Describe a case that highlights central nervous system (CNS) involvement in Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) that is beyond the recognized meningitis and encephalitis complications.

DRESS is a severe immune reaction which develops 2-6 weeks after exposure to a particular drug and is attributed to T cell expansion. Clinical manifestations include fever, lymphadenopathy, eosinophilia, and morbiliform eruption covering more than half of the body surface area, often accompanied by hepatic, renal, and pulmonary manifestations. Other organ involvement occurs less frequently. CNS involvement in DRESS has been described as encephalitis and meningitis. There are few case reports of vasculitis or vasculitis-like presentations in DRESS, and we would like to contribute this case to the emerging data.

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A thirty-one-year old woman with a past medical history of Crohn’s disease on Infliximab, multiple drug allergies, and refractory peripheral cutaneous vasculitis, developed a diffuse morbiliform rash and increasing fatigue three weeks after starting Dapsone. She became acutely somnolent and was hospitalized. On exam, she was encephalopathic with right upper extremity monoparesis. Laboratory studies revealed leukocytosis with eosinophilia and transaminitis. Cerebrospinal fluid studies were unremarkable. Brain MRI revealed acute multifocal infarcts in bilateral frontal, parietal, and occipital lobes that resembled a watershed distribution with scattered, patchy enhancement. Head MRA with 3D MIP reformats showed distal middle cerebral artery (MCA) and anterior cerebral artery (ACA) vessel irregularities, suggestive of vasculitis.  This case met  RegiSCAR criteria and Japanese group criteria for DRESS. Neurologic and other organ system involvement improved with high dose steroids.

CNS manifestations in DRESS syndrome may not be limited to encephalitis and meningitis, as was previously accepted. It is crucial for Neurologists to recognize CNS involvement in DRESS, particularly given that the 2018 European Medical Agencies (EMA) documents on Daclizumab included DRESS cases with CNS manifestations.