Ocular cicatricial pemphigoid (Review)

摘要: Ocular cicatricial pemphigoid is a particular form of mucous membrane and it characterized by chronic bilateral conjunctivitis with relapsing-remitting periods. Without therapy 75% the cases develop visual loss due to major ocular complications (e.g. severe dry-eye syndrome, corneal erosions, keratinization, entropion, symblepharon). Pathogenesis remains uncertain probably linked an autoimmune type II hypersensitivity response in patients genetic predisposition exposure different environmental triggers. With worldwide distribution, no racial predilection estimated incidence that largely varies from 1/10,000-1/60,000, predominantly affects women aged ~60 years. Conjunctival biopsy direct immunofluorescence gold standard diagnosis confirmation, but up 40% have negative result does not rule out diagnosis. The skin many other membranes oral, trachea, esophagus, pharynx, larynx, urethra, vagina anus) may be involved. disease grading relies on Foster staging system (based clinical signs) Mondino Brown inferior fornix depth loss). differential includes atopy, allergies, trauma, chemical burns, radiation, neoplasia, infectious, inflammatory etiologies. main goals treatment are stop progression, relieve symptoms prevent complications. long-term systemic 90% can efficiently controlled. While Dapsone first-line mild moderate without G6PD deficiency, more require immunosuppressant azathioprine, mycophenolate mofetil, methotrexate or cyclosporine. Cyclophosphamide, biologics (etanercept rituximab) intravenous immunoglobulin usually reserved for recalcitrant unsatisfactory results conventional therapy. Dry eye syndrome requires constant lubricating medication topical steroids, cyclosporine-A tacrolimus. Surgery should planed only quiescent phase as minor conjunctival trauma significantly worsen disease.