Steven-Johnson Syndrome

Stevens-Johnson Syndrome

I . Introduction

Each year , allergic reactions to drugs are a major cause of death in the United States . One disease that contributes to the annual death rate caused by allergic reactions to drugs is Erythema multiforme major also known as Stevens-Johnson syndrome (SJS . SJS is a serious and life-threatening allergic reaction to medication . It is a complex immunological skin disease that can be fatal . Also known as erythema multiforme major , SJS is part of an overlapping spectrum of three related diseases that manifest symptoms on the skin and in

the eyes SJS occurs most often in children and young adults

The entire spectrum of related diseases includes Steven-Johnson Syndrome (SJS , toxic epidermal necrolysis (TEN ) and erythema multiforme (EM . These encompass an overlapping spectrum of acute , self-limited exanthems . EM is a relatively common but mild form frequently related to herpes simplex virus (HSV ) infection . SJS and TEN are less common and can be life-threatening forms with mucocutaneous involvement usually due to a reaction to drug medications . Generally , the term SJS is used to refer to disease limited to less than 10 of the body surface area and TEN to disease involving more than 30 of the skin . Both are characterized by acute skin blistering and blistering on at least two mucous membranes . Although EM , SJS , and TEN are widely believed to represent various expressions of a disease continuum (Chan H .L . 1984 Revus et al . 1984 ) referred to as the "EM /SJS /TEN disease spectrum (Power et al , 1995 Wilkins et al , 1992 , this view is not universally held (Roujeau , 1997 Auquier-Dunant et al 2002 ) TEN is the most severe form of erythema . Many researchers consider SJS and TEN as different manifestations of the same disease . Individuals with TEN can experience skin loss because the skin sloughs off is sheets as the name implies (epidermal necrolysis

Ferdinand von Hebra first reported erythema multiforme (EM ) in 1860 . He described it as a self-limited cutaneous disease that was characterized by multiform skin lesions . Just over sixty years later in 1922 , Stevens and Johnson (Mondino , 1990 Power et al , 1995 ) for whom SJS was subsequently named described two boys with Stevens-Johnson syndrome (SJS , a more severe mucocutaneous disease (eruptive fever and stomatitis ) with ophthalmologic manifestations . A similar and apparently related disease was later uncovered and described by Lyell (5 , 6 ) who characterized the condition by extensive epidermal "scalding " and named it "toxic epidermal necrolysis (TEN . Today EM SJS and TEN are generally viewed as related conditions or different extremes of a set of related diseases

The cause of the EM /SJS /TEN complex remains unknown , but it is generally assumed to be an autoimmune response to an environmental factor such as a drug or virus (Revus et al . 1984 Wilkins et al 1992 ) Certain drugs and classes of drugs are now known to trigger some cases of the EM /SJS /TEN complex as will be discussed later . Abe et al showed that the epidermal necrolysis , that is , the breaking off of dead...