Chronic Ocular Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: The Role of Systemic Immunomodulatory Therapy

Semin Ophthalmol. 2016;31(1-2):178-87. doi: 10.3109/08820538.2015.1114841.

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, but potentially blinding diseases that affect the skin and mucous membranes. Although the cutaneous manifestations tend to be self-limited and resolve without sequelae, the chronic ocular complications associated with SJS/TEN can persist despite local therapy. Poor understanding of the underlying pathophysiology and lack of a standardized clinical approach have resulted in a paucity of data in regards to suitable treatment options. Inflammatory cellular infiltration and elevated levels of ocular surface cytokines in the conjunctival specimens of affected patients give credence to an underlying immunogenic etiology. Furthermore, the presence of ongoing ocular surface inflammation and progressive conjunctival fibrosis in the absence of exogenous aggravating factors suggest a possible role for systemic immunomodulatory therapy (IMT). We review in detail the proposed immunogenesis underlying chronic ocular SJS/TEN and the possible utility of systemic IMT.

Keywords: Anti-inflammatory agents; autoimmune bullous diseases; chronic disease; drug therapy; eye diseases.

Publication types

  • Review

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use*
  • Chronic Disease
  • Conjunctival Diseases / drug therapy*
  • Conjunctival Diseases / etiology
  • Corneal Diseases / drug therapy*
  • Corneal Diseases / etiology
  • Eyelid Diseases / drug therapy*
  • Eyelid Diseases / etiology
  • Humans
  • Immunomodulation*
  • Stevens-Johnson Syndrome / complications*
  • Stevens-Johnson Syndrome / drug therapy*

Substances

  • Anti-Inflammatory Agents