title: Systemic lupus erythematosus presenting as Stevens-Johnson syndrome/toxic epidermal necrolysis.
abstract: Stevens-Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN)-like lesions in acute cutaneous lupus erythematosus [LE]) are an unusual manifestation of systemic LE. We describe a patient with widespread vesiculobullous lesions diagnosed as SJS/TEN-like acute cutaneous LE as the initial presentation of systemic LE. Stevens-Johnson syndrome/TEN-like LE may be differentiated from other vesiculobullous lesions by factors including a history of recent LE exacerbation, photodistribution of lesions, lack of a precipitating infection or medication exposure, minimal mucosal involvement, a prolonged course, response steroid treatment, and histologic and immunofluorescence findings. It is paramount to identify SJS/TEN-like LE as this condition requires early and aggressive intervention. The optimal treatment approach for SJS/TEN-like LE is unclear, and although some case reports have shown glucocorticoids to be useful, there are also reports of cases in which additional measures, such as intravenous immunoglobulin and plasmapheresis, were required to achieve a response. Our patient's condition was refractory to high-dose corticosteroids and intravenous immunoglobulin but was successfully treated using plasma exchange. As such, this treatment may hold potential for improving the care of other patients with refractory SJS/TEN-like LE.
authors.affiliation: From the *School of Medicine, †Division of Rheumatology, Departments of Medicine, ‡Dermatology, and §Pathology and Dermatology, University of Virginia, Charlottesville, VA.
authors.forename: Mary Grace
authors.initials: MG
authors.lastname: Baker
authors.forename: Nicole D
authors.initials: ND
authors.lastname: Cresce
authors.forename: Mariam
authors.initials: M
authors.lastname: Ameri
authors.forename: Adam A
authors.initials: AA
authors.lastname: Martin
authors.forename: James W
authors.initials: JW
authors.lastname: Patterson
authors.forename: Donald L
authors.initials: DL
authors.lastname: Kimpel
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000628:therapy; D010956:Plasmapheresis; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis* / Q000209:etiology*; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D016756:Immunoglobulins, Intravenous