2012 KAAACI Annual International Congress and East Asia Allergy Symposium

SJS and TEN: Pathogenesis and Management

Diagnosis of Stevens-Johnson Syndrome and Toxic Epidermal Necrolyis

Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea

Seung-Chul Lee

Steven Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are regarded as severity variants of the

same disease entity. Detachment of 10% of body surface area (BSA) is defined as SJS and > 30% of BSA as TEN. Detachment of 1030% of BSA is defined as SJS/TEN-overlap. SJS/TEN are characterized by an acute onset of erythema followed by extensive necrosis and detachment of the epidermis and mucous membrane. The life-threatening mucocutaneous reactions were caused by drugs in most of cases, but were rarely related with infections and unidentified causes. As for severe adverse reactions in the skin and mucous membrane, erythma multifore major (EMM) should be included for differential diagnosis. Previously, EMM has been considered to be SJS, but SJS/TEN should be differentiated with EMM, based on different clinical manifestations and prognosis between the two conditions. EMM should be considered for skin lesions to be restricted to acral distribution of classical targetoid lesions, either with or without mucosal involvement. SJS/TEN were applied to mucous membrane involvement and widespread vesicles without the classical lesions. Distinguishing the two conditions is also possible by their etiologies, as most of SJS/TEN are associated with drugs, but EMM is strongly related with infections, especially herpes viral infection. Diagnosis for SJS/TEN is obviously possible by skin manifestations, but skin biopsy is important to exclude other diseases. In routine histological study, fully developed SJS is distinguished by full thickness epidermal detachment with splitting above the basement membrane, minimal inflammatory infiltrate, and normal immunofluorescence. The histopathology of TEN is similar. One of most important complications of SJS/TEN is ocular involvement that may include bacterial conjunctivitis, suppurative keratitis and endophthalmitis. Late complications include impaired tear production and drainage, aberrant lashes, metaplasia of conjunctiva, and corneal ulcers. Visceral involvement including pulmonary, gastrointestinal or renal complications are possible. Laboratory tests should be carefully performed to evaluate patients status or predict prognosis as well. SCORTEN (a severity-of-illness score for TEN), which was proposed by Bastuji-Garin et al in 2000, was based on a minimal set of well-defined variables evaluated during the 24 hours after patient admission to hospital and during

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2012 KAAACI Annual International Congress and East Asia Allergy Symposium

hospitalization. It evaluates seven predictive factors, namely age over 40 years, presence of malignancy, detached BSA over 10%, heart rate over 120/min, serum urea level over 10 mM, serum bicarbonate level less 20 mM, and serum glucose level over 14mM, and with each of these factors having a value of one point. More than 5 point of SCORTEN was revealed to be 90% of mortality rate. From our study, we found that serum level of lactate dehydrogenase is a novel marker for the evaluation of disease severity in the early stage of SJS/TEN. SJS/TEN should be promptly diagnosed to minimize sequelae and save patients lives, in that management and complications of SJS/TEN are similar to those of extensive burn.

References
1. Bastuji-Gardin S, Razny B, Stern RS, Shear H, Naldi L, Roujeau J. Clinical classification of cases of toxic epidermal necrolysis, Stevens-Johnson syndrome, and erythema multiforme. Arch Dermatol. 1993;129:92-6. 2. Bastuji-Gardin S, Fouchard N, Bertocchi M, Roujeau JC, Revuz J, Woklenstein P. SCORTEN: a severityof-illness score for toxic epidermal necrolysis. J Invest Deramtol. 2000;115:149-53. 3. Valeyrie-Allanore L and Roujeau JC. Epidermal necrolysis (Stevens-Johnson syndrome and toxic epidermal necrolysis). In: Wolff K et al. Fitzpatrics dermatology in general medicine, 7th ed. New York: McGraw Hill; 2008, p. 349-55. 4. Yun SJ, Choi MS, Piao MS, Lee JB, Kim SJ, Won YH, Lee SC. Serum lactate dehydrogenase is a novel marker for the evaluation of disease severity in the early stage of toxic epidermal necrolysis. Deramtology. 2008;217:254-9.

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