Lupus & antiphospholipid syndrome
Clinical, pathological and immunological features of toxic-epidermal necrolysis like lupus erythematosus: A systematic review and proposal for diagnostic criteria
Authors' conclusion
TEN-like LE can be the first manifestation of LE in up to half of cases. Photodistribution, lupus non-specific lesions, ANA positivity, or change in ANA type/titre can be important diagnostic clues. Unlike previously believed, mucosal involvement is not uncommon and can be severe.
Study context
- Population
- Patients with toxic epidermal necrolysis like lupus erythematosus (TEN-like lupus erythematosus (LE)), studies published 1977–202416 case series · 52 case reports
- Participants
- 112 patients
- Outcome
- Clinical, pathological and immunological features of TEN-like LE
Methodological detailsstructured search, proposal for diagnostic criteria, Search April–May 2024 of PubMed, Medline, Embase, SCOPUS, VHL, ProQuest, clinicaltrials.gov, COCHRANE, POPLINE, ISI, mRCT, UC library and ResearchGate
- Structured search
- Proposal for diagnostic criteria
- Search April–May 2024 of PubMed, Medline, Embase, SCOPUS, VHL, ProQuest, clinicaltrials.gov, COCHRANE, POPLINE, ISI, mRCT, UC library and ResearchGate
Pooled result
As reported in the abstract
- Mean age
- 43.39 years
- Female
- 86.6%
- TEN-like LE as presenting manifestation of LE
- 48.21%
- Antinuclear antibody (ANA) positive in all patients where reported
- 109
- SSA/Ro positive (most common)
- 60.55%
- dsDNA positive
- 50%
- Photodistributed lesions
- 78.57%
- Palmoplantar involvement
- 20.54%
- Mucosal involvement
- 56.25%
- Significant mucosal involvement
- 26.78%
- Lupus non-specific lesions
- 25%
- Paraneoplastic association
- 7
Further pooled results
Factors associated with hemodynamic instability
- Anemia, pulmonary and GI involvement positively associated; anti-Ro antibodies negatively associated
Factors associated with systemic involvement
- Positive dsDNA, La positivity and low C4 associated with increased odds; Ro positivity with reduced odds
Original publication
Clinical, pathological and immunological features of toxic-epidermal necrolysis like lupus erythematosus: A systematic review and proposal for diagnostic criteria. Lupus. Published 26 August 2025. doi:10. 1177/ 09612033251371801
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