• Inward rotation of the eyelid margin, allowing the eyelashes and skin to abrade the globe.
– Involutional (most common)(1)[C].
• Involutional—increases with age.
• Spastic—temporary secondary to trauma, ocular irritation, blepharospasm.
– Cicatricial—ocular cicatricial pemphigoid (OCP)—females > males
– Average age 60–70 years.
Cicatricial—OCP—1 in 15,000–20,000.
OCP–associated with HLA–DQB7∗301.
• Increased orbicularis muscle tone and override secondary to loss of adhesions to orbital septum.
• Thinning/dehiscence of lower lid retractors.
• Horizontal laxity.
• Atrophied tarsal plates.
• Dipivefrin, practolol, pilocarpine, timolol, echothiophate, iodide, epinephrine eye drops.
• OCP—a systemic autoimmune pemphigoid disorder that has ocular and nonocular manifestations.
– OCP thought to be a type 2 hypersensitivity reaction, genetically predisposed.
COMMONLY ASSOCIATED CONDITIONS
• Stevens–Johnson (erythema multiforme major).
• Chemical burns.
• Chronic topical medications.
• Foreign body sensation.
• Redness of conjunctiva.
• Inverted eyelid margin.
• Thickened muscle band of orbicularis muscle.
• Lashes abrading globe.
• Superficial punctuate keratopathy, corneal abrasion.
• Corneal scarring, thinning, or ulceration, vascularization in severe cases.
• Symblepharon—cicatricial—linear scar/fold of palpebral conjunctiva to bulbar conjunctiva, shortening of fornix.
– Scarring of upper eyelid tarsal conjunctiva on eyelid eversion (trachoma).
DIAGNOSTIC TESTS & INTERPRETATION
Initial lab tests
Follow-up & special considerations
• OCP—elevated soluble CD 8 glycoprotein, elevated tumor necrosis factor.
• OCP—biopsy of conjunctiva for direct immunofluorescence studies or indirect immunofluorescence for presence of antibodies—positive in >80%.
• Topical lubrication.
– Frequent artificial tears, ophthalmic lubricating ointment.
Taping of lower eyelid skin to cheek.
Issues for Referral
• Decreased vision.
OCP—prednisone, dapsone, methotrexate, cyclophosphamide, CellCept, doxycycline (2)[C].
• Surgical treatment is almost always necessary, timing depends on corneal health.
• Suture repair—may be done bedside—highest recurrence rate.
• Outpatient eyelid surgery, including horizontal tightening, lower lid retractor tightening, orbicularis muscle stabilization or marginal rotation (3)[C].
Ophthalmologist 1 week to monitor cornea.
Warning signs: Pain, redness, tearing, photophobia.
Ocular cicatricial pemphigoid also be present in other mucous membranes.
• Most patients require surgical repair.
• Ocular cicatricial pemphigoid is characterized by remissions and exacerbations and regardless of therapy can lead to loss of vision.
Corneal abrasion, ulcer, scarring, perforation, loss of eye.