Stevens-Johnson syndrome
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Stevens-Johnson syndrome
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Bullae p. 483
Stevens-Johnson syndrome p. 490
Stevens-Johnson syndrome p. 191, 490, 560
as drug reaction p. 249
sulfa drug allergies p. 251
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Stevens-Johnson Syndrome is named for the physicians who first reported an acute, immune-mediated condition that affects the skin and mucous membranes such as the mouth and genitals.
Stevens-Johnson syndrome and toxic epidermal necrolysis are considered to be the same condition, but are along a spectrum where toxic epidermal necrolysis is the more severe form.
Both can be fatal if not treated, and the risk of fatality increases with severity.
The skin plays an important role in protecting underlying muscles, bones, ligaments, and internal organs by forming a barrier to infectious pathogens; and preventing water loss from the body.
The outermost layer of the skin is the epidermis and, itself, is made of several layers of cells.
The deepest layer is called the stratum basale, or the base layer.
And cells here are anchored to a basement membrane, a thin layer of delicate tissue containing proteins like collagen and laminins, which attach the epidermis to the underlying skin layer called the dermis.
Similar to how the skin lines the outside of the body, mucous membranes, or mucosa, line the inner body surfaces like the mouth, tongue, respiratory tract, conjunctiva of the eyes, genitals, and anus.
Mucosa is made up of one or more layers of epithelial cells, which, again, are attached to a basement membrane that sits on top of a layer called the lamina propria.
Now, most cells in the body have a protein called major histocompatibility complex or MHC class I molecule on the surface of their membrane.
This protein presents peptides from within the cell to immune cells called cytotoxic T cells, also called a CD8+ T cells.
If the cell is healthy, the cytotoxic T cell doesn’t recognize the peptides as foreign, and nothing happens.
But if a cell is infected, say with a virus, the cytotoxic T cell can recognize the peptides as foreign and trigger an immune response.
Once all infected cells have been destroyed, the immune response resolves.
Stevens-Johnson Syndrome and toxic epidermal necrolysis are both type IV hypersensitivity reactions.
Summary
Stevens �Johnson syndrome is a life-threatening skin condition, in which cell death causes the epidermis to separate from the dermis. It is a type of IV hypersensitivity reaction where cytotoxic T-cells inappropriately attack and kill epithelial cells in the mucosa and skin epidermis. The most well-known causes are medications, such as lamotrigine and other seizure drugs. Treatment includes immediately removing the trigger, followed by pain medication, hydration, and sterile wound care.
Sources
- "Robbins Basic Pathology" Elsevier (2017)
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
- "Toxic epidermal necrolysis" Journal of the American Academy of Dermatology (2013)
- "Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis" Clinical Reviews in Allergy & Immunology (2017)