Toxic Epidermal Necrolysis - Stevens Johnsons Syndrome

Toxic Epidermal Necrolysis - Stevens Johnsons Syndrome

Toxic Epidermal Necrolysis is a life-threatening inflammatory condition affecting the SKIN and underlying connective tissues, also called Stevens-Johnson syndrome. Toxic epidermal necrolysis usually results as an adverse DRUG reaction though may occur as a complication of INFECTION or CANCER. Doctors believe toxic epidermal necrolysis develops when an external event triggers the mechanism for programmed cell death (apoptosis), causing massive numbers of keratinocytes (the cells that primarily comprise the skin) to die. This in turn activates the body’s IMMUNE RESPONSE, which attacks the dying cells. The massive death of keratinocytes results in large segments of skin sloughing off, leaving the underlying tissue exposed. Toxic epidermal necrolysis typically evolves over a period of 10 to 14 days, though once the skin eruptions begin deterioration is rapid.

Diagnosis is by skin biopsy, which shows the characteristic pattern of cell destruction and abundance of killer T-cells. In most situations the first line of treatment is plasmapheresis, a process somewhat similar to dialysis in which a mechanical BLOOD separator removes the serum and replaces it with donor serum. Plasmapheresis helps clear antibodies from the serum, reducing the immune response. Other treatments include frequent surgical débridement of skin surfaces, skin grafts to cover denuded surfaces, and precise fluid and electrolyte replacement.

Toxic epidermal necrolysis has a survival rate of about 60 percent. Those who survive often have long-term complications and face a challenging road to rehabilitation and recovery. The massive loss of skin causes extensive scarring similar to that of serious BURNS. The eyes also experience damage as the sloughing affects the conjunctiva and sclera (EYE tissues).

See also ADVERSE REACTION; ANTIBODY; HEMAPHERESIS; KERATINOCYTE; STAPHYLOCOCCAL SCALDED SKIN SYNDROME.

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