Stevens Johnson Syndrome: Toxic Epidermal Necrolysis Overlap Due to Pantoprazole: Report of a Fatality

Abstract

Proton pump inhibitors (PPIs) are widely prescribed and generally well-tolerated. Severe cutaneous hypersensitivity reactions such Stevens Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but potentially life threatening, complications of PPI use. Omeprazole, esomeprazole, lansoprazole, and pantoprazole have all been implicated in a small number of cases of SJS or TEN. Here we present the first documented fatality related to severe desquamating hypersensitivity reaction to pantoprazole. A 74-year-old woman with chronic obstructive pulmonary disease, peripheral vascular disease, and hypertension presented to the emergency department with a painful skin eruption. One month prior, an admission for gallstone pancreatitis was complicated by melena. Endoscopy showed severe erosive distal esophagitis and several small duodenal ulcers. She was discharged after laparoscopic cholecystectomy on sucralfate for 2 weeks and pantoprazole 40mg PO twice daily. Her home medications, unchanged for more than a year, were continued: aspirin 81mg daily, vitamin D3 2000U daily, donepezil 10mg daily, hydrochlorothiazide 25mg daily, lisinopril 5mg daily, and metoprolol tartrate 25mg twice daily. On admission to the burn unit she was found to have a generalized morbilliform eruption over her face, trunk, bilateral upper and lower extremities, and labia majora, with erythematous flaccid vesicles and bullae and multiple open areas of desquamation with positive Nikolsky sign (Image 1). There was involvement of the oral mucosa and conjunctiva. Sloughing progressed until hospital day 8, when TBSA was estimated at 30% (Image 2). Testing for Mycoplasma pneumoniae was negative, liver enzymes, renal function, and complete blood count were normal and there were no fevers or other symptoms on presentation to suggest viral infection. There were no other potential offending medications in the month prior to presentation. Skin biopsies showed full thickness epidermis with partial necrosis, consistent with SJS. On hospital day 9 the patient developed severe sepsis of unknown source and passed away the following day. PPIs are commonly used medications, and intolerance remains rare. However, here we describe a case of an elderly woman appropriately prescribed pantoprazole for gastrointestinal hemorrhage who experienced a fatal hypersensitivity reaction. All prescribers of PPIs should be aware of this rare but serious potential reaction.Figure: Initial presentation. Intact and ruptured bullae on right posterolateral thigh.Figure: Hospital day 8. Right flank, buttock, and thigh.