Vol.67 No.6 November 2019
Diagnosis and management of drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DiHS/DRESS)
Department of Dermatology, Kyorin University School of Medicine, 6-20-2 Shinkawa, Mitaka, Tokyo, Japan
Abstract
Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DiHS/DRESS) is a life-threatening multiorgan system reaction caused by a limited number of drugs, including carbamazepine, phenytoin, phenobarbital, zonisamide, allopurinol, lamotrigine, salazosulfapyridine, and mexiletine. However, this syndrome has several unique clinical features that cannot be solely explained by drug allergy. DiHS/DRESS is characterized by a paradoxical deterioration of clinical symptoms, frequent flare-ups and a stepwise development of several organs system failures multiorgan failure after withdrawal of the causative drug. We demonstrated that several herpes viruses, especially human herpesvirus 6 (HHV-6), could be reactivated during this syndrome in a sequential order, as demonstrated in other settings of the immune reconstitution inflammatory syndrome (IRIS). Given the observation that paradoxical worsening of the clinical symptoms is typically observed after withdrawal of the causative drugs with immunosuppressive properties at the onset of DiHS/DRESS, it is attractive to suppose that DiHS/DRESS is a manifestation of non-HIV IRIS. Various infections have been noted in corticosteroid-treated patients with DiHS/DRESS, including herpes simplex, herpes zoster, Pneumocystis jirovecii pneumonia, and cytomegalovirus diseases. Of note, most infectious diseases appeared within 1 month, and coincided with a tapering of the corticosteroid dose. This paper presents clinical features of DiHS/DRESS, and a management-based approach to the diagnosis and treatment.
Key word
drug-induced hypersensitivity syndrome, drug reaction with eosinophilia and systemic symptoms, immune reconstitution inflammatory syndrome
Received
October 29, 2018
Accepted
June 25, 2019
Jpn. J. Chemother. 67 (6): 620-627, 2019
