Symptoms of Stevens-Johnson Syndrome
Typically, Stevens-Johnson syndrome affects the mucous membranes of the oral cavity, nostril, eyes, and both the anal and genital regions. It may or may not be associated with skin lesions elsewhere on the body. Oropharyngeal (mouth) lesions may be so intolerable as to prevent eating, and there may be recurring oral ulcers. Often, the skin lesions may look like a target-lesion (3 concentric zones of color change) or as bullae (bubble-like).
A painful conjunctivitis may occur in the eye, frequently with a pus discharge, and can lead to corneal scarring and loss of vision. In addition to the mucous membrane lesions, fever and exhaustion are usual.
Approximately one-third of patients have pulmonary involvement with cough and patchy infiltrates (material that has permeated into the tissues) on chest x-ray. In fatal cases, renal failure and pneumonia may occur.
Symptoms
Stevens-Johnson syndrome and toxic epidermal necrolysis usually begin with fever, headache, cough, and body aches. Then a flat red rash breaks out on the face and trunk, often spreading later to the rest of the body in an irregular pattern. The areas of rash enlarge and spread, often forming blisters in their center. The skin of the blisters is very loose and easy to rub off. In Stevens-Johnson syndrome, less than 10% of the body surface is affected. In toxic epidermal necrolysis, large areas of skin peel off, often with just a gentle touch or pull. In many people with toxic epidermal necrolysis, 30% or more of the body surface peels away. The affected areas of skin are painful, and the person feels very ill with chills and fever. In some people, the hair and nails fall out. The active stage of rash and skin loss can last between 1 day and 14 days.
In both disorders, blisters break out on the mucous membranes lining the mouth, throat, anus, genitals, and eyes. The damage to the lining of the mouth makes eating difficult, and closing the mouth may be painful, so the person may drool. The eyes may become very painful, swell, and become so filled with pus that they seal shut. The corneas can become scarred. The opening through which urine passes (urethra) may also be affected, making urination difficult and painful. Sometimes the mucous membranes of the digestive and respiratory tracts are involved, resulting in diarrhea and difficulty breathing.
The skin loss in toxic epidermal necrolysis is similar to a severe burn and is equally life threatening. Huge amounts of fluids and salts can seep from the large, raw, damaged areas. A person who has this disorder is very susceptible to organ failure and infection at the sites of damaged, exposed tissues. Such infections are the most common cause of death in people with this disorder.
Symptoms and Outcome of SJS
An issue that may possibly hinder an early diagnosis of Stevens Johnson Syndrome lies in the symptoms. The initial symptoms of Stevens Johnson Syndrome are quite similar to those of influenza (flu): high fever, sore throat, cough, feeling lethargic, and even nausea and vomiting. In the early stages of the illness, when flu-like symptoms occur it is still too soon to diagnose. Misdiagnosis is common, even at the onset of other symptoms.
A diagnosis normally comes at the onset of target lesions (lesions with two zones of color, resembling a target) or a rash. However, target lesions are not always seen in patients. Diagnosis at this point is crucial: it has been observed that death rates are lower when causative drugs are withdrawn no later than the day when blisters, rash or lesions first occur. That rash may look like hives or mere red and purple skin pigmentation but will eventually lead to blisters and sloughing of the skin. The blisters, when ruptured, leave the denuded skin susceptible to secondary infection and severe scarring. Although the palms, soles, dorsum of the hands (the back of the hands between the wrist and knuckles), and extensor surfaces (the backs of the fingers) are most commonly affected, lesions may appear anywhere.
Symptoms that typically follow the rash are facial and tongue swelling. The swelling can force the eyes shut and a patient may be unable to speak and/or swallow. A patient with SJS, if he or she goes untreated for even a short amount of time, will eventually experience painful blisters on their skin -- especially in or on their mouth, nose, and eyes. The blisters can eventually fuse the patient's tongue to the palette, cut off the patient's airway, and result in scarring of the cornea, affecting the patient's ability to breathe on their own, eat through anything other than a feeding tube, and will likely render the patient unable to see. These effects can be long term and may alter the future life of any SJS sufferer.
If an SJS case is left untreated for longer periods of time, some say just mere weeks, a patient will most likely experience sloughing of the skin; where the epidermis (outer layers of skin) actually separate from the dermis (inner layers), exposing the nerve endings in the area, resulting in excruciating pain. When blisters and sloughing occur, the patient is normally admitted to the burn unit for treatment. As seen in severe burns, the patient typically experiences a massive loss of fluids, electrolyte imbalance, super infection, thermoregulation impairment, excessive energy expenditure and an alteration in immunologic functions, making the patient incredibly susceptible to other diseases and viruses. Once the sloughing phase begins, the pain can be so unbearable that the probability of the patient going into shock is very high. If the pain sends the patient into shock, or any of the other conditions worsen, it may lead to death.
