Photodistributed Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Systematic Review and Proposal for a New Diagnostic Classification

Background: Ultraviolet radiation (UVR) exposure is commonly reported as a risk factor for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, minimal evaluation of photo-induced SJS/TEN has been conducted. Thus, this review identifies all cases of SJS/TEN that are linked to an acute exposure of UVR and outline the unifying characteristics of these cases. Furthermore, the theoretical pathogenesis, differential diagnoses, and proposed diagnostic criteria are defined. Methods: PubMed, Google Scholar, and other databases and websites were searched from inception to September 2021 to identify studies that met inclusion criteria. The following keywords were utilized: “Stevens-Johnson syndrome” and “toxic epidermal necrolysis” with “ultraviolet,” “photodistributed,” “photo-induced,” “photosensitivity,” and “photo.” One reviewer assessed study characteristics, with confirmation by a second. The risk of bias was assessed independently by another. Results: Thirteen patient cases were identified, all reporting ultraviolet radiation prior to rash onset and an underlying causal drug. Case classifications included 7/13 SJS and 6/13 TEN. All cases described the rash as photodistributed with UVR exposure prior to rash onset (delay of 1-3 days) and a causal drug. 10 cases provided evidence that the photodistributed rash distribution lacked linear demarcation (as in a sunburn) with satellite target-like lesions. No cases described a flu-like prodrome. Discussion: Mucositis, palmar and plantar rash, a positive Nikolsky sign, and a prolonged disease course can help distinguish from photosensitive reactions, while a negative direct immunofluorescence test is important to distinguish from other photo-induced disorders. Conclusion: Physicians should be aware that UVR may precipitate SJS/TEN in patients taking susceptible drugs. After a 24-hour delay from UVR exposure, a non-distinct, photodistributed rash appears with no flu-like prodrome and progresses for at least 48 hours to include vesiculobullous eruptions and mucous membrane involvement. Photodistributed SJS/TEN appears to be photo-drug-induced with a unique onset and rash presentation that should be recognized as a distinct diagnosis.