Background: Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a rare idiosyncratic and unpredictable drug reaction most commonly attributed to anticonvulsants, allopurinol, and antibiotics. Fever in combination with cutaneous manifestations and eosinophilia are the cardinal clinical findings, while organ involvement and very rarely pancytopenia can be present. Case presentation: We describe a 26-year-old female patient with pancytopenia in the context of lamotrigine-induced DRESS and we summarize this as a potentially life-threatening hypersensitivity reaction. Conclusion: DRESS should be suspected in patients presenting with fever, eosinophilia, skin involvement, and/or visceral organ involvement, who have started a new drug during the past 2 to 6 weeks, as early recognition of this syndrome with subsequent discontinuation of the offending drug could be lifesaving.

Keywords: DRESS; lamotrigine; hypersensitivity reaction; pancytopenia