Stevens–Johnson Syndrome

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Annotated Information

Classification and Resources

ID 118
MeSH MSH:D013262;


Stevens–Johnson syndrome, a form of toxic epidermal necrolysis, is a life-threatening skin condition, in which cell death causes the epidermis to separate from the dermis The syndrome is thought to be a hypersensitivity complex that affects the skin and the mucous membranes The most well-known causes are certain medications, but it can also be due to infections, or more rarely, cancers~


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SJS usually begins with fever, sore throat, and fatigue, which is commonly misdiagnosed and therefore treated with antibiotics Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips, but also in the genital and anal regions Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink Conjunctivitis of the eyes occurs in about % of children who develop SJS[medical citation needed] A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp~Pathology[edit]~~This section needs additional citations for verification Please help improve this article by adding citations to reliable sources Unsourced material may be challenged and removed (July )~~This section does not cite any references or sources Please help improve this section by adding citations to reliable sources Unsourced material may be challenged and removed (June )~SJS, like TEN and erythema multiforme, is characterized by confluent epidermal necrosis with minimal associated inflammation The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum An idiosyncratic, delayed-hypersensitivity reaction has been implicated in the pathophysiology of SJS Certain population groups appear more susceptible to develop SJS than the general population Slow acetylators, patients who are immunocompromised (especially those infected with HIV), and patients with brain tumors undergoing radiotherapy with concomitant antiepileptics are among those at most risk~Slow acetylators are people whose liver cannot completely detoxify reactive drug metabolites For example, patients with sulfonamide-induced toxic epidermal necrolysis have been shown to have a slow acetylator genotype that results in increased production of sulfonamide hydroxylamine via the cytochrome P- pathway These drug metabolites may have direct toxic effects or may act as haptens that interact with host tissues, rendering them antigenic~Antigen presentation and production of tumor necrosis factor (TNF)–alpha by the local tissue dendrocytes results in the recruitment and augmentation of T-lymphocyte proliferation and enhances the cytotoxicity of the other immune effector cells A "killer effector molecule" has been identified that may play a role in the activation of cytotoxic lymphocytes The activated CD+ cytotoxic T cells, in turn, can induce epidermal cell apoptosis via several mechanisms, which include the release of granzyme B and perforin~Perforin, a pore-making monomeric granule released from natural killer cells and cytotoxic T lymphocytes, kills target cells by forming polymers and tubular structures not unlike the membrane attack complex of the complement system Apoptosis of keratinocytes can also take place as a result of ligation of their surface death receptors with the appropriate molecules Those can trigger the activation of the caspase system, leading to DNA disorganization and cell death~Apoptosis of keratinocytes can be mediated by direct interaction between the cell-death receptor Fas and its ligand Both can be present on the surfaces of the keratinocytes Alternatively, activated T-cells can release soluble Fas ligand and interferon-gamma, which induces Fas expression by keratinocytes Researchers have found increased levels of soluble Fas ligand in the sera of patients with SJS/TEN before skin detachment or onset of mucosal lesions~The death of keratinocytes causes separation of the epidermis from the dermis Once apoptosis ensues, the dying cells provoke recruitment of more chemokines This can perpetuate the inflammatory process, which leads to extensive epidermal necrolysis Higher doses and rapid introduction of allopurinol and lamotrigine may also increase the risk of developing SJS/TEN Risk is lessened by starting these at the low doses and titrating gradually Some evidence indicates systemic lupus is a risk factor, as well~

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