TABLE 2

Diagnostic Features of the Differential for Epidermal Necrosis in SLE

TENSJS/TEN-like Cutaneous LEBullous LERowell Syndrome
ClinicalFlu-like prodrome; Dusky macules that coalesce; Bullae and sloughing of epidermisMay have preceding diagnosis of lupus; Bullae and sloughing of epidermisTense vesicles and bullae on sun-exposed areasFeatures of DLE; Targetoid macules with centripetal spreading
Nikolsky signPositivePositive or negativeNegativeNegative
Mucous membrane involvementSevereLess severe and predominantly oral mucosaSometimesSometimes
Serology (ANA, anti-dsDNA, anti-Ro/La, RF)NegativeANA positive; Anti-dsDNA positive (often); Anti-Ro/SSA or La/SSB positive (often); RF negativeANA positive; RF negativeANA positive (speckled); Anti- Ro/SSA or La/SSB positive; RF positive
HistopathologyFull thickness epidermal necrosis with sparse superficial lymphocytic inflammatory infiltrateFull thickness epidermal necrosis with sparse superficial lymphocytic inflammatory infiltrate; Features of interface dermatitisVacuolar degeneration of basal layer resulting in subepidermal blister with predominantly neutrophilic infiltrateNecrotic keratinocytes with subepidermal blistering and a sparse lymphocytic infiltrate consistent with erythema multiforme
IFNegativeDirect: May show granular IgM, IgG, and/or C3 binding at the BMZ (lupus band); Indirect: NegativeDirect: Linear or granular IgG, C3, IgM, and IgA binding at BMZ; Indirect: Collagen VII antibodies binding on the dermal side of the BMZNegative
Drug etiologyMost casesNoSome casesNo
CourseEvolves over 3–5 d and heals over 3–4 wk. Scarring may occur. Mortality up to 30%–40%.Subacute course with rapid improvement within 1–2 wk after treatmentRapid response to dapsone treatment with cessation of blister formation within 1–2 d. May have intermittent exacerbationsMay recur over many years
  • BMZ, basement membrane zone; IF, immunofluorescence; RF, rheumatoid factor. Adapted from Ryan et al4 and Mandelcorn et al.1 Altered with permission from Ryan E, Marshman G, Astill D. Toxic epidermal necrolysis-like subacute cutaneous lupus erythematosus. Australas J Dermatol. 2012;53(4):305.