A Case of Fatal DRESS Syndrome Caused by Dapsone in a Patient with Behcet's Syndrome

Chang Hoon Lee; Sung Jo Jang; Seong Rheol Oh; Hyun Jung Kim; Myeung Su Lee

doi:10.4078/jkra.2009.16.3.253

Journal List > J Korean Rheum Assoc > v.16(3) > 1003681

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Lee, Jang, Oh, Kim, and Lee: A Case of Fatal DRESS Syndrome Caused by Dapsone in a Patient with Behcet's Syndrome

Original Article

J Korean Rheum Assoc 2009;16(3):253-257.

Published online: 20 September 2009

DOI: https://doi.org/10.4078/jkra.2009.16.3.253

A Case of Fatal DRESS Syndrome Caused by Dapsone in a Patient with Behcet's Syndrome

Chang Hoon Lee¹, Sung Jo Jang³, Seong Rheol Oh², Hyun Jung Kim², Myeung Su Lee²

¹Department of Internal Medicine, College of Medicine, The Catholic University of Seoul, Seoul, Korea

²Department of Internal Medicine, College of Medicine, Wonkwang University Iksan, Korea

³Department of Neurosurgery, College of Medicine, Wonkwang University Iksan, Korea

Received 30 May 2009 Revised 16 July 2009 Accepted 20 July 2009

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Dapsone has been used for several dermatological conditions such as immunobullous disease and ulticarial vasculitis. Dapsone is very useful drug for treating the mucocutaneous manifestations of Behcet's disease. The widely recognized side effects of dapsone are headache, methemoglobinemia and hemolysis. The severer, but rarer side effect of dapsone is known as dapsone hypersensitivity syndrome, which consists of exfoliative dermatitis, hepatitis, lymphadenopathy and hemolytic anemia. When this is associated with eosinophilia, we can diagnose and treat this drug reaction with eosinophilia and systemic symptoms (DRESS). DRESS is a syndrome of fever, rash, and internal organ involvement that's secondary to administering the offending medication. We describe here a 47-year-old woman who was hospitalized with fever, skin rash, hemolytic anemia, lymphadenopathy, eosinophilia, pleural effusion and life threatening hepatitis, which could lead to hepatic failure, within three weeks of starting of dapsone therapy for controlling her oral and genital ulcers. We diagnosed the woman as suffering with DRESS syndrome and we started treatment with corticosteroid. Three weeks after starting therapy, her symptoms almost completely resolved and she was discharged.

Keywords: DRESS, Drug hypersensitivity syndrome, Dapsone, Behcet disease

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Fig. 1.

A morbilliform erythematous skin eruption involving the back of the patient.

Fig. 2.

Chest X-ray showing the right side pleural effusion at 3 days after admission.

Fig. 3.

CT imaging of the liver and spleen shows inhomogeneous enhancement of the liver parenchyma, hepatosplenomegaly, a perihepatic fluid collection and thickening of the gall bladder wall, indicating diffuse liver disease.

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