Secondary Corneal Amyloidosis Research Articles

Classification of Secondary Corneal Amyloidosis and Involvement of Lactoferrin

To classify secondary corneal amyloidosis (SCA) by its clinical appearance, to analyze the demographics of the patients, and to determine the involvement of lactoferrin. Retrospective, observational, noncomparative, multicenter study. Twenty-nine eyes of 29 patients diagnosed with SCA by corneal specialists at 9 ophthalmologic institutions in Japan were studied. The clinical appearance of SCA was determined by slit-lamp biomicroscopy and was classified into 3 types. The demographics of the patients, for example, age, gender, and the duration of the basic disease (trichiasis, keratoconus, and unknown), were determined for each clinical type. Surgically excised tissues were stained with Congo red and antilactoferrin antibody. The postoperative prognosis also was determined. Clinical appearance of the 3 types of SCA, along with the gender, age, and duration of the basic diseases were determined. Classification of SCA into 3 types based on clinical appearance found 21 cases with gelatinous drop-like dystrophy (GDLD)-like appearance (GDLD type), 3 cases with lattice corneal dystrophy (LCD)-like appearance (LCD type), and 5 cases with the combined type. Patients with the GDLD type were younger (average age: 40.9 years for the GDLD type, 74.3 years for the LCD type, and 46.8 years for the combined type), predominantly women (85.7% for the GDLD type, 33.3% for the LCD type, and 60% for the combined type), and had the basic disease over a longer time (average duration: 22.1 years for the GDLD type, 14.0 for the LCD type, and 11.4 for the combined type). The distribution of the basic diseases (trichiasis vs. keratoconus vs. unknown) was not significantly different for each type. Surgical treatments, for example, phototherapeutic keratectomy, lamellar keratoplasty, and simple keratectomy, resulted in a good resolution in all surgically treated cases. One subject dropped out of the study. Spontaneous resolution was seen in one subject after epilation of the cilia. Amorphous materials in the excised tissues showed positive staining results by Congo red and by antilactoferrin antibody. Secondary corneal amyloidosis can be classified into 3 clinical types based on its clinical appearance. Larger numbers of females and lactoferrin expression were seen in all 3 types. The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Tuméfaction cornéenne secondaire à un trichiasis

A rare lesion of secondary amyloidosis of the cornea caused by trichiasis is reported. A 33-year-old Asian woman presented with progressive decrease of visual acuity of the right eye evolving over several years, combined with the growth of a lower budding, gelatinous, pink corneal mass located next to an inferior lid trichiasis. The treatment consisted of eyelid surgery for trichiasis, followed by ablation of the corneal lesion by anterior keratectomy. After 14 months of follow-up, no recurrence was observed. Visual acuity is 9/10 P3 without correction. Pathological examination of the mass revealed secondary corneal amyloidosis, AL type (light chains), rarely reported in the literature (18 cases). No other organ involvement by amyloidosis was identified. Corneal amyloidosis is a rare complication of trichiasis. To avoid recurrence, this anomaly should be recognized as the cause and should be treated prior to removal of the corneal lesion.

What Is This Thing Called “Amyloidosis”?

Abstract:This article reviews our data on secondary localized corneal amyloidosis after chronic ocular inflammation including trichiasis and keratoconus. Previous findings have suggested that lactoferrin is a precursor protein in secondary corneal amyloidosis. The clinical finding that amyloidosis i

Secondary Amyloidosis in the Hydrops Lesion of a Patient With Pellucid Marginal Degeneration

To report a case of secondary corneal amyloidosis in a chronic hydrops lesion of a patient with pellucid marginal degeneration (PMD). Clinicopathologic case report with a review of the literature. A 63-year-old man with PMD developed acute hydrops in 1999, which never resolved and became an elevated, gelatinous lesion with peripheral neovascularization. Penetrating keratoplasty was performed in 2005, and the excised button was examined histopathologically. The excised button revealed substantial subepithelial acellular deposits consistent with amyloid in the area of the unresolved gelatinous lesion, overlying a disruption in Descemet membrane, confirming the diagnosis of secondary corneal amyloidosis. Secondary corneal amyloidosis is a corneal response to chronic injury and irritation that is not commonly reported and often overlooked. To the best of our knowledge, this is the first report of a dramatic secondary amyloidosis deposit in the chronic hydrops lesion of a patient with PMD.

