Track: Case Reports

Topic: Inflammatory Diseases

Abstract:

Steven’s Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a life-threatening mucocutaneous adverse drug reaction characterized by epidermal necrosis and detachment. The role of cytotoxic T cells in the pathogenesis of SJS/TEN is well known, and several pathways have been proposed involving presentation of antigens to T cell receptors, activation of cytotoxic effector molecules, and subsequent apoptosis of keratinocytes. The role of innate immunity in the development of SJS/TEN, however, is not well understood. Classically, the histopathology of SJS/TEN shows a sparse inflammatory infiltrate comprised mostly of lymphocytes. Recent studies, though, have proposed a mechanism by which neutrophil extracellular traps (NETs) develop in early lesional skin, initiating a cascade that leads to keratinocyte necroptosis in addition to apoptosis. Here we present a case of neutrophil-rich SJS/TEN in a critically ill patient with multidrug-resistant pyelonephritis. Shave biopsy revealed full-thickness epidermal necrosis with numerous individual and clustered neutrophils in the papillary dermis, representing an unusual presentation of SJS/TEN, likely due to a cephalosporin antibiotic. We discuss the challenges of diagnosing SJS/TEN in critically ill patients and present a differential for blistering disorders in this patient population. Furthermore, we review the literature on the role of innate immunity in the development of SJS/TEN.

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Lisa Akintilo, MD