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What is Steven Johnson Syndrome?



Steven Johnson Syndrome is a very serious systemic disorder that can lead to extreme morbidity and in some cases even death because of a high magnitude allergic reaction that is basically an expression of Erythema Multifome that is signified by a rash involving the skin and the mucous membranes. There may also be some minor observations related to mucous membranes in the oral, visual, nasal, vaginal, urethral, GI, and lower respiratory tract. The involvement of the respiratory organs may progress to necrosis during the development of this syndrome. In most cases the cause of illness is not usually known.



Symptoms of Steven Johnson Syndrome

Systemic symptoms of Steven Johnson Syndrome can be severe with extensive lesions that will involve multiple body areas. The skin rash will comprise of vesicles, bullae, erythematous papules, and there may even be iris lesions. Reactions of Steven Johnson Syndrome are most visible in mucous membranes and the lesions in them will include conjunctivitis and oral and genital ulcers. A majority of the cases in the United States are reported during early spring and winter and the same pattern is seen all over the world with variations for the local ecology and lifestyle.
The formation of mucosal pseudo membranes often leads to scarring in the mucous membranes and could also cause loss of the affected organ system. A mucosal shedding in the tracheobronchial tree will lead to respiratory failure and ocular sequelae that might include anterior uveitis and corneal ulceration. Primary symptoms are panophthalmitis and keratitis though in 3-10% of the patients it might lead to blindness. Penile scarring and vaginal stenosis has also been reported in some cases along with a few rare cases of renal complications.

Most common complications with Steven Johnson syndrome include keratitis, uveitis, and a perforation of the eye that results in permanent visual impairment. Other complications include gastrointestinal bleeding, nephritis, hepatitis, arthralgia, pneumonia, fever, arthritis, and myalgia. There are also some other diseases that may arise from cutaneous and mucous membrane lesions like pemphigus vulgaris, erosive lichen planus, and varicella zoster. Reiter syndrome and Bechet syndrome also show some similar symptoms and can be confused with Stevens Johnson syndrome.

Diagnosin Steven Johnson Syndrome

Successful diagnosis of SJS only takes place after 1-3 weeks with the appearance of the rash and this is brought about by exposure to known stimulus. There is no explanation as to this diagnosis.

What are the treatments for Steven Johnson Syndrome?

Treatment of SJS depends on the suspected cause. Drugs including anticonvulsants and sulfa drugs like penicillin are discontinued during the treatment. It is important that the SJS treatment be combined with diagnoses of infectious agents so that the treatment is not complicated by secondary factors.

The first diagnosis is most likely to be made by a dermatologist by identify the rash associated with Steven Johnson Syndrome. Extreme cases might require the assistance of a burn specialist and in some cases even plastic surgeons. Further treatment depends on which organs are affected and thus a pulmonologist, gastroentrologist, or nephrologists, or all three may be required for complete treatment. Systemic corticosteroids are the most commonly prescribed form of medication though there is some controversy regarding their usage.


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