title: A decade of burn unit experience with Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: Clinical pathological diagnosis and risk factor awareness.
abstract: Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN) is a rare and often fatal spectrum of mucocutaneous diseases usually attributable to severe adverse drug reactions. Burn units are referral centers for patients at the most extreme end of the disease continuum. Our burn center admits a much higher percentage of TEN (>30% BSA) cases than reported in most prior reviews. The purpose of this study was to analyze the diagnostic and prognostic value of variables collected on referred SJS/TEN patients. We retrospectively analyzed 94 patients admitted to our unit with a presumptive SJS/TEN diagnosis made in most cases by the referring center. Most of the diagnoses were clinical. Fifty of the 94 patients underwent biopsy when the clinical diagnosis was questionable. Of the 50 patients who underwent biopsy, 18 (36%) received an alternative diagnosis. Analysis was therefore limited to 76 patients, i.e. 44 patients felt to have firm clinical diagnoses plus 32 patients with diagnoses confirmed by biopsy. Mean age was 54.3 years (17-93) and overall gender ratio was 43 F vs. 33 M (56.6% vs. 43.4%). Mean LOS was 15.2 days (1-48) and overall mortality was 23.7% (18/76). Univariate analysis revealed percent body surface area (%BSA) did not show statistically significant association with mortality. Histopathological correlation for diagnosis is not standardized across institutions worldwide. Due to challenges in the diagnosis of SJS/TEN and the high incidence of error in clinical diagnosis, it is recommended that all patients with presumed SJS/TEN receive skin biopsies with H&E and direct immunofluorescence. We propose a diagnostic approach in order to address this need. Lack of association between %BSA and mortality suggests that all biopsy-proven SJS/TEN cases belong in specialty centers due to the unstable nature of the disease and risk for rapid progression.
authors: Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68178, USA. Electronic address: victoriamlim@gmail.com.
authors: Victoria M
authors: VM
authors: Lim
authors: Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68178, USA.
authors: Annie
authors: A
authors: Do
authors: University of California San Francisco, 1701 Divisadero St, San Francisco, CA 94115, USA.
authors: Timothy G
authors: TG
authors: Berger
authors: Creighton University School of Medicine, 2500 California Plaza, Omaha, NE 68178, USA.
authors: Austin H
authors: AH
authors: Nguyen
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Jeffrey
authors: J
authors: DeWeese
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: J David
authors: JD
authors: Malone
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Kathleen
authors: K
authors: Jordan
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Fred
authors: F
authors: Hom
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Lucia
authors: L
authors: Tuffanelli
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Paula
authors: P
authors: Fillari
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Shirley
authors: S
authors: Siu
authors: Bothin Burn Center, Saint Francis Memorial Hospital, 900 Hyde St, San Francisco, CA 94109, USA.
authors: Richard
authors: R
authors: Grossman
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000704:Analysis of Variance; D001830:Body Surface Area; D002053:Burn Units / Q000706:statistics & numerical data*; D003937:Diagnosis, Differential; D003875:Drug Eruptions / Q000209:etiology; D005260:Female; D006801:Humans; D007902:Length of Stay; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution; D013262:Stevens-Johnson Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology; D055815:Young Adult
keywords: Blistering; Differential diagnosis; Drug reactions; Lyell's syndrome; Stevens-Johnson Syndrome; Toxic epidermal necrolysis