PL3 Stevens Johnson syndrome and mucous membrane pemphigoid: ocular manifestations and their management

Abstract

Stevens Johnson Syndrome (SJS), its severe form Toxic Epidermal Necrolysis (TEN), and mucous membrane pemphigoid (MMP) are the major autoimmune causes of conjunctival scarring. Between them these diseases present some of the most difficult management problems in ophthalmology. SJS/TEN develop conjunctival disease as part of the acute generalised mucosal involvement. The conjunctivitis varies from a papillary reaction with watery discharge to a membranous conjunctivitis with sloughing of the conjunctival epithelium. Corneal epithelial defects are common and may progress to corneal ulceration with or without bacterial superinfection. Although the morbidity may be due to the acute corneal complications it more usually results from the progressive effects of the conjunctival scarring, the secondary ocular surface disease, infections & treatment toxicity that occur later. Some cases of SJS/TEN develop the following late inflammatory diseases: recurrent conjunctival inflammation without scarring (recurrent SJS), inflammation with cicatrisation like ocular MMP (SJS‐MMP) and scleritis. On the other hand ocular MMP (MMPO) presents with acute conjunctivitis and limbitis in only 10% of cases; the remainder present with subacute or low grade chronic inflammation and progressive scarring. Progression in MMPO is due to the same factors that cause the morbidity in SJS/TEN (conjunctival scarring, the secondary ocular surface disease, infections & treatment toxicity) coupled with the effects of the progressive conjunctival inflammation and scarring that is a feature of all cases of MMPO, but occurs in a few cases of SJS/TEN with ocular involvement. Successful management of both SJS/TEN and CP demands identification of the components of the disorder due to (i) surface disease, (ii) treatment toxicity (iii) immune mediated inflammation and (iv) infection. These problems lead to a dry eye, surface failure and corneal blindness. All these components of the problem require prompt treatment to prevent progression to surface failure because of the poor prognosis for rehabilitation, by corneal graft surgery and/or surface reconstruction, in this group of patients.