Lupus & antiphospholipid syndrome

Clinical, pathological and immunological features of toxic-epidermal necrolysis like lupus erythematosus: A systematic review and proposal for diagnostic criteria

LupusPublished Systematic review of case series and case reports

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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