Bartly J. Mondino

Ocular Cicatricial Pemphigoid

The characteristic feature of ocular cicatricial pemphigoid (OCP) is progressive shrinkage of the conjunctiva. In our series of 78 patients with OCP, 21% had cutaneous involvement and 50% had involvement of the oral mucosa. Immunoglobulins and the third component of complement are found bound to the conjuctival epithelium and basement membrane of patients with OCP. Circulating antibodies which bind to the conjunctival and corneal epithelium but not to the conjunctival basement membrane have also been demonstrated. OCP is associated with an increased prevalence of HLA-B12. The lids and conjunctiva of patients with OCP demonstrate an increased incidence of potential pathogens when compared with age- and sex-matched controls. When followed for a period averaging 22 months, the majority of patients not treated with systemic immunosuppressives or topical corticosteroids progress. However, OCP has a variable course because there were patients in all stages who did not progress. The acute manifestations of OCP may cause rapid shrinkage of the conjunctiva and may be suppressed systemic corticosteroids.

The Acute Manifestations of Ocular Cicatricial Pemphigoid: Diagnosis and Treatment

Seven patients with ocular cicatricial pemphigoid displayed acute inflammatory activity that could not be attributed to secondary bacterial infections, trichiasis, or lagophthalmos secondary to symblepharon. This acute inflammatory activity was manifested either as a localized conjunctival mound that was ulcerated and intensely hyperemic or as diffuse and intense conjunctival hyperemia and chemosis. Acute disease activity developed shortly after conjunctival biopsy in three patients and appeared spontaneously in the other four patients. Conjunctival biopsy specmens disclosed a heavy infiltrate of polymorphonuclear leucocytes within and beneath the conjunctival epithelium in addition to the chronic inflammatory cells typically found in this condition. The acute manifestations of ocular cicatricial pemphigoid cause rapid shrinkage and scarring of the conjunctiva. Systemic corticosteroids suppressed the acute disease activity and prevented additional scarring in all five patients treated.

Allergic and toxic reactions in soft contact lens wearers

The allergic and toxic effects of preservatives found in soft contact lens solutions are reviewed. Thimerosal, a preservative commonly found in soft contact lens solutions, may cause ocular delayed hypersensitivity. Patients with delayed hypersensitivity to thimerosal may develop conjunctival hyperemia, corneal infiltrates and intolerance to lens wear with the use of soft contact lens solutions containing thimerosal. Delayed hypersensitivity to thimerosal can be demonstrated by an occlusive patch test or intradermal injection. Discontinuation of chemical disinfection with substitution of thermal disinfection using unit-dose, non-preserved saline causes resolution of signs and symptoms. Another cause of lens wear intolerance is contact lens-associated giant papillary conjunctivitis. Protein deposits on the lens may act as antigens and initiate an allergic condition which disappears when the lens is discontinued. Alternatively, the giant papillae may be the result of mechanical irritation induced by the contact lenses. The chemical preservatives that are found in soft contact lens solutions cause epithelial toxicity when applied to isolated rabbit corneas. However, corneal toxicity toxicity in a clinical setting has not been demonstrated.

Corneal Toxicity with Systemic Cytarabine

Three patients with leukemia developed corneal toxicity while receiving high doses (3 g/m2) of systemic cytarabine. Symptoms began five to seven days after initiation of treatment with high doses of systemic cytarabine and consisted of ocular pain, tearing, foreign-body sensation, photophobia, and blurred vision. All three patients developed bilateral conjunctival hyperemia and fine corneal epithelial opacities and refractile microcysts that were more numerous in the central than in the peripheral cornea. The symptoms disappeared without treatment in approximately one week. The corneal changes we observed with high doses of systemic cytarabine resembled descriptions of corneal toxicity from topical cytarabine and were probably secondary to inhibition of DNA synthesis in the corneal epithelium.

Autoimmune Phenomenon in Mooren's Ulcer

Direct immunofluorescent techniques demonstrated immunoglobulins localized to the conjunctival epithelium in three cases of Moorens ulcer. In addition, complement was found in association with the immunoglobulins in the two active cases. Circulating antibodies to the conjunctival and corneal epithelium were demonstrated by indirect immunofluorescent techniques in all three patients. Further immunological examinations of the three cases failed to demonstrate other significant immune alterations.

Cellular Immunity in Mooren's Ulcer

Seven patients with the clinical diagnosis of Moorens ulcer underwent macrophage migration inhibition factor testing for evidence of cellular immunity to corneal antigen. Six of seven patients demonstrated positive macrophage migration inhibition in response to corneal antigen, thus suggesting that cell-mediated immunity may play a role in the cause of Moorens ulcer. One of these patients had a negative macrophage migration inhibition factor when the disease was inactive, but developed a positive response in association with a homograft rejection episode.

Autoimmune phenomena of the external eye.

The immunologic status of patients with ocular pemphigoid, Moorens ulcer, chronic herpetic keratitis, and staphylococcal peripheral corneal ulcers was studied. Although tissue-fixed and circulating antibodies to the conjunctival epithelium were found in all groups, patients with Moorens ulcer demonstrated these findings most consistently. Immunoglobulins bound to the conjunctival basement membrane were found not only in ocular pemphigoid but also in patients with Moorens and staphylococcal ulcers. Approximately one half of the patients with ocular pemphigoid and Moorens ulcer demonstrated elevations in serum IgA levels. An increased prevalence of HLA-B12 was found in ocular pemphigoid.

Peripheral Corneal Ulcers, Conjunctival Ulcers, and Scleritis after Cataract Surgery

After undergoing uneventful cataract extractions, four patients developed an inflammatory reaction that included scleritis and peripheral corneal infiltrates and ulcers. Two of these patients also developed conjunctival ulcers. The inflammatory reaction in all four patients responded to topical corticosteroids.

Primary Familial Amyloidosis of the Cornea

The stromal deposits of a failed corneal graft from a patient with primary familial amyloidosis of the cornea stained positively with Congo red and showed green birefringence with polarized light. These deposits contained protein AP but not protein AA, immunoglobulins, light chains, or prealbumin. The amyloid deposits of primary familial amyloidosis of the cornea differ from those in lattice corneal dystrophy which contain both protein AA and protein AP.

Peripheral Corneal Ulcers with Herpes Zoster Ophthalmicus

Four patients with herpes zoster ophthalmicus developed peripheral corneal ulcers with steep central edges. An anterior uveitis was associated with all cases. One patient developed bilateral corneal ulcers that were typical of Moorens ulcer and eventually destroyed both corneas. The other three cases were unilateral and were not relentlessly progressive.