Abstract
The case of a 38-year-old female who developed drug-induced hypersensitivity syndrome (DIHS) 38 days after starting a course of diaphenylsulfone (DDS) for pityriasis lichenoides et varioliformis acuta (PLEVA) is reported. She presented with a generalized erythematous skin rash, fever, and lymph node swelling. The DDS was discontinued immediately, and the systemic administration of steroids (1 mg/kg/day) was started. Leukocytosis, liver dysfunction, and human herpesvirus 6 reactivation were detected during the course of the patient's condition. Although the erythematous lesions remained for three weeks, the patient's other symptoms, including her high fever, leukocytosis, and liver dysfunction, were abrogated and did not relapse, and the PLEVA-induced eruptions also gradually disappeared. A drug-induced lymphocyte stimulation test of DDS produced a positive result, and a diagnosis of DIHS was made. We retrospectively examined the Japanese cases of DIHS/DDS syndrome and reports that described the relationship between DIHS and human leukocyte antigen (HLA)-B*13:01 due to DDS. This is the first report about DIHS due to DDS associated with HLA-B*13:01 from Japan.Skin Research, 15: 112-118, 2016