TEN-like lupus may present at any age and in any race / ethnicity, but the majority of reported cases have been in White females with an average age of 43 years (range 11-87 years). Given that the prevalence of lupus in Black individuals is up to 4 times greater than in White individuals, it is possible that the condition is underreported in Black patients. TEN-like lupus is often triggered by medications (in around two-thirds of patients in one study), most commonly antibiotics and NSAIDs. Other triggers include ultraviolet light, lupus flares, and lupus drug holidays. Patients may have other autoimmune diseases, including antiphospholipid syndrome, Sjögren syndrome, connective tissue disease, and systemic sclerosis.
The proposed pathophysiology for TEN-like lupus is thought to be due to Fas and Fas-Fas ligand interaction upregulation. For Stevens-Johnson syndrome / toxic epidermal necrolysis (SJS/TEN), granulysin is thought to be an important cytotoxin responsible for apoptosis. Currently, it is unknown if granulysin is involved in the mechanism of TEN-like lupus.
Distinguishing TEN-like lupus from SJS/TEN is of vital importance as the two conditions have a comparable clinical appearance and there is an increased incidence of SJS/TEN in patients with SLE.
The following clinical features of TEN-like lupus are helpful in making this distinction:
- Concurrent malar or maculopapular eruptions may be seen in TEN-like acute cutaneous lupus erythematosus (ACLE), and an annular or papulosquamous rash may be seen in TEN-like subacute cutaneous lupus erythematosus (SCLE), although these features are not always present.
- TEN-like lupus tends to have a more subacute onset and slower progression than SJS/TEN, and it may display photodistribution, with the eruption starting on the face and progressing to the trunk and extremities.
- TEN-like lupus appears more likely to spread to the palms and soles than SJS/TEN.
- Although there is mucous membrane involvement, this may be less severe than in SJS/TEN, and when present, the oral mucosa is predominantly affected.
- Nikolsky sign may be positive or negative.
- Associated manifestations of lupus may be present, including fever, arthralgias, anemia, serositis, vasculitis, and alopecia, in addition to lupus nephritis or cerebritis.
- Laboratory findings that are more indicative of TEN-like lupus include positive antinuclear antibody (ANA), pancytopenia, low complement, interface dermatitis on histology, and lupus band on direct immunofluorescence (DIF).
