Photodistributed Stevens–Johnson syndrome and toxic epidermal necrolysis: a systematic review and proposal for a new diagnostic classification

Table 1 Case specific details for SJS/TEN-like LE rule-out

Reference numbers	Suggestive SJS/TEN-like LE rule-out	Definitive SJS/TEN-like LE rule-out
[28]	N/A	N/A
[22]	N/A	Negative direct immunofluorescence for immunoglobulins and complement
[29]	Normal complement concentrations (C3, C4)	Negative antinuclear antibody
[30]	Normal complement concentrations (C3, C4) Previously diagnoses of seronegative symmetrical polyarthritis	N/A
[31]	N/A	N/A
[32]	Diagnosed with Sjogren’s syndrome 7 years prior, confirmed with serology and a labial biopsy No history of sensitivity to sunlight	Negative direct immunofluorescence for immunoglobulins and complement Negative antibodies to double-stranded DNA. (Positive antinuclear antigen at a dilution of 1: 4000 with speckled pattern and positive anti-Ro and anti-La antibodies.)
[33]	Multi-specialty workup: included dermatology, infectious disease, and plastic surgery	N/A
[34]	Multi-specialty workup: Evaluated and treated in burn unit and dermatology, ophthalmology and gynecology services were consulted	N/A
[35]	N/A	N/A
[36]	N/A	Negative direct immunofluorescence Negative antinuclear antibodies
[37]	N/A	N/A
[38]	N/A	Negative direct immunofluorescence
[39]	N/A	Negative antinuclear antibody Negative anti-Smith antibody Negative anti-DNA antibody

Authors interpretation of evidence for ruling out SJS/TEN-like LE is as follows: a negative direct immunofluorescence is observed in photodistributed SJS/TEN, whereas it is positive in SJS/TEN-like LE. A negative antinuclear antibody has a strong negative predictive value for ruling-out LE. If ANA is positive but anti-Smith Antibody or double-stranded DNA Antibody is negative, LE is unlikely. In this study, cases that report a negative direct immunofluorescence or a negative lupus specific autoimmune workup are considered definitive rule out of SJS/TEN-like LE. Additional suggestive findings: normal serum C3 and C4 suggests absence of SLE, active SLE often results in decreased compliment proteins; multispecialty work-up, suggesting that further workup was performed but not reported; and previous rheumatological diagnosis, suggesting that SLE would have been ruled out at the time of previous diagnosis
SJS Steven’s-Johnson Syndrome, TEN toxic epidermal necrolysis, LE lupus erythematosus, N/A not available

ISSN: 2047-783X