A Rare Presentation of Toxic Epidermal Necrolysis – Like Acute Systemic Lupus Erythematosus

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Brandon T Beal
Taryn Blaha
David D Xiong
Sarah H Schneider
Steven D Billings
Anthony P Fernandez
Shilpi Khetarpal


toxic epidermal necrolysis (TEN), acute systemic lupus erythematosus (ASLE), systemic lupus erythematosus (SLE), immunoderamatology, medical dermatology, connective tissue disease


A 25-year-old woman with a history of systemic lupus erythematosus (SLE) was transferred from an outside hospital with a worsening painful generalized rash and oral ulcerations for the prior 3 weeks due to concern for Stevens-Johnson Syndrome / toxic epidermal necrolysis (TEN). Exam revealed denuded erythematous plaques covering over 80% of the patient’s body surface area and a 4.2 x 2.6 cm ulcerated plaque of the superior hard palate. Histology demonstrated parakeratosis and compact hyperkeratosis overlying an atrophic epidermis with vacuolar interface change, prominent keratinocyte dyskeratosis, and a thickened basement membrane zone (BMZ). The superficial dermis had a mild predominantly lymphocytic perivascular infiltrate and superficial dermal mucin deposition. Direct immunofluorescence was positive for IgG (granular, BMZ), C3 (granular, BMZ), and IgA was negative. Labs were remarkable for positive ANA (1:160), ds-DNA, anti-Smith, anti-RNP, low C3/C4, with negative anti-SSA/SSB. The clinicopathological correlation was most consistent with the diagnosis of TEN-like acute systemic lupus erythematosus (TEN-like ASLE). Our patient improved with treatment for her ASLE. This case highlights a challenging clinicopathologic differential diagnosis.


1. Ziemer M, Kardaun SH, Liss Y, et al. Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature. Br J Dermatol. 2012 Mar;166(3):575-600.

2. Mandelcorn R, Shear N. Lupus-associated toxic epidermal necrolysis: A novel manifestation of lupus? J Am Acad Dermatol. 2003;48(4):525-29.

3. Tiao J, Reng R, Carr K et al. Using the American College of Rheumatology (ACR) and Systemic Lupus International Collaborating Clinics (SLICC) criteria to determine the diagnosis of systemic lupus erythematosus (SLE) in patients with subacute cutaneous lupus erythematosus (SCLE). J Am Acad Dermatol. 2016 May;74(5):862-9.

4. Ting W, Stone MS, Racila D, Scofield RH, Sontheimer RD. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus. 2004;13:941-50.

5. Yildirim CG, Sayar H, Ozkan F, et al. A case of toxic epidermal necrolysis-like skin lesions with systemic lupus erythematosus and review of the literature. Lupus. 2013 Jul;22(8):839-46.