Stevens Johnson Syndrome
- A History of the Disease

Stevens Johnson Syndrome is an immune-complex-mediated hypersensitivity (allergic) condition that typically occurs as an adverse reaction to certain drugs. Eliciting fatal results in approximately 3% to 15% of sufferers, Stevens Johnson Syndrome is one of three similar skin conditions of varying severity:

Erythema multiforme (EM): a type of skin condition that is characterized by the development of symmetrically raised red skin areas without displaying whole-body symptoms. EM is a condition that frequently occurs in children and young adults. It is the least severe of the three related skin disorders.

Stevens Johnson Syndrome (SJS): also referred to as EM-major, Stevens Johnson Syndrome causes the inflammation of the skin and mucous membranes of the body. Stevens Johnson Syndrome is viewed to be a more severe type of erythema multiforme (hence the EM-major classification) and typically affects people over the age of 40; however, SJS cases have been reported in children as young as three months old.

Toxic epidermal necrolysis (TEN): the most serious of the three related skin diseases, toxic epidermal necrolysis is a life-threatening condition that is characterized by the blistering / peeling of the top layer of skin. With a mortality rate of 40%, TEN can also be caused as an allergic reaction to certain drugs.

SJS can cause damage to many parts of the body, including the eyes, mouth and large areas of skin. For corneal scarring, certain types of laser eye surgery, such as PRK, can be used for treatment. Ask an eye surgeon about PRK complications that could occur related to laser eye surgery to correct corneal scarring. For mouth pain, it is best to find a local dentist, but not one who specializes in orthodontics.

History of SJS

Stevens Johnson Syndrome was initially described by Drs. Stevens and Johnson in 1922 following a chance encounter with two young boys (aged seven and eight) who were suffering from a mysterious skin disease. The boys had been diagnosed by their primary care physician to be suffering from a severe case of hemorrhagic measles.

Stevens and Johnson described the boys' condition as "extraordinary." The boys showed signs of inflamed buccal mucosa (mucous lining of the cheeks) and severely puss-filled eyeballs (purulent conjunctivitis) in addition to generalized skin lesions that are now commonly associated with Stevens Johnson Syndrome. Certain that the hemorrhagic measles diagnosis was incorrect, Stevens and Johnson next considered a skin disease called erythema multiforme.

- Erythema multiforme was originally described in 1866 by an Austrian physician and dermatologist, Ferdinand Von Hebra. Von Hebra was instrumental in the founding of the Vienna School of Dermatology and responsible for penning one of the most famous dermatological books of all time, Atlas der Hautkrankeiten. Von Hebra described EM as a mild illness causing the sudden onset of many "red papules" (elevation of the skin) that were recurrent in some patients. Some of the red papules developed into what Von Hebra dubbed to be "target" lesions. -

Based on Von Hebra's description, Stevens and Johnson were able to exclude EM as a possible diagnosis for the boys' condition because of a variety of symptoms that didn't fit with erythema multiforme:

  • The skin lesions were of a more severe nature than the papules associated with EM.
  • Lack of subjective symptoms (symptoms you experience but don't necessarily complain of)
  • Continually high fever
  • "Terminal heavy crusting"

Stevens and Johnson instead believed that what they were seeing was in fact a type of infectious disease stemming from unknown causes. In 1950, this discovery was dubbed Stevens Johnson Syndrome or EM-major by Bernard Thomas, MD.

The classification of Stevens Johnson Syndrome as EM-major has been something of a controversy since the early 1980s. It has been suggested that erythema multiforme and Stevens Johnson Syndrome are of a different enough variety to warrant distinctly separate classifications.

Regardless of its classification, Stevens Johnson Syndrome remains an incurable skin disease that can be potential life-threatening.

Last Revision: July 28, 2010

MediaFact is an important Internet resource about drug side effects related to Stevens Johnson Syndromeand Primary Pulmonary Hypertension.