P3.T384: More Than Skin Deep: A case of DRESS Syndrome Highlighting the Importance of Rash Evaluation in Dark Skinned Individuals

Introduction: Drug reaction with eosinophilia with systemic symptoms (DRESS) syndrome is a rare drug reaction that typically involves eosinophilia, atypical lymphocytes, erythematous maculopapular skin rash and multi-organ involvement. Here we describe a case of DRESS syndrome in a black child whose diagnosis was confounded by difficulty characterizing his rash.

Case Description: A five-year-old Black male with history of autism spectrum disorder and subdural empyema complicated by meningoencephalitis and focal seizures presented to the emergency department (ED) nine days after discharge from the inpatient ward with low grade fevers, a pruritic rash described by parents but difficult to appreciate on exam, and diarrhea. He had been on phenobarbital for 20 days, levetiracetam for 26 days and ceftriaxone for 24 days. He was diagnosed with a viral syndrome with exanthem and discharged home. DRESS syndrome was considered due to elevated CRP of 2.11mg/dL and eosinophilia 7%; however, he did not meet criteria suggestive of DRESS (RegiSCAR score 1). Subsequently he was seen clinic for similar concerns and had a repeat presentation to the ED for persistent rash, his parents’ biggest concern, and intermittent fevers. Because he had a peripherally inserted central catheter for parenteral antibiotics, he was admitted to the pediatric ward for a central-line associated bloodstream infection rule out. Despite broadening antibiotic coverage and an extensive infection work up, his fevers persisted. His pruritic diffuse maculopapular rash progressed to the lower extremities, face, back, and upper extremities, dermatology was consulted and agreed on the difficulty identifying his rash etiology (Figure 1). He was found to have persistent elevation of AST/ALT, GGT, ferritin and atypical lymphocytes. This together with his skin findings led to the diagnosis of DRESS on hospital day 4, 21 days after the first ER visit for fever and rash. He was started on high dose steroids resulting in resolution of his fevers, improvement in his rash, and down trending inflammatory markers. His course was complicated by liver and kidney failure, and posterior reversible encephalopathy syndrome. He required long term high dose steroids and immunomodulatory agents for management of the effects of DRESS syndrome.

Discussion: DRESS syndrome is difficult to diagnose in the pediatric population due to a wide variation of presenting symptoms and systemic involvement. Diagnosis was delayed in our patient partly due to difficulty appreciating the extent of his rash. This case highlights the need for vigilant evaluation of rashes in our pediatric patients with darker skin colors and increased education of medical practitioners on the presentation and evaluation of rashes in dark skinned individuals.

Conclusion: This presentation of DRESS in a dark-skinned child, accompanied by high-quality photographs of the rash manifestation, may serve as a valuable resource for future diagnoses and education.