Familial Corneal Scarring: A New Dystrophy?

Hirst, Lawrence W.; Farmer, Evan R.; Green, W. Richard; Silver, Allan; Walsh, Frank B.

doi:10.1016/s0161-6420(84)34320-2

Cited by 12 publications

(5 citation statements)

References 15 publications

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“…It may be idiopathic, however, often associated with pre-existing chronic corneal diseases. 11,12 Today, an increasing number of cases lack a history of keratitis. The number of cases without evidence of keratoconjunctivitis were few at that time.…”

Section: Etiologic Factorsmentioning

confidence: 99%

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Salzmann's Nodular Degeneration of the Cornea

Das

Link²,

Seitz³

2005

Cornea

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Salzmann's nodular degeneration is a rare, noninflammatory, slowly progressive, degenerative condition. Bluish-white nodules raised above the surface of the cornea characterize it. It has usually developed in corneas with a history of phlyctenulosis, trachoma, vernal keratoconjunctivitis, measles, scarlet fever, and various other viral diseases. However, today the majority of cases have been seen without recognized previous keratitis. It is composed of dense irregularly arranged collagen tissue with hyalinization between epithelium and Bowman's layer or beyond. Manual removal, phototherapeutic keratectomy (PTK) with or without the use of topical mitomycin-C, lamellar or penetrating keratoplasty have been used in the treatment of this disease. Salzmann's nodular degeneration does not seem to consist of one clinical entity. In some cases, elevated and pannus-like tissue can be separated easily from the corneal surface leaving Bowman's layer almost untouched. In these eyes, subsequent PTK may be necessary to smooth the surface. Recurrences are rare in these eyes. In contrast, some eyes (often with major peripheral vascularization) are left with deep defects in Bowman's layer and superficial stroma after difficult mechanical removal of nodules. In these eyes, multiple masking/laser ablation procedures are mandatory to acquire a homogenous surface. In our experience, the required laser ablation depth is significantly greater and the best-corrected visual acuity to be expected is reduced in contrast to the eyes with easy removal of the nodules. In these eyes recurrences seem to occur more frequently after treatment. Of 35 eyes documented to have Salzmann's nodular degeneration during the last 15 years in our department, 22 needed PTK treatment. Visual acuity increased from 0.4 to 0.7 on average. As a routine, laser ablation should be combined with previous conventional removal of nodules and excessive pannus tissue. By doing so, lamellar and penetrating keratoplasty techniques are hardly ever required in those eyes.

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Section: Etiologic Factorsmentioning

confidence: 99%

“…11,26 Histologically the excised nodular areas show only disorganized collagenous scarring without specific identifying features. 11,26 Histologically the excised nodular areas show only disorganized collagenous scarring without specific identifying features.…”

Section: Hereditary Hypertrophic Scarringmentioning

confidence: 99%

Salzmann's Nodular Degeneration of the Cornea

Das

Link²,

Seitz³

2005

Cornea

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“…Corneal scarring is often due to infection or injury [3]. Other causes include corneal dystrophies, corneal ectasia, or inflammatory diseases, such as allergies or ocular rosacea [4,5].…”

Section: Introductionmentioning

confidence: 99%

Lycium barbarum Polysaccharide Suppresses Expression of Fibrotic Proteins in Primary Human Corneal Fibroblasts

Kwok

Wong

Shih

et al. 2020

JCM

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(1) Objective: To study the anti-fibrotic effects of Lycium barbarum polysaccharides (LBP) on corneal stromal fibroblasts and assess LBP’s effect on cell viability. (2) Methods: Primary human corneal keratocytes of passage 3 to 6 were used for all experiments. Cells are pretreated with LBP solution for 24 h and then transforming growth factor beta 1 (TGFβ1) for 48 h and collected for experiments. Fibrotic protein analysis was performed using immunofluorescence and Western blot. The effect of LBP on cell viability was assessed using the MTS assay. (3) Results: LBP significantly reduced the expression of fibrotic proteins, including α-SMA and extracellular matrix proteins (collagen type I and III). LBP significantly decreased the viability of myofibroblasts but not the fibroblasts. Conclusions: In this study, LBP was effective in the prevention of fibrosis gene expression. Further studies to assess the underlying mechanism and pharmacological properties will facilitate the formation of a topical LBP solution for in vivo studies.

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“…Salzmann's nodular degeneration is a rare condition characterised by elevated bluish-white to grey subepithelial nodules in the cornea. [1][2][3][4][5][6][7][8][9][10] The disease is seen more often in women and is bilateral in 80 per cent of patients. 4 Some cases have been reported with a possible autosomal inheritance pattern.…”

mentioning

confidence: 99%

“…4 Some cases have been reported with a possible autosomal inheritance pattern. 9 Salzmann's nodular degeneration is often a late sequela to previous ocular disease such as phlyctenular keratitis, vernal keratoconjunctivitis, trachoma, scarlet fever and measles. 8 It has also been associated with chronic uveitis and band keratopathy.…”

mentioning

confidence: 99%