A Rare Case of Fatal Toxic Epidermal Necrolysis (TEN) and Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome in a Patient with Rheumatoid Arthritis.

Abstract

Introduction: This case study presents a rare and fatal instance of Toxic Epidermal Necrolysis (TEN) and Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome in a 51-year-old male patient diagnosed with Rheumatoid Arthritis (RA).

Case presentation: The patient was initially treated with sulfasalazine, leflunomide, and hydroxychloroquine, following which he developed a rash, fever, and loose stools. Drug allergy was suspected, and the antirheumatic medications were withdrawn, following which, the patient improved. A subsequent attempt was made to treat the RA with methotrexate, prednisolone, and hydroxychloroquine, following which the rash returned along with an increase in severity, including detachment of the epidermis and mucosa, and systemic involvement, both hepatic and renal. The patient ultimately succumbed to multiple organ dysfunction syndrome and neutropenic sepsis.

Conclusion: This case highlights the possibility of DRESS syndrome and Stevens-Johnson Syndrome (SJS)/TEN following treatment with anti-rheumatic medications. Evidence of this is rare, with the exception of sulfasalazine. This case also considers that the signs of a moderately severe adverse drug reaction could be the early warning signs of DRESS syndrome, which can be difficult to manage and may turn to be fatal. Additionally, this case highlights the need for maintenance of quality health records in low- and middle-income countries due to the failure to identify hydroxychloroquine as a suspected drug inducing the initial adverse reaction that resulted in it being prescribed again, leading to a fatal outcome.