If you are like most people, you may think there are no dermatological emergencies… Well, there are a few, and one of them is called Steven-Johnson’s Syndrome. Unfortunately, it can be lethal. In fact, just today, June 19th, a Sudanese basketball Player named Manute Bol died at the age of 47 from complications of Stevens-Johnson Syndrome. Manute Bol was a 7′7” basketball player that played for the Warriors and also participated in numerous charities in Sudan. He will be missed by many.
Steven Johnson’s syndrome is a life-threatening disease that is considered to be a hypersensitivity reaction in which immune complexes are deposited to affect the skin and mucosa. It is considered to be an extreme form of erythema multiforme in which cell death cause the top layer of the skin/epidermis separates and peels off from the dermis/second layer of the skin. It is often talked about in conjunction with a similar disease called toxic epidermolysis syndrome (TEN). Steven Johnson’s Syndrome (SJS) may be precipitated by viral illness, drugs (especially cocaine), malignancies. In Bol’s case, it was most likely due to his medications for his kidney disease. Bol also suffered from kidney failure requiring dialysis and it was thought that the medications he received may have caused the SJS. What is interesting about this case, is most cases of SJS tend to involve Caucasians, but like our cas with men twice more likely than women to be affected. This disease can affect any age group, even infants, but clusters in teh 2nd-4th decade of life. Death like that Bol suffered is similar to death by burning, you are losing your skin and you are losing fluids. Therefore, death is likely through dehydration. However, also because the skin has lost its functional ability to act as an innate barrier against infection, infection and consequent sepsis may arise as well and lead to death.
(Above is a patient with SJS- notice the sloughing of the skin)
According to emedicine, “Mortality is determined primarily by the extent of skin sloughing. When body surface area (BSA) sloughing is less than 10%, the mortality rate is approximately 1-5%. However, when more than 30% BSA sloughing is present, the mortality rate is between 25% and 35%, and may be as high as 50%.”
Treatment is geared at replacing fluids and electrolytes and securing the ABCDEs. The offending agent must be addressed and if it is a drug, it should be discontinued. It is unfortuante these efforts were not enough to save Manute Bol.