The Steven-Johnson Syndrome

Steven-Johnson Syndrome is technically listed as a rare and serious disorder. More and more cases of lawsuits and attorneys are being noticed in the news and on the internet. This syndrome can result in extreme anguish and even in some cases death.

What exactly is Steven-Johnson Syndrome?

Patients with this syndrome is marked by a rash that involves the skin and mucous membranes. Minor signs have been seen in the mucous membranes in the oral, visual, nasal, vaginal, urethral and lower respiratory tract. Numerous organs throughout the entire body may be affected and these even include the eyes, digestive system and lungs. It is a severe appearance of a condition known as erythema mutiforme. Erythema mutiofrme when it is present and noticeable appears as a skin lesion associated to herpes simplex or mycoplasma. This mostly happens in children and young adults.

This syndrome is greatly more severe as it affects numerous areas of the body and broad span of lesion formations. These lesions can reach as far as the mucous membranes which in turn affect the lungs, eyes, mouth, stomach and eventually every organ there is in the body.

Usually this syndrome first makes its presence known by the start of flu-like symptoms. It then develops a painful red or purple colored rash that spreads and blisters. In time it will cause the first layer of your skin to die and shed.

Symptoms

The early symptoms are noticed in the one to fourteen day period which can include fever, sore throat, chills and headaches. Vomiting and diarrhea have also been observed in early symptoms.

Skin and mucous membrane lesions can develop quickly with clusters of outbreaks which can last from two to four weeks.

If fever persists or localized lesions become worse this indicates a superimposed infection. Continued fever has been noticed in 85% of the cases and in some cases the lesions are so bad in the mouth and mucous membranes the patient cannot consume food or drink.

You must seek immediate medical attention for this syndrome. Go to emergency medical care if any of the following occurs:

Skin pain that is vastly spread out and cannot be explained.

Face swells

Blisters on skin and mucous membranes.

Hives

Red or purple skin rash which spreads

You are shedding first layer of skin

The syndrome cause:

The exact cause for this syndrome is not always identified. Usually the cause is from medications, infection or illness.

Medication causes have included:

Antibiotics such as sulfa and penicillin

Prescription pain killers including Celebrex and Daypro

Over-the-counter medications including Advil, Ibuprofen, Motrin and even Children's Motrin or Advil

Anti-gout medications like allopurinol

Anti-convulsants used to treat seizures

Cocaine has just been added as causing this disease

Infection causes include:

Herpes

Flu

Hepatitis

Other causes which have been noted include radiation therapy and ultraviolet light. In twenty-five to fifty percent of all cases the cause is unknown.

Risk Factors

Due to the fact this syndrome is rare and not predictable, no tests are available to help list any risk factors. Some have been determined that could add to your risk for the syndrome:

Current medical conditions such as viral infections or HIV could raise the risk.

A gene called HLA-B12 if you carry it.

Complications from disease

These complications are possible and include the following:

Cellulitis an acute skin infection that could result in life-threatening complications such as meningitis.

Sepsis happens from bacteria when it enters your system due to a massive infection and gets into your blood stream. It progresses quickly and can cause death.

Eye problems from the rash getting into the eyes in severe cases it can end up in extensive tissue damage.

Internal organs can be damaged and result in conditions such as hepatitis.

Permanent damage to skin which includes scars and in enduring skin problems can cause results such as hair loss and toe nails not growing normal.

Diagnosis

Most of the times practitioner can diagnoses Steven-Johnson Syndrome by medical history, physical exam and symptoms and signs which are distinct to this syndrome. For confirmation purposes, a tissue sample of skin could be taken.

Treatments

This syndrome does require hospitalization usually in the intensive care or burn unit.

All medications in which could be causing this syndrome will be stopped. Due to the fact it is hard to determine which medication is the cause, practitioners may order all no vital medications be stopped.

Since there is really no standard care available the following may be done:

Fluid replacement due to the fact skin loss can end up in fluid loss. Fluids and nutrients may be given via a tube through your nose or stomach.

Skin care such as cool, moist compresses to aide in soothing blisters so they can heal. Health professionals also may very gently remove the dead skin and then cover with a dressing of topical cream over the areas

Some medication which normally are used to help have included:

Pain relievers for discomfort.

Antihistamines to relieve itching.

Topical steroids for inflammation.

The following may also be given and are currently being studied for this condition:

Corticosteroids by intravenously administered. They could lessen the severity of symptoms and speed up the recovery time.

Immunoglobulin IV which contains antibodies that may help the immune system stop the process of the syndrome.

Skin grafting only if a large portion of your body is affected.

If you have the syndrome it is vital to:

Know what had caused your reaction if it was by medication become aware of the name and any other closely related medications.

Make sure you alert your health care practitioner(s) that you have a history of this syndrome. If it was caused by a medication make sure they know the name of medication involved.

Wear a medical alert bracelet or necklace with information of your condition and what caused the condition.

You can view the current list of medications associated to this syndrome online at SJS Drug List.

The FDA is suggesting that patients with allergies to sulfa medications do not take Bextra or Celebrex as they have been associated to this syndrome. They are also considering adding Cymbalta to the list of drugs for this syndrome.

For more information on Steven-Johnson Syndrome:

Steven-Johnson Foundation

Steven-Johnson Syndrome Attorney's

Steven-Johnson Syndrome Support Group

Sources:

Media Fact

Steven-Johnson Foundation

Fuel The Mind

Mayo Clinic

SJS Legal