A 56 year old female presented to our clinic from referral from their eye doctor for “trichiasis”. The patient had suffered from foreign body sensation for several months and irritation of the eye. After unsuccessful treatment from their primary care doctor with topical antibiotics for pink eye they saw their optometrist who located aberrant lashes under slit lamp exam.
The patient’s history was significant for Lasik in the early 2000s but no other surgeries. She also had a history of Thyroid hyperactivity that was now controlled on medication.
On examination it was revealed the that patient had a cicatricial entropion with lashes that were angled towards the eye for approximately ¼ of the eyelid. The patient also has frank keratitis from the lashes. She admitted that in the past a previous eye doctor had attempted to pulled the lashes but gave up after it seemed that was making the eyelashes “worse over time”
A cicatricial entropion repair was performed with biopsy. During this procedure a full thickness portion of the eyelid was sent to biopsy and the lashes were rotated away from the eye.
The biopsy came back positive for Ocular Cicatricial Pemphigoid. The patient was immediately started on dapsone and sent to rheumatology for workup and management.
Ocular Cicatricial Pemphigoid
Ocular Cicatricial Pemphigoid (OCP) is a systemic immune disease that affects the mucous membranes. It is a disease primarily characterized by overactivation of T cells. It can affect he mouth, larynx trachea, esophagus, vagina and urethra.
The disease primarily occurs under a “two hit hypothesis”. Some patients have a genetic predisposition and then are exposed to offending agents. Topical glaucoma drops have been implicated in some cases of OCP.
The epidemiology of the disease shows that the disease affects women 3:1 over men and commonly occurs in the 50s to 60s. There are no race predisposition.
Patients commonly present with redness, irritation and foreign body sensation initially though quickly they may present with ankyloblepharon, symblepharon and corneal scarring. Keratinization of the cornea and conjunctiva can occur and cicatricial entropion can occur from advanced scarring of the eyelid.
Diagnosis is via a conjunctiva biopsy and immunoperioxidase staining revealing IgA, IgG, C4, C3. The sensitivity of a biopsy is only 83% so false negatives are possible.
Treatment for OCP includes dapsone, methotrexate and steroids orally. Topical drops are used as adjuvant therapy but never alone in treatment. Because the side effect profile of these systemic medications which include hemolysis for dapson and nephrotoxicity and hepatoxicity for methotrexate, these medications are often given by a rheumatologist in conjunction with an eye doctor.