title: Toxic Epidermal Necrolysis-Like Cutaneous Lupus in Pediatric Patients: A Case Series and Review.
abstract: 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.
authors.affiliation: Department of Dermatology, Children's Hospital of Wisconsin, Milwaukee, Wisconsin;
authors.forename: JiaDe
authors.initials: J
authors.lastname: Yu
authors.affiliation: Divisions of Dermatology and.
authors.forename: Heather
authors.initials: H
authors.lastname: Brandling-Bennett
authors.affiliation: Children's Hospital of Wisconsin, Milwaukee, Wisconsin; Section of Rheumatology, Department of Pediatrics, and.
authors.forename: Dominic O
authors.initials: DO
authors.lastname: Co
authors.affiliation: Children's Hospital of Wisconsin, Milwaukee, Wisconsin; Section of Rheumatology, Department of Pediatrics, and.
authors.forename: James J
authors.initials: JJ
authors.lastname: Nocton
authors.affiliation: Rheumatology, Department of Pediatrics, Seattle Children's Hospital, Seattle, Washington; and Division of Rheumatology, Department of Pediatrics, University of Washington, Seattle, Washington.
authors.forename: Anne M
authors.initials: AM
authors.lastname: Stevens
authors.affiliation: Division of Pediatric Dermatology, Departments of Dermatology and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin; ychiu@mcw.edu.
authors.forename: Yvonne E
authors.initials: YE
authors.lastname: Chiu
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000175:diagnosis* / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008297:Male; D012867:Skin / Q000473:pathology*; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis* / Q000209:etiology