Spheroidal Keratopathy Associated with Subepithelial Corneal Amyloidosis: A Clinicopathologic Case Report and a Proposed New Classification for Spheroidal Keratopathy

An unusual case of primary spheroidal degeneration associated with subepithelial corneal amyloidosis is described clinically and histopathologically, and a new classification for spheroidal keratopathy is proposed. The corneal button obtained by lamellar keratoplasty was examined by light and transmission electron microscopy. Amyloid deposits were evaluated further by means of the thioflavin T fluorescence technique, the potassium permanganate reaction, and immunohistochemical studies using antibodies against immunoglobulin light chains and proteins AA and AP. Spheroidal droplets were observed in basement membrane, Bowman's layer, and anterior stroma, and exhibited characteristic staining with Verhoeff's iron hematoxylin. Amyloid deposits were found beneath the epithelium, almost exclusively in the central area of the corneal button. The deposits showed no immunohistochemical staining with antibodies to amyloid proteins AA and AP or kappa and lambda light chains. The histochemical properties of the spheroidal droplets in this case were similar to those observed by other authors and suggested a sulfur-rich protein other than keratin. The combination of clinical and pathologic findings permitted the differential diagnosis of subepithelial amyloidosis and distinguished this condition from that of gelatinous drop-like dystrophy. To the authors' knowledge, this is the first description of primary spheroidal keratopathy associated with secondary subepithelial corneal amyloidosis in an eye that had not undergone previous keratoplasty.

Secondary Localized Amyloidosis in Interstitial Keratitis: Clinicopathologic Findings

Cases of secondary localized corneal amyloidosis have been described in various corneal and ocular inflammations but are believed to occur uncommonly. The authors examined histopathologic specimens from 33 consecutive patients with interstitial keratitis who underwent penetrating keratoplasty for visual rehabilitation. In 11 cases (33%), multiple fusiform deposits of amyloid were identified and confirmed in middle and deep corneal stroma using histochemical staining. Characteristic electron microscopic findings were present in two cases. These results indicate that secondary localized corneal amyloidosis occurs frequently in interstitial keratitis and displays a distinct pattern of deposition.

An Immunohistochemical Study of Gelsolin Immunoreactivity in Corneal Amyloidosis

A variant of the actin-modulating protein gelsolin has recently been identified as a component of the amyloid deposits in familial amyloidosis, Finnish type (Meretoja's syndrome), and has been demonstrated immunohistochemically in amyloid deposits in the cornea, and in the skin, kidney, heart, thyroid gland, salivary gland, and rectum of patients with this disease. With the use of immunohistochemistry involving an antibody against gelsolin, we examined a corneal specimen from a patient with Meretoja's syndrome and 14 corneal specimens with lattice dystrophy type I, atypical lattice dystrophy, polymorphic amyloid degeneration, primary familial amyloidosis, or secondary corneal amyloidosis. Our results showed the presence of a gelsolin-related protein either within or around corneal amyloid deposits in nine of the 15 specimens and markedly increased anti-gelsolin immunoreactivity of the corneal keratocytes in 13 of the 15 diseased corneas. These data indicated that the accumulation of gelsolin may be seen in various forms of amyloidosis and may not be confined to Meretoja's syndrome.

Secondary Localized Amyloidosis of the Cornea Associated with Tertiary Syphilis

Secondary corneal amyloidosis is a rare finding and only 17 cases have been reported in the literature. A case of unilateral secondary localized corneal amyloidosis is presented in a patient with bilateral interstitial keratitis and other stigmata of tertiary syphilis.