A Case of DRESS Syndrome in a Middle-aged Woman

Abstract

Purpose: A 51-year-old Hispanic female with history of osteoarthritis and iron deficiency anemia presented with progressively worsening epigastric pain, nausea, vomiting, loss of appetite, fatigue, and fevers for over 2 weeks. She also noticed dark urine and pale-colored stools. About a week before, she noticed a rash on her face, which spread to the trunk and extremities, sparing palms and soles, prompting her to come to ED. She denied sick contacts or recent travel history. She also denied toxic habits or herbal medication use. On exam, the temperature was 103 F. Cervical lymphadenopathy, epigastric tenderness, and generalized erythematous maculopapular non-blanching rash was presenting all over, sparing palms and soles. Labs showed mild leukocytosis with eosinophilia. Liver panel showed Ast 313, alt 352, Alk P 291, Tbil 2, T bil 1.7. The coagulation profile and platelets were normal. Her measles IgG Ab was positive. CT abdomen showed no obstruction or mass. On further questioning, the patient revealed she was taking sulfasalazine for 1 month, which she had borrowed from a friend for arthritis. A diagnosis of DRESS syndrome was made (drug reaction, eosinophilia, and systemic symptoms). She was initially started on empiric antibiotics, which were stopped once cultures came back negative, and was managed conservatively. Over the next 4 days, her enzymes worsened (Ast 1050, Alt 957, Alk P 303), along with the worsening of the rash. Her serum glucose was normal. Her MELD score was 16 (bilirubin 4.8, INR 1.36, creatinine 0.3). She was transferred to a tertiary center for evaluation for possible liver transplant. A skin biopsy was done, which showed interface dermatitis. She was started on steroids with improvement of her clinical picture, including the liver enzymes. She was discharged from the outside facility on tapering dose of steroids. Discussion: DRESS Syndrome is a rare potentially life-threatening, drug-induced hypersensitivity reaction causing rash, eosinophilia, lymphadenopathy, and multiple organ involvement (liver, kidneys and lungs). A latency period of about 2-8 weeks is seen between the drug exposure and disease onset. Treatment usually consists of removing the offending drug and supportive care. It is unclear whether systemic steroids shorten the clinical course, even in patients with acute liver injury. Liver transplant is the only effective therapy for patients with worsening liver function not responding to supportive care.