Microcystic Dystrophy of the Cornea

Cogan, David G.; Kuwabara, Toichiro; Donaldson, David I.; Collins, Eleanor M.

doi:10.1001/archopht.1974.01010010484004

Cited by 64 publications

(29 citation statements)

References 7 publications

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“…Subepithelial granular material in Cogan's microcystic dystrophy, which is morphologically similar to that seen in the present study of bleb dystrophy, has been described as 'basement membrane-like' by Cogan et al (1974). The present study does not provide evidence for this assertion.…”

Section: Discussioncontrasting

confidence: 54%

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Bleb dystrophy of the cornea: histochemistry and ultrastructure.

Dark¹

1977

British Journal of Ophthalmology

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SUMMARY Corneal epithelial biopsies from two patients affected with asymptomatic bleb dystrophy were examined by light and electron microscopy. The basis of this disorder appears to be the deposition of a neutral mucopolysaccharide-protein complex as a continuous layer between the basement membrane and Bowman's layer. This material, although homogeneous in light microscopy, has a fine granular ultrastructure. It is friable, and in view of the apparent integrity of the basement membrane/hemidesmosome system it is suggested that the recurrent epithelial erosions which can occur in this disorder result from shearing of this layer. Fissures in the bleb material contain cells which may play a role in its degradation.A recently described group of superficial corneal disorders includes the fingerprint, net, bleb (Bron and Brown, 1971) and microcystic dystrophies (Cogan et al., 1964). Brown and Bron (1976) have shown that these dystrophies may be associated with recurrent epithelial breakdown. Thus, they were present in 59% of their patients with the recurrent erosion syndrome. Conversely the same authors (1971) had noted previously that 38% of patients with these superficial dystrophies were also afflicted with symptoms of recurrent epithelial dehiscence. These studies naturally suggest that this group of superficial corneal dystrophies is an important precursor of defective epithelial adherence.The common ultrastructural basis of these dis- Examination showed corrected vision OD=20/80, OS=20/20. A dendritic ulcer of the herpes simplex type was present in the epithelium of the right cornea. Profuse subepithelial blebs were present in clusters in the parapupillary zones of both corneae. Their distribution in the left cornea is illustrated in Fig. 1. The blebs, which varied in size, were on the average about 50 ,u wide. With the direct ophthalmoscope the blebs had an orange-skin appearance when set against the fundal reflex. Viewed by indirect slit-lamp illumination from the iris the blebs appeared as transparent bubbles with an unreversed lighting effect (Fig. 2), a phenomenon which, as noted, indicates a refractive index higher than that of the surrounding tissue. In focal illumination the blebs were seen as black dots set in a relucent background. There were no demarcation or other lines in either cornea. In the left eye it was possible to determine that there was no interference with the precorneal tear film and that

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Section: Discussioncontrasting

confidence: 54%

“…The common ultrastructural basis of these disorders (Cogan et al, 1974;Rodrigues et al, 1974;Broderick et al, 1974) Examination showed corrected vision OD=20/80, OS=20/20. A dendritic ulcer of the herpes simplex type was present in the epithelium of the right cornea.…”

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confidence: 99%

Bleb dystrophy of the cornea: histochemistry and ultrastructure.

Dark¹

1977

British Journal of Ophthalmology

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“…Two types of corneal epithelial dystrophy have been described: epithelial basement membrane (map-dot-fingerprint, Cogan) dystrophy [Cogan et al, 1964;Cogan et al, 1974;Rodrigues et a1 1974;Brodrick et al, 19741 and Meesman's juvenile epithelial dystrophy [Kuwabara and Ciccarelli, 1964;Burns, 19681. Both of these conditions are inherited in an autosomal dominant pattern and neither is reported in association with other defects.…”

Section: Commentsmentioning

confidence: 99%

Corneal changes, hyperkeratosis, short stature, brachydactyly, and premature birth: A new autosomal dominant syndrome

Stern¹,

Lubinsky²,

Durrie³

et al. 1984

Am. J. Med. Genet.

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We report on an autosomal dominant syndrome consisting of unique corneal epithelial changes, diffuse palmoplantar hyperkeratosis, distal onycholysis, brachydactyly, short stature, premature birth, and dental problems. This condition has been present in seven persons in three generations of one family. Corneal biopsies demonstrate mild dysplastic changes in the epithelium. Skin biopsies show hyperkeratosis and acanthosis. In both eye and skin specimens, results of stains for polysaccharides, amyloid, and tyrosine were unremarkable. Roentgenograms of the hands show short distal phalanges, short 4th metacarpals, and constriction of the heads of some of the metacarpals. In three of four affected relatives, a variable medullary narrowness is seen. In mode of inheritance, clinical appearance, and/or associated defects. This syndrome appears to differ from previously reported conditions that include palmoplantar hyperkeratosis and/or corneal changes.

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“…E ditor ,—Map-dot epithelial dystrophy, also called Cogan’s microcystic dystrophy,1 is a well known and relatively frequent clinical entity. The microscopic basis for the biomicroscopic appearance of the typical superficial corneal opacities and epithelial erosions are intraepithelial cysts and a multilaminated or bilaminated aberrant epithelial basement membrane 1-5…”

mentioning

confidence: 99%