The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy

The 2016 Bowman Lecture Conjunctival curses: scarring conjunctivitis 30 years on

“…A small subset of patients with these diseases develop autoantibody-positive or negative progressive conjunctival indistinguishable from MMP Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) with progressive scarring 33 Only a small subset of patients with these diseases develop autoantibody-positive or negative progressive conjunctival scarring similar to that in MMP which may continue from the acute episode or develop acutely years later…”

“…45 For the majority of patients there are no identifiable precipitating factors. However, in subsets of patients, including cases of ocular MMP following SJS/TEN 33,46 and topical glaucoma treatment, 34,47 it is possible that damage to the conjunctival basement membrane precipitates the disease by exposing basement membrane epitopes triggering a pathological autoimmune response to neoantigens. The latter mechanism is an alternative to the development of loss of tolerance to basement membrane antigens, that is described below, and thought to be the underlying mechanisms in most cases.…”

“…17 Alternatively, ALDH/RA might cause its effects through altered cellular energy metabolism via activation of TGFβ or as a consequence of the modulation of the transcription of metabolic genes. 33 The ALDH/RA metabolic pathway and these potential molecular mechanisms are described in Figure 10.…”

Clinical, Etiologic, and Histopathologic Features of Stevens-Johnson Syndrome During an 8-Year Period at Mayo Clinic

“…However, SJS-related ocular disease is not only the sequelae of the acute disease process but also can occur with a variable course several years after onset of SJS and is not always the direct result of conjunctival scarring. 27 In addition to developing clinical classification schemes based on the patterns and distribution of skin lesions, some authors have sought to differentiate EM from SJS and TEN by their histopathologic features. Rzany et al 6 examined biopsy specimens from patients with EM major, SJS, and TEN and found no differences in histologic features (eg, eosinophils) by etiology (ie, drug vs infection).…”

Amoxycillin and clavulanic acid induced Stevens-Johnson syndrome: a case report

“…11 Antigen presentation and production of tumor necrosis factor (TNF)-alpha by the local tissue dendrocytes results in the recruitment and augmentation of T-lymphocyte proliferation and enhances the cytotoxicity of the other immune effector cells. 12 A "killer effector molecule" has been identified that may play a role in the activation of cytotoxic lymphocytes. 13 The activated CD8+ lymphocytes, in turn, can induce epidermal cell apoptosis via several mechanisms, which include the release of granzyme B and perforin.…”