Steven-Johnson Syndrome (SJS) is an immune mediated hypersensitivity complex, most often triggered by medications (Foster, 2011). It is characterized by a prodrome of malaise and fever, followed by rapid onset of erythematous or purpuric macules and plaques. The skin lesions progress to epidermal necrosis and sloughing. Mucosal membranes are affected in 92 to 100 percent of patients, usually at two or more distinct sites (High & Nirken, 2012).
The syndrome was first described in 1922, when the American pediatricians Albert Mason Stevens and Frank Chambliss Johnson reported the cases of 2 boys aged 7 and 8 years with "an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis". Both cases had been misdiagnosed by primary care physicians as hemorrhagic measles (Foster, 2011).
Erythema multiform (EM), originally described by von Hebra in 1866, was part of the differential diagnosis in both cases but was excluded because of the "character of skin lesions, the lack of subjective symptoms, the prolonged high fever, and the terminal heavy crusting." Despite the presence of leukopenia in both cases, Stevens and Johnson in their initial report suspected an infectious disease of unknown etiology as the cause (Hazin, Ibrahimi, Hazin & Kimyai-Asadi, 2008).
In 1950, Thomas divided EM into 2 categories: erythema multiform minor and erythema multiform major. Since 1983, erythema multiform major and Stevens-Johnson syndrome had been considered synonymous (Foster, 2011).
In the 1990s, however, Bastuji and Roujeau each proposed that erythema multiform major and Stevens-Johnson syndrome are 2 distinct disorders (Roujeau, 1997). They suggested that the diagnosis of erythema ...
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...of the mucous membranes, vaginal synechiae, and nail dystrophy, and diffuse hair loss (Habif, 2004).
Foster, C. S. (2011, September 23). Steven-Johnson syndrome. Retrieved from
Hazin, R., Ibrahimi, O. A., Hazin, M. I., & Kimyai-Asadi, A. (2008). Steven-Johnson syndrome:
Pathogenesis, diagnosis, and management . Annals of Medicine, 40(2), 129-138.
High, W.A., Nirken, M.H. (2012). Steven-Johnson syndrome and toxic epidermal necrolysis:
Management, prognosis, and long-term sequelae. Retrieved from http://www.uptodate.com/contents/steven-johnson-syndrome-and-toxic-epidermal-necrolysis
McCance, K. L., Huether, S. E., Brashers, V. L., & Rote, N. S. (2010). Pathophysiology: The biological basis for disease in adults and children. (6th ed., pp. 1644-1645). Maryland Heights, Virginia: Mosby.
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