E. Lee Stock

Absence of Normal Keratan Sulfate in the Blood of Patients With Macular Corneal Dystrophy

We measured levels of sulfated keratan sulfate in serum using a monoclonal antibody in an enzyme-linked immunosorbent assay. Sulfated keratan sulfate was not detected in the serum of 16 patients with macular corneal dystrophy, but was present at normal levels in 66 patients with other corneal diseases. There were no differences with respect to age, sex, and other ocular findings. This monoclonal antibody recognizes a sulfated carbohydrate epitope present in both corneal and skeletal keratan sulfate. Since most serum keratan sulfate is derived from the cartilages, the defect in keratan sulfate synthesis in macular corneal dystrophy may not be restricted to corneal cells. This assay should prove useful in the diagnosis of macular corneal dystrophy, particularly in children at risk before the appearance of opacification.

Macular corneal dystrophy Lack of keratan sulfate in serum and cornea

An ELISA assay using a monoclonal antibody (ET-4-A-4) that recognizes a sulfated carbohydrate epitope in both keratan sulfate type I (corneal) and type II (skeletal) was employed to quantify keratan sulfate in serum and corneal tissue from patients with macular corneal dystrophy (MCD). This assay disclosed significant quantities of keratan sulfate in the serum in 45 healthy individuals (251 +/- 78 ng/ml), and in 66 patients with various corneal diseases (273 +/- 101 ng/ml). In contrast keratan sulfate was not detected (less than 2 ng/ml) in the serum of 16 patients with histopathologically confirmed MCD. Keratan sulfate was also detected in extracts of normal corneas and corneal tissue with a variety of pathologic conditions, but was virtually absent in corneal tissue from five patients with MCD. In corneas with MCD the chondroitin sulfate/keratan sulfate ratio was considerably higher than that of all normal and pathologic corneas studied. Since keratan sulfate in the serum appears to be derived predominantly from the normal turnover of cartilage these studies strongly suggest that the defect in keratan sulfate synthesis in MCD is not restricted to corneal cells and that MCD is one manifestation of a systemic disorder of keratan sulfate. The cartilage changes, however, do not have clinical significance. Moreover, since keratan sulfate can be detected in the blood of newborns it should be possible to diagnose MCD prior to corneal opacification.

Conjunctival inflammation and amyloidosis in allergic granulomatosis and angiitis (Churg-Strauss syndrome).

A 37-year-old woman developed nodular, waxy conjunctival lesions associated with the exacerbation of Churg-Strauss syndrome. Conjunctival and skin biopsy specimens demonstrated a diffuse inflammatory process composed of eosinophils, plasma cells, lymphocytes, and focal collections of histiocytes. There were amyloid deposits in the conjunctiva and skin. To our knowledge, this is the first report of conjunctival changes and amyloidosis associated with the Churg-Strauss syndrome.

Trantas Dots and Limbal Inflammation Associated with Soft Contact Lens Wear

A 30-year-old man developed vernal-like limbal changes associated with soft contact lens wear including Trantas dots, limbal nodules, and diffuse limbitis. These findings add to the contention that the conjunctival changes found in giant papillary conjunctivitis and vernal conjunctivitis share a common immunopathogenesis.

Laser Photocoagulation of Feeder Vessels in Lipid Keratopathy

Argon laser photocoagulation was performed on rabbit eyes with lipid keratopathy. The lipid keratopathy was induced by rendering 30 rabbits hypercholesterolemic and inciting neovascularization by insertion of corneal sutures. The number of laser shots required to occlude corneal vessels increased with the suture residence time, whereas the number of limbal shots remained essentially constant. Corneal opacification and cholesterol content increased as a result of greater suture residence time and unexpectedly from the laser applications which were administered in a single treatment session.

The uptake of horseradish peroxidase by the conjunctival epithelium of the guinea-pig

It has been suggested that in immediate hypersensitivity of the guinea-pig conjunctiva, induced by the topical application of an antigen, the antigen is selectively taken up by the conjunctiva-associated lymphoid tissue. We tested this hypothesis by applying horseradish peroxidase (HRP) to the guinea-pig conjunctiva and studying its uptake by light- and electron microscopy. As early as 30 min after the application of the HRP, precipitate indicating the presence of HRP could be seen in the epithelial cells in membrane limited granules in the non-lymphoid and lymphoid epithelium. There was no selective uptake of HRP by the lymphoid-associated epithelial cells. We hypothesize that the epithelial cells of the conjunctiva, in contrast to those of the small intestine, phagocytose antigen and transfer it directly to the substantia propria of the conjunctiva for local immunologic processing.

Scleritis and Wegener's Granulomatosis in Children

We treated two children with scleritis (one unilateral, one bilateral), in whom Wegeners granulomatosis was diagnosed on the basis of pathologic changes in respiratory tract mucosa. Both patients were girls, 13 and 14 years of age, respectively. One patient had otitis media and a nodular scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate and microscopic hematuria. A biopsy of the sinus confirmed the diagnosis of Wegeners granulomatosis. The second patient had fever, arthralgias, a nonproductive cough, and bilateral scleritis. Laboratory test results demonstrated an increased erythrocyte sedimentation rate, positive test results for rheumatoid factor, and bilateral pulmonary nodules on chest x-ray. Open-lung biopsy confirmed the diagnosis of Wegeners granulomatosis. Both patients responded well to treatment with a combination of prednisone and cyclophosphamide.

Stromal keratitis complicating anterior membrane dystrophy.

Recurrent erosion of the cornea has been well documented in patients with nontraumatic anterior membrane dystrophies of various types. We examined five patients who, in addition to an erosion, developed stromal keratitis. Three of these patients were subjected to a complete microbiologic workup, but the lesions were all sterile. The lesions healed with conservative treatment of patching and, in some cases, a soft contact lens. Stromal keratitis should be recognized as a complication of the non-traumatic recurrent erosion syndrome, which in turn is frequently associated with anterior membrane dystrophy. The finding of such anterior membrane changes in either eye will lead to the correct diagnosis and treatment of the affected eye.

Cutaneous basophil hypersensitivity in the guinea pig conjunctiva

Cutaneous basophil hypersensitivity (CBH) is an immunologic reaction extensively studied in the guinea pig. We elicited this reaction in the conjunctiva by systemically sensitizing guinea pigs to ovalbumin in incomplete Freunds adjuvant. Skin tests showed basophils in the skin one week after sensitization when challenged with ovalbumin. However, the conjunctiva showed an eosinophilic reaction with few basophils. This is compatible with other extracutaneous manifestation of the CBH reaction.