific tcells. Dapsone hypersensitivity syndrome not related to g6pd. 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.
The pathogenesis of the dapsone syndrome is as yet unknown. 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. Increase in the incidence of dapsone hypersensitivity. Background this clinical case illustrates the challenges of the potentially lethal dapsone hypersensitivity reaction. 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. Pdf dapsone induced hypersensitivity syndrome top journal. Lakshmi department of dermatology, osmania medical collegehospital hyderabad.
The authors report a case of dapsoneinduced methemoglobinemia observed in the emergency department during routine workup for contact dermatitis in a patient with celiac disease. Dapsoneinduced hypersensitivity syndrome, hemolytic anemia, and severe agranulocytosis jemshad alungal 1, mansoor c abdulla 1, noorudheen kaladi kunnummal 2, amrutha sivadasan 1 1 department of general medicine, m. Dhs is characterized by exfoliative dermatitis, fever, internal organ. Dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. 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. Dapsone, a potent antiparasitic and antiinflammatory compound, is mainly used in the treatment of leprosy and a variety of blistering skin diseases. The need for a more reliable route of administration led to investigation the possibility of an i. 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. Thus, the aims of this study were to i explore the immunogenicity dds and nitroso dds ddsno using pbmc from. Table table1 1 lists the multiple adverse effects of dapsone, including the dapsone hypersensitivity syndrome dhs and dress syndrome. Dapsone avlosulfon aids information, education, action. Dapsonetrimethoprim for pneumocystis carinii pneumonia in.
Case report dapsone hypersensitivity syndrome not related to. It may cause a severe adverse drug reaction with multiorgan involvement known as dapsone hypersensitivity syndrome. Sep 05, 2011 the need for a more reliable route of administration led to investigation the possibility of an i. Dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome dhs, which is potentially fatal.
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