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Pediatrics. 2016 Jun;137(6). pii: e20154497. doi: 10.1542/peds.2015-4497.

Toxic Epidermal Necrolysis-Like Cutaneous Lupus in Pediatric Patients: A Case Series and Review.

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Department of Dermatology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;
Divisions of Dermatology and.
Children's Hospital of Wisconsin, Milwaukee, Wisconsin; Section of Rheumatology, Department of Pediatrics, and.
Rheumatology, Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington; and Division of Rheumatology, Department of Pediatrics, University of Washington, Seattle, Washington.
Division of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin;


Bullous eruptions in patients with underlying systemic lupus erythematosus (LE) can mimic toxic-epidermal necrolysis (TEN), a rapidly progressive mucocutaneous reaction usually associated with medication use. Differentiating between classic drug-induced TEN and TEN-like cutaneous LE is important but difficult. We report a series of 3 patients with pediatric systemic LE who were admitted with severe worsening of skin disease resembling TEN. However, the initial photo-distribution of the eruption, subacute progression, limited mucosal involvement, mild systemic symptoms, supportive biopsy and laboratory results, and lack of culprit drugs was more suggestive of a TEN-like cutaneous LE. These patients recovered with various systemic immunosuppressive medications including methylprednisolone, intravenous immunoglobulin, and plasmapheresis. Our cases are rare and demonstrate key clinical and histologic features of TEN-like cutaneous LE in young patients and the importance of differentiating this entity from drug-induced TEN.

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