What is SJS/TEN?

Stevens-Johnson syndrome (SJS) or its severe form ‘toxic epidermal necrolysis (TEN)’ is an acute systemic mucocutaneous disease characterized by a sudden high fever which is followed by erosion and blistering of the skin and mucous membranes throughout the body. Since it often develops after medication of some form, it is regarded as “severe drug eruption,” a serious adverse drug reaction. The mechanism of onset of the disease has not been revealed. Therefore, it is sometimes difficult even for specialists to make a diagnosis, and only a poor prognosis may be achieved even with all treatment possible.

SJS/TEN primarily requires systemic management because of its high mortality rate in the acute phase. Even patients who survive the disease often have sequelae of severe visual impairment and dry eye and can extremely rarely achieve rehabilitation (See the figure below).

Since SJS/TEN is a rare disease with an extremely low incidence and newly develops only in a few out of a million people annually, not many physicians have an experience of diagnosing and treating acute-phase SJS/TEN. On the other hand, patients who suffer from ocular sequelae will have visual impairment and dry eye as lifelong sequelae.

This website provides explanations on the diagnosis and treatment of SJS/TEN and the social-welfare system for the disease in order to improve the prognoses of the ocular sequelae associated with the disease and assist patients suffering from it.

Disclaimer

There are many other diseases that may be mistaken for SJS/TEN. Therefore, it is sometimes difficult even for experienced physicians to make a diagnosis. In addition, patients physically respond differently to treatment, depending on their ages, concurrent conditions and systemic conditions. It is attending physicians that make diagnoses and determine treatment strategies, and no one can take responsibility for patients’ prognoses as a result of practice of what is on this website. Please also note that physicians in the actual clinical practice will never be held responsible for not complying with the descriptions on this website.

Copyright

This website was created as part of “Establishment of the prophylactic and treatment methods of the sequelae of SJS/TEN based on patient support” and “Research study on severe erythema exudativum multiforme,” which are the Research Program for Overcoming Intractable Diseases by the Ministry of Health, Labour and Welfare with the cooperation of an order-made medicine realization program “Identification of the genetic predispositions related to the onset of severe drug eruption due to common cold medicines and clarification of the clinical conditions.” Unauthorized reprinting of all or partial descriptions on this website is prohibited.