Editorial: The Updated Understanding of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
Shigeru Kinoshita, Mayumi Ueta
Frontiers in Medicine · 2021
doi:10.3389/fmed.2021.811570 (opens in a new tab)
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Abstract
The Updated Understanding of Stevens-Johnson Syndrome and Toxic Epidermal NecrolysisStevens-Johnson Syndrome (SJS) is an acute inflammatory vesiculobullous reaction of the skin and mucosa, including the ocular surface, oral cavity, and genitals.In SJS patients with extensive skin detachment and a poor prognosis, the condition is called toxic epidermal necrolysis (TEN).The treatment of both SJS and TEN is extremely difficult, and in fact, in our Ophthalmology Residency Program in the 1970s, we were instructed that there was currently no surgical or medical treatment for visual disturbance of patients afflicted with SJS/TEN.However, for corneal specialists, proper treatment of the devastating SJS/TEN-associated ocular surface disorder is vital.Depending on the drugs and infections involved, SJS/TEN is not always accompanied by severe ocular complications (SOC).However, the subgroup of SJS/TEN develops SOC due to corneal epithelial stem cell deficiency at the acute phase, thus resulting in severe visual impairment that prolongs into the chronic phase as well.The corneal opacity in SJS/TEN cases cannot be treated with regular corneal transplantation due to the ocular-surface stem cell deficiency and the proliferative changes caused by postoperative inflammation.Thus, when in search of a treatment for such devastating ocular surface disorders that cannot be treated with regular corneal transplantation, the use of several surgical strategies, such as keratoprosthesis (1), have been tried.In 2002, our team developed a new treatment method for cases with severe ocular surface disorders, known as Cultivated Oral Mucosal Epithelial Transplantation (COMET) (2).In SJS/TEN patients with severe visual impairment, treatment with COMET might provide a novel pathway toward the improvement of vision and overall quality of life.Recently, we developed a limbal-supported hard contact lens for use after the COMET procedure (3), which we found improved the patient's visual acuity.Hence, patients with SJS/TEN (which is an extremely rare disease) with SOC are now visiting the Department of Ophthalmology at Kyoto Prefectural University Hospital from all over Japan for treatment.Previously, research on SJS/TEN was primarily conducted by dermatologists, as they are the clinical specialists who treat SJS/TEN patients in the acute phase in many countries, including Japan, Taiwan, Korea, and Europe.However, ophthalmologists also treated chronic SJS/TEN patients with ocular sequelae, such as the visual impairment that exists throughout their lives.Hence, we ophthalmologists have now joined with dermatologists in a nationwide Japanese SJS/TEN Study Group to deepen our understanding on SJS/TEN patients with and without SOC, complications that can lead to ocular sequelae in the chronic phase.Quite surprisingly, this partnership has allowed us to more deeply understand SJS/TEN-related ocular sequelae, as well
Abstract licence: CC BY
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