Sutureless Amniotic Membrane (ProKera®) and Intravenous Immunoglobulin in the Management of Ocular Complications of Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis Overlap.

PURPOSE

To report a case of Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, with severe acute ocular manifestations successfully managed with sutureless Amniotic Membrane device ProKera (Bio-Tissue, Inc., Miami, FL) and topical steroids, followed by late complications that were successfully managed with intravenous immunoglobulin (IVIG) therapy.

OBSERVATIONS

A 24-year-old lady, known case of epilepsy, admitted to the burn unit with SJS-TEN overlap attributed to a recent change of her anti-convulsant therapy, with severe ocular manifestations, inability to open both eyes, and poor visual acuity. Early management included intensive topical steroids and lubrication, in addition to the application of a ProKera device. Despite achieving full epithelialization within two weeks with the improvement of ocular manifestations, the patient presented three weeks later with recurrence of conjunctival epithelial defects, partial ankyloblepharon, and severely dry corneas. These late sequelae were managed with bandage contact lens (BCL) application, intensive topical steroid, and lubrication in addition to IVIG therapy. After six cycles of IVIG therapy, ocular manifestations improved significantly and the patient achieved uncorrected visual acuity of 6/9 in both eyes. Conclusion and importance: Existing evidence suggests that the use of IVIG in combination with systemic steroids in the early phase of SJS-TEN can reduce mortality, without affecting the final visual outcome in patients with ocular manifestations. This case highlights the possible role of IVIG therapy alone - without systemic steroids - in managing and preventing long-term ocular complications of SJS-TEN.