BASICS

DESCRIPTION

• Inward rotation of the eyelid margin, allowing the eyelashes and skin to abrade the globe.

• Types:

– Congenital.

– Involutional (most common)(1)[C].

– Spastic.

– Cicatricial.

EPIDEMIOLOGY

Incidence

• Involutional—increases with age.

• Spastic—temporary secondary to trauma, ocular irritation, blepharospasm.

– Cicatricial—ocular cicatricial pemphigoid (OCP)—females > males

– Average age 60–70 years.

Prevalence

Cicatricial—OCP—1 in 15,000–20,000.

RISK FACTORS

Genetics

OCP–associated with HLA–DQB7^∗301.

PATHOPHYSIOLOGY

• 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.

ETIOLOGY

• 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

• Cicatricial.

• OCP

• Stevens–Johnson (erythema multiforme major).

• Trachoma.

• Trauma.

• Chemical burns.

• Chronic topical medications.

DIAGNOSIS

HISTORY

• Foreign body sensation.

• Tearing.

• Redness.

PHYSICAL EXAM

• 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

Lab

Initial lab tests

• None.

• OCP—ANA.

Follow-up & special considerations

• OCP—elevated soluble CD 8 glycoprotein, elevated tumor necrosis factor.

Diagnostic Procedures/Other

Cicatricial—conjunctival biopsy.

Pathological Findings

• Cicatricial–-ANA

• OCP—biopsy of conjunctiva for direct immunofluorescence studies or indirect immunofluorescence for presence of antibodies—positive in >80%.

DIFFERENTIAL DIAGNOSIS

• Epiblepharon.

• Trichiasis.

• Distichiasis.

TREATMENT

MEDICATION

• Topical lubrication.

– Frequent artificial tears, ophthalmic lubricating ointment.

ADDITIONAL TREATMENT

General Measures

Taping of lower eyelid skin to cheek.

Issues for Referral

• Decreased vision.

• Pain.

Additional Therapies

OCP—prednisone, dapsone, methotrexate, cyclophosphamide, CellCept, doxycycline (2)[C].

SURGERY/OTHER PROCEDURES

• 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].

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Ophthalmologist 1 week to monitor cornea.

Patient Monitoring

Warning signs: Pain, redness, tearing, photophobia.

PATIENT EDUCATION

Ocular cicatricial pemphigoid also be present in other mucous membranes.

PROGNOSIS

• Most patients require surgical repair.

• Ocular cicatricial pemphigoid is characterized by remissions and exacerbations and regardless of therapy can lead to loss of vision.

COMPLICATIONS

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