Systemic and Local Management at the Onset of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis with Ocular Complications

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal necrolysis (TEN) are recognized as two of the most devastating ocular surface disorders and are extremely difficult to treat both at the acute and later stages. Massive inflammation on the ocular surface at the acute stage often is uncontrollable. Even after the acute-stage impairments subside, ocular complications such as serious visual impairment with dry eye and keratinization remain at the later stage. Recently, we reported in our retrospective analysis that visual outcomes at the later stage were significantly better in the group receiving topical steroids at the acute stage compared with the no-treatment group ( P < .00001). Furthermore, using a prospective study, we confirmed the therapeutic importance of steroid therapy at disease onset for reducing the degree of ocular complications. Therefore, we read with great interest the editorial entitled “Acute Management of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis to Minimize Ocular Sequelae” by Scheffer C. G. Tseng, in which the author states that “systemic high-dose of glucocorticoids may heighten the risk of inciting (existing) infections in the wake of managing patients with life-threatening diseases.” In this editorial, the author suggests that amniotic membrane transplantation (AMT) may be a better strategy for reducing immunoreactions on the ocular surface. However, in some aspects, we are not in agreement with the author’s comments.

First, the author pointed out the difficulties of distinguishing SJS and TEN from erythema multiforme, which is known to be a high underlying cause of herpes simplex virus infection. However, a biopsy of the skin lesion can reveal the histologically characteristic appearance of SJS and TEN such as apoptosis, necrosis, and epidermal detachment. Moreover, bilateral eye symptoms before or concurrently with the onset of skin eruptions and oral and nail involvement help the clinician to recognize SJS and TEN with ocular involvement. Thus, we can distinguish most cases of SJS and TEN with ocular involvement from erythema multiforme at disease onset. As we mentioned in our report, the possibility of common viral and microbial infections can be excluded by polymerase chain reaction and microbial cultures of the tissue and the blood. The delay of the diagnosis itself increases the risk of secondary infection at erosive sites.

Second, our recent study proved that the patients with SJS and TEN are severely affected systemically and on the ocular surface because of a cytokine storm that occurs at the acute phase (unpublished data). Thus, a systemic therapeutic method such as steroid pulse treatment is essential for subsiding this cytokine storm, controlling the ocular surface inflammation in an indirect manner. Although we understand that AMT may be effective in diminishing ocular surface inflammation and immunoreactions, AMT alone cannot manage systemic inflammation. Furthermore, details of the general management of SJS and TEN by AMT are uncertain in most published reports. In addition, in the patients with poor general conditions, surgical treatment is very difficult and general anesthesia is impossible.

In conclusion, steroid pulse therapy and topical betamethasone should be considered to prevent corneal epithelial stem cell loss in the limbal region and cicatricial changes, and AMT may prove to be a beneficial addition to this therapy.

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Jan 17, 2017 | Posted by in OPHTHALMOLOGY | Comments Off on Systemic and Local Management at the Onset of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis with Ocular Complications
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