A patient presents with flu-like symptoms, followed by onset of a painful red or purplish rash that spreads and blisters, and may cause the top layer of skin to die and shed. What’s your diagnosis?
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Stevens-Johnson Syndrome (SJS), or erythema multiforme major, is a rare but serious cytotoxic immune reaction to drugs, particularly sulphonamides, and is less commonly caused by viral infections, vaccination or graft-versus-host disease.
Sulphonamide drugs are chemotherapeutic agents effective against a wide range of bacteria, including those causing meningitis and gonorrhea. SJS has also been linked to anticonvulsive drugs including phenytoin, carbamazepine, phenobarbital and hydantoins, as well as to nonsteroidal anti-inflammatory drugs, particularly butazone and oxicam derivatives. In developing countries antituberculosis drugs have been implicated in SJS.
Patients with multiple conditions that involve immune activation, such as collagen vascular disease, neoplasia and lymphoma, may be at higher risk for SJS, as well as those who have had recent exposure to UV light and x-rays.
SJS is characterized by severe blistering of the skin and lesions on the mucosa of the eyes, mouth or genitalia. Patients with rapidly developing SJS may experience a mucocutaneous reaction known as toxic epidermic necrolysis (TEN), characterized by widespread erythemas, necrosis and bullous detachment of the epidermis that resembles scalding.
Patients may experience a nonspecific one to 14 day prodromal period with fever, malaise, headache, rhinitis, cough, sore throat, chest pain, vomiting, diarrhea, myalgias and arthralgias.
Onset is sudden and varies widely in terms of the lag period between drug exposure and onset, rapidity of disease progression, body-surface area and internal organ involvement. Symptoms may include facial and tongue swelling; hives; skin pain; a red or purple rash that can spread over the course of hours or days; blisters on the mouth, nose or eyes; and skin shedding.
Secondary complications may include secondary skin infections, sepsis, eye problems and permanent skin damage. Although rare, patients with SJS and TEN may experience severe internal organ involvement, most often affecting the respiratory and gastrointestinal tracts.
Currently, no guidelines exist for SJS treatment. Caring for patients with SJS-TEN can be difficult and requires flexibility to adjust for the cause, type and stage of the condition, as well as the presence of complications. The first step is attempting to identify the provocative agent and discontinuing use.
Very mild cases may be treated on an outpatient basis, but the majority of cases require admission to hospital dermatology units and some may require admission to an intensive care burn unit.
Treatment often includes supportive care with fluid replacement and skin care such as applying cool, wet compresses to soothe blisters as they heal, dead skin removal and dressing affected areas with clean bandages and topical anesthetics.
Clinicians may consider prescribing pain medication, antihistamines and topical steroids to reduce discomfort, itching, and inflammation, as well as antibiotics for secondary skin infections.
Course and prognosis
SJS mortality rates typically depend on disease severity and quality of care and can range from 1% for uncomplicated cases to between 5% and 50% of SJS-TENS cases. Recovery can take anywhere from three to six weeks to months. New lesions may appear for days or weeks if a long acting drug is the inciting agent and disease can recur if the drug is taken again.