Autoimmune Bullous Diseases

Abstract

The ocular manifestations of autoimmune bullous diseases are common and potentially sight-threatening. Major ophthalmic involvement is most commonly seen in mucous membrane pemphigoid (cicatricial pemphigoid), epidermolysis bullosa acquisita, linear IgA bullous disease, pemphigus vulgaris and paraneoplastic pemphigus. The main pathological process is related to autoimmune-induced conjunctival inflammation with consequent lid and corneal pathology, which may eventually result in permanent visual loss. Ocular involvement can be asymptomatic. Early detection is aided by careful attention to symptoms and signs of early ophthalmic disease. Ocular disease can be difficult to treat and management usually involves systemic therapy with immunomodulators to control inflammation and prevent progression to irreversible blindness, as well as surgical intervention in advanced disease. Recent advances in treatment, including methotrexate, mycophenolate mofetil, monoclonal antibodies and topical tacrolimus therapies, have led to promising results.

Paraneoplastic Pemphigus: A Paraneoplastic Autoimmune Multiorgan Syndrome or Autoimmune Multiorganopathy?

“…Bronchilitis obliterans is a common feature seen in 30–40% of PNP cases [1], while the involvement of mucous membranes of esophagus, stomach, duodenum, and colon is infrequent in contrast to their involvement in pemphigus. This combined with reports of glomerulonephritis, neurological involvement, and ocular involvement (conjunctivitis, symblepharon, and corneal scarring) in PNP are further suggestive of this being a disease of multiorgan system [19, 20]. We tend to agree with Czernik et al [6] that “paraneoplastic autoimmune multiorgan syndrome (PAMS)” is more inclusive nomenclature.…”

Amniotic Membrane Graft in Ocular Cicatricial Pemphigus: A More Conservative Approach

“…Different factors can be involved to induce corneal ulcers in the setting of OCP, such as stem cells defects, limbitis, trichiasis, entropion, symblepharon, blepharitis, and conjunctival keratinization [5]. The initial management of a persisting corneal defect after excluding infectious etiologies by appropriate microbiological assessment could be lash ablation by cryotherapy, laser thermoablation or electrolysis [7], non-preserved ointment as a lubricant; therapeutic contact lens [8] and even autologous serum drops [9].…”

Ocular Involvement and Blindness Secondary to Linear IgA Dermatosis

“…In this case there were no previous medications or vaccinations associated with drug-induced linear IgA dermatosis [8, 9]. …”

“…Despite that, theoretically, end-stage disease is rare in LAD [9], and even though we treated the patient with systemic immunosuppression agents and surgical procedures the ocular response was very poor. There are previous reports of corneal perforations [1, 4] and the use of corneal transplantation and limbal stem cell grafts [4], but in those cases the outcomes were also unsatisfactory.…”

“…Even though up to 50% of the patients with LAD presented ocular involvement [1, 2, 6, 9, 11], there is scanty literature in the ophthalmologic field [2, 4, 11]. …”