Drug‐induced hypersensitivity syndrome/drug rash with eosinophilia and systemic symptoms due to diaminodiphenylsulfone during the treatment of pemphigus foliaceus

Abstract

Dear Editor, We report a case of 57yearold Japanese male who developed druginduced hypersensitivity syndrome (DIHS), also known as drug reaction with eosinophilia and systemic symptoms (DRESS) due to diaminodiphenylsulfone (DDS, also known as dapson). The patient started DDS for pemphigus foliaceus (PF) due to flare up of the disease while taking 1.0 mg/day of betamethasone. Thirtythree days after initiation of DDS, he visited our office with a highgrade fever (>38.0°C), generalized maculopapular rush and purpura over his whole body except around the eyelids. Pityriatic scales were found around the mouth, and lymph nodes swelling was also noted (Figure 1A,B). Laboratory examination identified leukocytosis (white blood cell 17,080/μl, neutrophils 88.0%, eosinophils 0.0%), atypical lymphocyte (1.0%), and liver dysfunction (aspartate aminotransferase 93 U/L, alanine aminotransferase 289 U/L). Elevation of thymus and activationregulated chemokine was significant (12,200 pg/ ml). A skin biopsy demonstrated exocytosis, liquefaction degeneration, spongiosis, and lymphocytic infiltration to the upper dermis (Figure 1C). He filled the six points of diagnostic criteria for DIHS and fulfilled that for DRESS and was diagnosed as DIHS/DRESS. The DDS was discontinued, and the oral prednisolone (0.6 mg/kg/ day) was started. The erythematous lesion and liver dysfunction relapsed and were prolonged; thus, intravenous immunoglobulin therapy (400 mg/kg/day for 5 days) was added and he recovered. Reactivation of human herpes virus6 (HHV6) was confirmed by the elevation of serum IgG titers to HHV6 (10 to 640) in 4 weeks after onset. Lastly, genetic examination revealed that our patient held human leukocyte antigen (HLA)B*13:01. DDS is an antibiotic against Mycobacterium leprae, a causative agent of leprosy. DDS is applied in a range of dermatological fields because of its antiinflammatory and immunomodulatory effect. DIHS/DRESS is a rare yet serious adverse drug reaction involving multiple organs.1 Recently, several HLA alleles have been associated with sever drug eruptions.2 HLAB*13:01 was first described as a marker of susceptibility to DDSinduced DIHS/DRESS among