Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay

Toxic epidermal necrolysis and Stevens-Johnson syndrome are actually one condition with the former name referring to the more serious condition while the latter refers to the milder form of the condition. It is a rare disease that has a genetic etiology but whose manifestation is precipitated by the use of certain medications. It shows as a cutaneous reaction and affects between 1.6 and 9.2 persons per annum in the United States (Seminario-Vidal et al., 2020). Mortality in the more severe toxic epidermal necrolysis is 50% while in the milder Stevens-Johnson syndrome it is 10% (NIH, 2020). Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay.

The characteristic findings of the immune dysfunction in this condition are a severe skin reaction to some particular drugs and damage to the mucous membranes, caused by genetic predisposition. The cutaneous reaction starts as blisters which then turn into burn-like lesions that then get easily infected (NIH, 2020; Seminario-Vidal et al., 2020). Because the mucous membranes are indiscriminately affected, the victims suffer respiratory, gastrointestinal, genitourinary, and visual problems. The symptomatology that the patient resents with is therefore fever, rhinorrhea, burn-like skin erosions or lesions which start from the face and then spread to the rest of the body, dysphagia, dyspnea, dysuria, corneal and conjunctival damage leading to visual impairment, and sexual dysfunction (NIH, 2020; Seminario-Vidal et al., 2020).

The symptoms of this condition complicate daily living in that the patient will suffer social stigma because of the unsightly burn-like lesions. This may lead to social isolation and depression. The patient cannot engage in meaningful socio-economic activities because the skin erosions are easily infected. So they remain indoors in sterile or semi-sterile conditions. The visual impairment will also curtail the victim’s movements. Lastly but not least, the sexual dysfunction as a result to the damage caused to the mucous lining of the genitourinary tract may cause marital problems and break relationships.

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References

National Institutes of Health [NIH] (2020). Stevens-Johnson syndrome/ toxic epidermal necrolysis. Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay. https://ghr.nlm.nih.gov/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis

Seminario-Vidal, L., Kroshinsky, D., Malachowski, S.J., Sun, J., Markova, A., Beachkofsky, T.M., Kaffenberger, B.H., Ergen, E.N., Mauskar, M., Bridges, A., Calhoun, C., Cardones, A.R., Chen, S.T., Chodosh, J., Cotliar, J., Davis, M.D.P., DeNiro, K.L., Dominguez, A.R., Eljure-Téllez, J., Femia, A. et al. (2020). Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/ toxic epidermal necrolysis in adults. Journal of the American Academy of Dermatology, 82, 1553-1567. https://doi.org/10.1016/j.jaad.2020.02.066

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a severe skin
reaction most often triggered by particular medications. Although Stevens-Johnson
syndrome and toxic epidermal necrolysis were once thought to be separate conditions,
they are now considered part of a continuum. Stevens-Johnson syndrome represents
the less severe end of the disease spectrum, and toxic epidermal necrolysis represents
the more severe end.
SJS/TEN often begins with a fever and flu-like symptoms. Within a few days, the skin
begins to blister and peel, forming very painful raw areas called erosions that resemble
a severe hot-water burn. The skin erosions usually start on the face and chest before
spreading to other parts of the body. In most affected individuals, the condition also
damages the mucous membranes, including the lining of the mouth and the airways,
which can cause trouble with swallowing and breathing. The painful blistering can also
affect the urinary tract and genitals. SJS/TEN often affects the eyes as well, causing
irritation and redness of the conjunctiva, which are the mucous membranes that protect
the white part of the eye and line the eyelids, and damage to the clear front covering of
the eye (the cornea). Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay.
Severe damage to the skin and mucous membranes makes SJS/TEN a life-threatening
disease. Because the skin normally acts as a protective barrier, extensive skin
damage can lead to a dangerous loss of fluids and allow infections to develop. Serious
complications can include pneumonia, overwhelming bacterial infections (sepsis),
shock, multiple organ failure, and death. About 10 percent of people with StevensJohnson syndrome die from the disease, while the condition is fatal in up to 50 percent
of those with toxic epidermal necrolysis.
Among people who survive, long-term effects of SJS/TEN can include changes in skin
coloring (pigmentation), dryness of the skin and mucous membranes (xerosis), excess
sweating (hyperhidrosis), hair loss (alopecia), and abnormal growth or loss of the
fingernails and toenails. Other long-term problems can include impaired taste, difficulty
urinating, and genital abnormalities. A small percentage of affected individuals develop
chronic dryness or inflammation of the eyes, which can lead to increased sensitivity to
light (photophobia) and vision impairment.
Frequency
SJS/TEN is a rare disease, affecting 1 to 2 per million people each year. StevensJohnson syndrome (the less severe form of the condition) is more common than toxic
epidermal necrolysis.
People who are HIV-positive and those with a chronic inflammatory disease called
systemic lupus erythematosus are more likely to develop SJS/TEN than the general
population. The reason for the increased risk is unclear, but immune system factors and
exposure to multiple medications may play a role.
Causes
Several genetic changes have been found to increase the risk of SJS/TEN in response
to triggering factors such as medications. Most of these changes occur in genes that
are involved in the normal function of the immune system.
The genetic variations most strongly associated with SJS/TEN occur in the HLA-B
gene. This gene is part of a family of genes called the human leukocyte antigen (HLA)
complex. The HLA complex helps the immune system distinguish the body’s own
proteins from proteins made by foreign invaders (such as viruses and bacteria). The
HLA-B gene has many different normal variations, allowing each person’s immune
system to react to a wide range of foreign proteins. Certain variations in this gene occur
much more often in people with SJS/TEN than in people without the condition.
Studies suggest that the HLA-B gene variations associated with SJS/TEN cause the
immune system to react abnormally to certain medications. In a process that is not well
understood, the drug causes immune cells called cytotoxic T cells and natural killer
(NK) cells to release a substance called granulysin that destroys cells in the skin and
mucous membranes. The death of these cells causes the blistering and peeling that is
characteristic of SJS/TEN.
Variations in several other HLA and non-HLA genes have also been studied as
potential risk factors for SJS/TEN. However, most people with genetic variations that
increase the risk of SJS/TEN never develop the disease, even if they are exposed to
drugs that can trigger it. Researchers believe that additional genetic and nongenetic
factors, many of which are unknown, likely play a role in whether a particular individual
develops SJS/TEN. Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay.
The drugs most frequently associated with SJS/TEN include several medications that
are used to treat seizures (particularly carbamazepine, lamotrigine, and phenytoin);
allopurinol, which is used to treat kidney stones and a form of arthritis called gout;
a class of antibiotic drugs called sulfonamides; nevirapine, which is used to treat
HIV infection; and a type of non-steroidal anti-inflammatory drugs (NSAIDs) called
oxicams. Other factors may also trigger SJS/TEN. In particular, these skin reactions
have occurred in people with an unusual form of pneumonia caused by infection with
Mycoplasma pneumoniae and in people with viral infections, including cytomegalovirus.
Researchers suspect that a combination of infections and drugs could contribute to the
disease in some individuals. In many cases, no definitive trigger for an individual’s SJS/
TEN is ever discovered.
Inheritance Pattern
SJS/TEN is not an inherited condition. However, the genetic changes that increase the
risk of developing SJS/TEN can be passed from one generation to the next. Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Essay.