Ocular involvement in autoimmune bullous diseases

Clin Dermatol. 2023 Jul-Aug;41(4):481-490. doi: 10.1016/j.clindermatol.2023.08.007. Epub 2023 Aug 14.

Abstract

Autoimmune bullous diseases represent a heterogenous group of disorders caused by autoantibodies against adhesion molecules; the location of the target protein determines the level of cleft formation. The spectrum of ocular lesions in autoimmune bullous diseases can range from mild symptoms to severe involvement with sight impairment and even, in some cases, blindness. In pemphigus vulgaris, the prevalence of ocular involvement has been reported to be between 7% and 26%. The most common clinical sign of ocular pemphigus vulgaris is bilateral conjunctivitis with hyperemia. Ocular involvement also occurs in 41% to 70% of patients with paraneoplastic pemphigus. The main ocular manifestations are bilateral cicatrizing conjunctivitis with symblepharon formation, and shortening of the fornices. In mucous membrane pemphigoid, ocular involvement is seen in 61% to 70% of patients; the most frequent ocular finding is cicatricial conjunctivitis. Patients with autoimmune bullous diseases having common ocular involvement should be assessed by an ophthalmologist to avoid serious complications. Diagnostic procedures and treatment require multidisciplinary care based on the close cooperation between dermatologists and ophthalmologists.

MeSH terms

  • Autoimmune Diseases* / diagnosis
  • Conjunctivitis*
  • Humans
  • Pemphigoid, Benign Mucous Membrane* / complications
  • Pemphigoid, Benign Mucous Membrane* / diagnosis
  • Pemphigoid, Benign Mucous Membrane* / drug therapy
  • Pemphigus* / drug therapy
  • Skin Diseases, Vesiculobullous* / complications
  • Skin Diseases, Vesiculobullous* / diagnosis