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Stevens Johnson Syndrome (SJS) - Symptoms and Treatment

Stevens Johnson syndrome (SJS) is a serious systemic disorder with the potential for severe morbidity and even death consisting in immune-complex–mediated hypersensitivity complex systemic allergic reaction that is a severe expression of Erythema Multiforme with a characteristic rash which typically involves the skin and the mucous membranes, including the buccal mucosa, and also minor presentations may occur with significant involvement of oral, nasal, eye, urethral, vaginal, GI, and lower respiratory tract mucous membranes. Respiratory involvement may progress to necrosis in the course of the illness which causes are often unknown.

Stevens Johnson Syndrome Picture

It was in 1922 when Stevens and Johnson first described 2 patients; boys aged 7 and 8 years showing "an extraordinary, generalized inflamed buccal mucosa, eruption with continued fever, and severe purulent conjunctivitis." Both cases were misdiagnosed by primary care physicians as hemorrhagic measles and Erythema multiforme, originally described by von Hebra in 1866, was part of the differential diagnosis in both cases, but excluded because of the "character of skin lesions, the prolonged high fever, the lack of subjective symptoms, and the terminal heavy crusting." In spite of leukopenia, Stevens and Johnson reported the suspected of an infectious disease of unknown etiology as the cause.

Stevens Johnson Syndrome Picture

This disease occurs due to an allergic reaction to one of a number of immunologic stimuli caused by many drugs, viral infections, and other malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome, although in up to half of the cases, no specific etiology has been identified. Stevens Johnson syndrome is more common in children and young adults with a Caucasian predominance and the sex risk factor is male-to-female ratio 2:1.

Stevens Johnson Syndrome Picture

The systemic symptoms are severe and the lesions are extensive, involving multiple body areas with skin rash that consists of vesicles, erythematous papules, bullae and there may also be iris lesions. The reaction is observed especially in the mucous membranes, and the mucosal lesions include conjunctivitis as well as oral and genital ulcers. In the United States, most cases tend to have a propensity for the early spring and winter while internationally Stevens Johnson syndrome occurs with a worldwide distribution similar in etiology and occurrence to that in the United States.

It is estimated in 3 to 15 percent of cases, patients with severe Stevens Johnson syndrome die. Lesions may continue to erupt in crops for as long as 2 to 3 weeks. Until recently this disease was considered to be the same as Erythema Multiforme, but now it is thought that erythema multiforme spectrum is a single entity and SJS is a generalized hypersensitivity reaction, usually to drugs, in which the skin and mucus membrane lesions are early manifestations but may progress to its more severe form of toxic epidermal necrolysis with high morbidity and up to 40 percent mortality.

Mucosal pseudomembrane formation may lead to mucosal scarring with loss of function of the involved organ system, as well as esophageal strictures that may occur when extensive involvement of the esophagus exists. In Stevens Johnson syndrome the mucosal shedding in the tracheobronchial tree may lead to respiratory failure and ocular sequelae which may include anterior uveitis and corneal ulceration, with blindness that may develop secondary to severe keratitis or panophthalmitis in 3 to 10 percent of patients. In some cases, vaginal stenosis and penile scarring have been reported, and some renal complications in rare cases.

The most frequent complications of Stevens Johnson syndrome are uveitis, keratitis, and perforation of the globe of the eye resulting in permanent visual impairment, however gastrointestinal bleeding, hepatitis, nephritis, pneumonia, arthralgia, arthritis, fever, and myalgia may appear as result of this disease. The differential diagnosis may include other diseases that can result in cutaneous and mucous membrane lesions such as erosive lichen planus, pemphigus vulgaris, and varicella zoster that may mimic the syndrome. Also Behcet syndrome and Reiter syndrome may have ocular and genital lesions that can be confused with those observed in Stevens Johnson syndrome.

Unfortunately, the diagnosis of the syndrome is usually made until the characteristic rash appears after 1 to 3 weeks of exposure to a known stimulus that cannot be explained by any another diagnosis. The treatment depends on the suspected precipitating cause, so any drug, including sulfa drugs such as penicillin or anticonvulsants, especially phenytoin, should be discontinued. In Stevens Johnson syndrome it is important that infectious agents as herpes simplex virus 1 or 2, or Mycoplasma pneumoniae, be identified in order to treat appropriately this disease.

Consultants may help establish the diagnosis and direct inpatient care. A dermatologist is the most likely clinician to establish the diagnosis, with or without biopsy. Severe cases may require the involvement of a burn specialist or plastic surgery specialist. Depending on organ system involvement, consultations with a gastroenterologist, pulmonologist, and nephrologist may be helpful. There are no specific drug treatments for Stevens Johnson syndrome, so the choice of antibiotic depends on the associated infection. The use of systemic corticosteroids, the most commonly prescribed medication, is controversial. They are useful in high doses early in the reaction, but morbidity and mortality actually may increase in association with corticosteroid use.

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