|Year : 2018 | Volume
| Issue : 1 | Page : 67-69
A case of Hansen's disease presenting with sulfone syndrome and hemolytic anemia
Preema Sinha1, Pankaj Das1, Nikita Sharma1, Saikat Bhattacharjee2
1 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Radiodiagnosis, Military Hospital (CTC), Pune, Maharashtra, India
|Date of Web Publication||9-Jul-2018|
Lt Col Preema Sinha
Department of Dermatology, Armed Forces Medical College, Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
Dapsone (4,4'-diaminodiphenylsulfone) is a drug commonly used to treat various infectious, immunological, granulomatous, and hypersensitivity disorders. It can cause varied adverse effects, including sulfone syndrome which can have a potentially fatal outcome. We report a case of a female diagnosed as a case of Hansen's disease with Type II lepra reaction, who developed sulfone syndrome and hemolytic anemia as adverse drug effects to dapsone. The case is presented to stress on the importance of timely diagnosis and institution of early treatment in the management of a rare complications of a commonly used drug with a favorable outcome.
Keywords: Hansen's disease, hemolytic anemia, sulfone syndrome
|How to cite this article:|
Sinha P, Das P, Sharma N, Bhattacharjee S. A case of Hansen's disease presenting with sulfone syndrome and hemolytic anemia. J Mar Med Soc 2018;20:67-9
| Introduction|| |
Common side effects of dapsone include idiosyncratic reactions such as skin hypersensitivity as well as dose-dependent side effects such as hemolytic anemia and methemoglobinemia. Severe cutaneous adverse reactions to dapsone include the drug hypersensitivity syndrome (DHS) also known as the sulfone syndrome and toxic epidermal necrolysis., Sulfone syndrome is characterized by fever, cutaneous rash, lymphadenopathy, eosinophilia, hepatic, pulmonary, and other systemic features. It can sometimes become fatal by causing irreversible organ damage. We herein report a case of severe sulfone syndrome with hemolytic anemia.
| Case Report|| |
A 45-year-old female recently diagnosed as a case of Hansen's disease (leprosy) with recurrent Type II lepra reaction on three drug multidrug therapy (MDT) and Thalidomide presented after 2 weeks of starting the MDT with a history of generalized reddish rash over face, trunk, and all four extremities along with intermittent high-grade fever with chills, myalgia, and malaise. No history of cough, chest pain, pain abdomen, vomiting, or altered sensorium was present.
On examination, she was febrile (101°F), had tachycardia (112 beats/min), and had pallor. She had facial edema and bilateral pitting edema over the legs up to mid-calf level and both arms up to the elbows. Both elbow and knee joints were tender on touching. No significant lymphadenopathy was present. Dermatological examination revealed generalized erythematous maculopapular rash with scaling at places involving face, trunk, and all four limbs [Figure 1] and [Figure 2]. Bilateral symmetrically distributed partially defined erythematous infiltrated, normoaesthetic, plaques over trunk, thighs, and buttocks was present. Glove - and - stocking type of anesthesia was present. No significant peripheral nerve thickening was present.
|Figure 1: Erythematous maculopapular rash with scaling at places involving the face|
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|Figure 2: Generalized erythematous maculopapular rash with scaling at places involving the trunk|
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Relevant hematological and biochemical evaluation revealed anemia with hemoglobin of 6 g/dL with features of hemolysis on peripheral blood smear and a reticulocyte count of 12.5%. Total leukocyte count was 13,300/cmm and liver function tests were deranged with indirect hyperbilirubinemia and raised liver enzymes and hypoalbuminemia. Direct and indirect Coombs test were negative. Her glucose-6-phosphate dehydrogenase (G6PD) level was normal. Serology for human immunodeficiency virus, hepatitis C virus, Hepatitis B surface antigen, Widal, Dengue, and Leptospira was negative. Skin biopsy from one of the infiltrated lesions revealed ill-defined granulomas with Fite-Faraco stain positive for acid-fast bacilli [Figure 3].
The patient was diagnosed as a case of lepromatous leprosy in Type II lepra reaction with Dapsone hypersensitivity syndrome with hemolytic anemia. Tablet Dapsone was stopped immediately. The patient was managed with transfusion of 2 units of packed red blood cells, oral corticosteroids, second-line MDT for leprosy, and Tablet Thalidomide with excellent response. There were no fresh crops of skin lesions and the patient became afebrile after 2 days with exfoliation of rash after 5 days. She was discharged on day 10 after her hemoglobin was 8.4 gm% and liver enzymes became normal. At present, the patient is on regular follow-up.
| Discussion|| |
Dapsone has both antimicrobial and antiprotozoal properties and anti-inflammatory effects resembling those of nonsteroidal anti-inflammatory drugs; hence, due to this dual action, it is used in many infectious, immunological, and hypersensitivity disorders., Dapsone is known to cause drug-induced hypersensitivity syndrome or the sulfone syndrome as named by Lowe and Smith in 1949. DHS is an idiosyncratic reaction which generally develops within 3 weeks to as late as 6 months after starting dapsone, with a reported incidence of 0.5%–3%. Etiopathogenesis includes complex interplay between the drug intake in a genetically predisposed individual, the individual's immunity, and sequential reactivation of viruses such as HHV-6, HHV-7, Epstein–Barr virus, and cytomegalovirus., Differences in dapsone metabolism, which affect the production and detoxification of its reactive metabolites, might be responsible for differential susceptibility of people to the adverse effects of dapsone. Testing for HLA-B*13:01 single-nucleotide polymorphism may be conducted in high-risk population to predict the possibility of DHS. Presenting complaints are generally high-grade fever associated with maculopapular rash involving the face, upper trunk, and upper extremities, later spreading to the lower half of the body. Atypical presentations include overlap of DHS with either toxic epidermal necrolysis or acute generalized exanthematous pustulosis. Hepatic involvement may be seen in the form of transaminitis, hepatomegaly, hepatobiliary dysfunction, cholangitis, and sometimes fulminant liver failure. The lungs may be affected in the form of interstitial pneumonitis, diffuse alveolar damage, or pleural effusion. The hematological system may also be frequently involved in the form of hemolytic anemia, thrombocytopenia, lymphopenia, lymphocytosis, eosinophilia, and leukocytosis with atypical lymphocytes., Dapsone-induced hemolytic anemia has been generally reported in patients with G6PD deficiency. Occurrence of hemolysis in patients with normal G6PD levels may be a dose-dependent event with elevated dapsone levels in patients with renal dysfunction or due to concomitant use of drugs utilizing the cytochrome P-450 enzyme system., Interstitial nephritis, myocarditis, pericarditis, necrotizing vasculitis, encephalitis or meningitis, thyroiditis, and colitis can be other complications. The diagnosis of sulfone syndrome is based on clinical features, fever, lymphadenopathy, characteristic skin rash, and other systemic features along with a history of dapsone intake. Histopathology is not specific for DHS. The Naranjo causality score in our patient was 5.
The management of DHS involves immediate discontinuation of dapsone, systemic corticosteroids with other supportive care.
This case is hence being reported to stress on rare side effects of dapsone such as sulfone syndrome and hemolytic anemia which can be fatal if not promptly diagnosed and treated.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]