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Year : 2018  |  Volume : 20  |  Issue : 1  |  Page : 67-69

A case of Hansen's disease presenting with sulfone syndrome and hemolytic anemia

1 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Radiodiagnosis, Military Hospital (CTC), Pune, Maharashtra, India

Date of Web Publication9-Jul-2018

Correspondence Address:
Lt Col Preema Sinha
Department of Dermatology, Armed Forces Medical College, Pune - 411 040, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmms.jmms_7_18

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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

How to cite this URL:
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 [serial online] 2018 [cited 2022 Jan 22];20:67-9. Available from: https://www.marinemedicalsociety.in/text.asp?2018/20/1/67/236262

  Introduction Top

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.[1],[2] Sulfone syndrome is characterized by fever, cutaneous rash, lymphadenopathy, eosinophilia, hepatic, pulmonary, and other systemic features.[3] It can sometimes become fatal by causing irreversible organ damage. We herein report a case of severe sulfone syndrome with hemolytic anemia.

  Case Report Top

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].
Figure 3: Ill-defined granulomas and grenz zone seen (H and E, ×200)

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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 Top

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.[4],[5] Dapsone is known to cause drug-induced hypersensitivity syndrome or the sulfone syndrome as named by Lowe and Smith in 1949.[6] 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%.[7] 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.[8],[9] 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.[9] 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.[10] 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.[5],[10] 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.[4],[8] Interstitial nephritis, myocarditis, pericarditis, necrotizing vasculitis, encephalitis or meningitis, thyroiditis, and colitis can be other complications.[10] 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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Tomecki KJ, Catalano CJ. Dapsone hypersensitivity. The sulfone syndrome revisited. Arch Dermatol 1981;117:38-9.  Back to cited text no. 1
Pandey B, Shrestha K, Lewis J, Hawksworth RA, Walker SL. Mortality due to dapsone hypersensitivity syndrome complicating multi-drug therapy for leprosy in Nepal. Trop Doct 2007;37:162-3.  Back to cited text no. 2
Zhu YI, Stiller MJ. Dapsone and sulfones in dermatology: Overview and update. J Am Acad Dermatol 2001;45:420-34.  Back to cited text no. 3
Kosseifi SG, Guha B, Nassour DN, Chi DS, Krishnaswamy G. The dapsone hypersensitivity syndrome revisited: A potentially fatal multisystem disorder with prominent hepatopulmonary manifestations. J Occup Med Toxicol 2006;1:9.  Back to cited text no. 4
Lakshmi R, Liniya S, Vijayalakshmi S. Dapsone induced hypersensitivity syndrome – A case report. Int J Pharm Pharm Sci 2015;7:585-7.  Back to cited text no. 5
Lowe J, Smith M. The chemotherapy of leprosy in Nigeria; with an appendix on glandular fever and exfoliative dermatitis precipitated by sulfones. Int J Lepr 1949;17:181-95.  Back to cited text no. 6
Rao PN, Lakshmi TS. Increase in the incidence of dapsone hypersensitivity syndrome – An appraisal. Lepr Rev 2001;72:57-62.  Back to cited text no. 7
Criado PR, Avancini J, Santi CG, Medrado AT, Rodrigues CE, de Carvalho JF, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): A complex interaction of drugs, viruses and the immune system. Isr Med Assoc J 2012;14:577-82.  Back to cited text no. 8
Wang N, Parimi L, Liu H, Zhang F. A review on dapsone hypersensitivity syndrome among Chinese patients with an emphasis on preventing adverse drug reactions with genetic testing. Am J Trop Med Hyg 2017;96:1014-8.  Back to cited text no. 9
Kumari R, Timshina DK, Thappa DM. Drug hypersensitivity syndrome. Indian J Dermatol Venereol Leprol 2011;77:7-15.  Back to cited text no. 10
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