Paraneoplastic pemphigus mimicking toxic epidermal necrolysis: An underdiagnosed entity?

Paraneoplastic pemphigus (PNP) is an autoimmune blistering syndrome with 5 well-described clinicopathologic phenotypes. Nguyen et al categorized these subtypes as pemphigus-like, pemphigoid-like, erythema multiforme-like, graft-vs-host-disease–like, and lichen planus–like.1 However, there is increasing recognition of PNP simulating Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). Herein, we propose SJS/TEN-like PNP as a distinct subtype of PNP. We present 2 new cases of SJS/TEN-like PNP and review the previously reported cases of this subtype.