Here we highlight the cutaneous progression of toxic epidermal necrolysis (TEN)-like systemic lupus erythematosus (SLE), from initial presentation to fulminant necrosis and eventual reepithelialization.
An 18-year-old woman presented with a 3-day history of pruritus and burning of her cheeks. She had been diagnosed with SLE 1 month prior after admission for a less severe facial rash, thrombocytopenia (101,000/μL), a positive antinuclear antibody (ANA) titer (1:1280), positive Sm and ribonucleoprotein autoantibodies, negative anti-dsDNA, a mildly elevated erythrocyte sedimentation rate (ESR; 21 mm/h), and normal complement levels. She was prescribed hydroxychloroquine and a prednisone taper; she did not take the former and took the latter inconsistently. At the current presentation, she was febrile (39.5 °C), with a higher ANA titer (1:2560), an elevated ESR (40 mm/h), and persistently normal complement levels. Throughout her course, she had no evidence of renal involvement, with unremarkable urinalysis and serum creatinine levels.
Initial exam revealed erythematous to dusky patches with yellow crusting on the bilateral cheeks (Figure 1A); skin culture grew Klebsiella pneumoniae. Over the next few days, her skin findings progressed to full-thickness skin sloughing on the face and chest, and to a lesser extent, the upper extremities (Figures 1B,C). Mucous membranes were notable for ulceration at the vaginal introitus.
(A) 18-year-old female patient with SLE presenting with photodistributed erythematous to dusky patches and yellow crusting on the bilateral cheeks and proximal arms (not shown). Skin biopsy showed interface dermatitis with keratinocyte necrosis at all levels of the epidermis, consistent with TEN-like SLE. (B) Subsequent necrosis with skin sloughing of the face. (C) Necrosis and skin sloughing also included the chest. (D) Skin exam 6 months after presentation with cushingoid features and pigmentary alteration. SLE: systemic lupus erythematosus; TEN: toxic epidermal necrolysis.
Left forearm biopsy demonstrated interface dermatitis with keratinocyte necrosis at all levels of the epidermis. The differential for epidermal necrosis in SLE includes bullous SLE, Rowell syndrome, Stevens-Johnson syndrome (SJS)/TEN, and TEN-like SLE.1 TEN-like SLE is a rare subtype of acute cutaneous lupus erythematosus that mimics the blistering, cutaneous and mucosal sloughing, and full-thickness epidermal necrosis found in SJS/TEN.1,2 TEN-like SLE was favored over TEN due to photodistribution, mild mucosal involvement, lack of drug culprits, and evidence of SLE, including positive serologies and interface dermatitis on biopsy.1,2
Treatments included prednisone (uptitrated to 60 mg or 1.1 mg/kg daily), mycophenolate mofetil (uptitrated to 1 g twice daily), hydroxychloroquine (200 mg daily), and systemic antibiotics. Topical therapies included petrolatum and mupirocin 2% ointment for the face and body, tacrolimus 0.03% ointment for the body, and topical corticosteroids for vaginal ulcers. Skin necrosis stabilized within 2 weeks, with eventual reepithelialization (Figure 1D). The patient is doing well and has tapered to low-dose prednisone. This case of TEN-like SLE illustrates the importance of recognizing initial skin findings that may evolve rapidly to full-thickness necrosis.
Footnotes
CONTRIBUTIONS
KWH: conceptualization, investigation, writing - original draft, visualization. AB: conceptualization, investigation, writing - original draft. ARD: conceptualization, writing - review & editing. ADB, LEL: conceptualization, writing - review & editing, supervision.
FUNDING
The authors have no sources of funding to disclose.
COMPETING INTERESTS
The authors declare no conflicts of interest relevant to this article.
ETHICS AND PATIENT CONSENT
Institutional review board approval is not required for single case reports, according to the authors’ institution. The authors have documented consent from the patient agreeing to publication of identifiable facial images. Potential risks were reviewed with the patient, including online dissemination and indexing in public search engines.
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