Stevens-johnson syndrome (sjs) and toxic epidermal necrolysis (ten)

[GarryDrold]

Stevens-Johnson syndrome and toxic epidermal necrolysis are two forms of the same life-threatening skin disease that cause rash, skin peeling, and sores on the mucous membranes.
(See also Introduction to Hypersensitivity Reactions and Inflammatory Skin Disorders.)

Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by drugs or infections.
Typical symptoms for both diseases include peeling skin, fever, body aches, a flat red rash, and blisters and sores on the mucous membranes.
Affected people are typically hospitalized in a burn unit and given fluids and sometimes drugs, and all suspected drugs are stopped.

Skin peeling is the hallmark of these conditions. The skin peeling involves the entire top layer of the skin (the epidermis), which sometimes peels off in sheets from large areas of the body (see Structure and Function of the Skin).

Stevens-Johnson syndrome causes only small areas of peeling skin (affecting less than 10% of the body).
Toxic epidermal necrolysis causes large areas of peeling skin (affecting over 30% of the body).
Involvement of 15 to 30% of body surface area is considered overlap of Stevens-Johnson syndrome and toxic epidermal necrolysis.

In both forms, blistering of the mucous membranes typically occurs in the mouth, eyes, and vagina.
Both disorders can be life threatening.
About half the cases of Stevens-Johnson syndrome and nearly all the cases of toxic epidermal necrolysis are caused by a reaction to a drug, most often sulfa and other antibiotics; anticonvulsants, such as phenytoin and carbamazepine ; and certain other drugs, such as piroxicam or allopurinol . Some cases are caused by a bacterial infection, vaccination, or graft-versus-host disease. Sometimes, a cause cannot be identified. In children with Stevens-Johnson syndrome, an infection is the most likely cause.
These disorders occur in all age groups. These disorders are more likely to occur in people with a bone marrow transplant, systemic lupus erythematosus, other chronic joint and connective tissue diseases, or with human immunodeficiency virus (HIV) infection (particularly when people also have pneumonia caused by Pneumocystis jirovecii). The tendency to develop one of these disorders can run in families.