Progressive, refractory keratoconjunctivitis associated with lichen planus.
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Biomedical subjects
Publications and source records attributed to J G Heathcote.
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An intralenticular foreign body with associated squamous epithelium was found incidentally at cataract extraction in a 61-year-old man 23 years after a penetrating injury. The squamous epithelium produced no adverse effects. To our knowledge this is the first report of persistence of surface squamous epithelium within the lens.
Incontinentia pigmenti (IP) (Bloch-Sulzberger syndrome) is an X-linked dominant condition usually fatal in males. Shortly after birth affected girls present with a rash followed by pigmentary changes. Abnormalities of the central nervous system and dentition are often present, and ophthalmic problems develop in approximately one-third of patients. The authors present the pathological findings in the skin and eye in a young woman who required enucleation as a result of long-standing retinal detachment and closed-angle glaucoma. A review of published ocular pathology reports indicates that retinal detachment and a fibrovascular retrolental membrane are the commonest intraocular abnormalities in IP. Changes in the retinal pigment epithelium are also prominent, although whether these are primary or secondary remains to be established.
We present a case of partial duplication of the short arm of chromosome 2 (karyotype 46,XX, dup [2p21-2p25]) in a newborn girl. The infant was born at 41 weeks of gestation and died approximately 3 hours after birth. At autopsy the characteristic dysmorphic features (hypertelorism, high, prominent forehead, micrognathia and low-set, malformed ears) and numerous other congenital malformations were observed. Bilateral microcornea with opacities was noted. Histopathological examination of the eyes showed dysgenesis of Bowman's membrane, with glycosaminoglycan deposition and a series of structural anomalies that form part of the anterior segment mesenchymal dysgenesis (ASMD) complex. To our knowledge this is the first case of partial duplication of the short arm of chromosome 2 in which the ocular histopathological features have been studied. Our case provides a further example of the association of ASMD with a chromosomal abnormality.
Collagen shields are a potential delivery system for antifibroblast drugs such as 5-fluorouracil after filtration surgery. To determine whether collagen shields produce histologic evidence of inflammation when implanted subconjunctivally, shields were implanted into four rabbit eyes and six guinea pig eyes and retained for 7 or 14 days. Two rabbit eyes and two guinea pig eyes served as controls. Seven days after implantation in the rabbit eyes foreign-body giant cells were present at the surface of the shield, and early deposition of connective tissue was evident around the shield. The inflammatory response at 14 days was similar but more intense. In the guinea pig eyes the collagen shields induced substantially less inflammation, and there was marked shield degradation at 14 days. The results suggest that the inflammatory response in rabbits may be species specific and that collagen shields may be of value as a drug-delivery system for antifibroblast drugs in other species.
A middle-aged woman developed unilateral, painless proptosis that increased slowly over 1 year. A clinical diagnosis of pleomorphic adenoma of the lacrimal gland was supported by computed tomographic scanning and the tumor was excised. On histological examination the tumor proved to be a benign, myxoid myoepithelioma of spindle-cell type. Although occasionally seen in the salivary glands, to our knowledge, this tumor has not previously been described in the lacrimal gland.
Current classifications of salivary gland tumors separate mucoepidermoid carcinoma from other neoplasms on the basis of a number of histological features, in particular the lack of participation of neoplastic myoepithelial cells. However, ultrastructural examination of low- and intermediate-grade mucoepidermoid carcinomas and pleomorphic adenomas reveals many common organizational and cellular features. Of prime importance is the relationship of intermediate cells to the luminal cells in mucoepidermoid carcinomas, which is remarkably similar to that seen between modified myoepithelial cells and luminal cells in pleomorphic adenomas. The results suggest that intermediate cells of mucoepidermoid carcinoma are the counterpart of the modified myoepithelial cells of pleomorphic adenoma. The generally accepted hypothesis that the former tumor develops from an excretory duct reserve cell, while the latter originates from an intercalated duct stem cell does not seem to be valid; pleomorphic adenoma and mucoepidermoid carcinoma appear to be closely related morphologically.
A 60-year-old woman receiving prednisolone therapy for Sjögren's syndrome presented with corneal perforation. Therapeutic penetrating keratoplasty was performed, but no causative organism was identified. Focal inflammatory infiltrates in both donor and host cornea and anterior uveitis developed postoperatively. Five months later the inflammation rapidly became much worse, with fulminating abscesses, and the eye was eviscerated because of severe endophthalmitis. Nocardia organisms were identified in the cornea, conjunctiva and vitreous by means of acridine orange and modified Ziehl-Neelsen stains. The infection produced both suppurative and granulomatous inflammation. Reexamination of the penetrating keratoplasty specimen with a modified Ziehl-Neelsen stain revealed a few Nocardia organisms, which suggested that infection had occurred at the time of corneal perforation.
A 31-year-old man with a 2-year history of recurrent dacryocystitis underwent dacryocystorhinostomy. An unexpected operative finding was a polyp in the lacrimal sac. Histopathological examination revealed a plasma cell granuloma. The nature of this uncommon inflammatory pseudotumour of the lacrimal sac is discussed.
Two cases of oncocytoma of the lacrimal caruncle are presented. This benign tumour is characterized by a uniform population of epithelial cells with abundant, finely granular, eosinophilic cytoplasm. The granules stain blue with Mallorys phosphotungstic acid hematoxylin and can be identified as mitochondria by electron microscopy. An additional 35 cases from the literature are reviewed. The tumour usually occurs in people over the age of 60 years and is more common in women (male:female ratio of 1:5).
A case of multicentric reticulohistiocytosis (MR) in a 24-yr-old woman is presented. MR is a rare disorder characterized by progressive polyarthropathy and a papulo-nodular skin rash. The diagnosis was established by histological examination of biopsies of erythematous nodules on the fingers which showed circumscribed collections of large mononuclear cells and multinucleate giant cells in the reticular dermis. These were embedded in a fine network of mature fibrous tissue with a scanty lymphocytic infiltrate. Histochemical, immunopathological and ultrastructural investigations confirmed that the large mononuclear cells had the properties of macrophages. The histopathological features of MR are reviewed in the light of current knowledge of macrophage physiology, and evidence for lymphocyte-histiocyte interactions in the pathogenesis of this bizarre granulomatous disorder is presented.
Rabbit lens epithelial cells synthesize and secrete a variety of [35S]sulphate-labeled glycoconjugates in vitro. Associated with the cell layer, and with the medium, was a high molecular weight glycoconjugate(s) that contained heparan sulphate which was apparently covalently linked to sulphated glycoprotein. This component(s) was eluted in the void volume of a Sepharose CL-2B column and could not be fractionated by detergent treatment or extraction with lipid solvents. The cell layer also contained glycosaminoglycans (72% heparan sulphate, 28% chondroitin sulphate), as well as a small proportion of a low molecular weight sulphated glycoprotein. The major 35S-labeled species secreted into the medium were sulphated glycoproteins with approximate molecular weights of 120,000 and 35,000 together with a heparan sulphate proteoglycan. This proteoglycan could be precipitated from the culture medium with 30% saturated (NH4)2SO4 and eluted from Sepharose CL-4B columns at approximately the same position (Kav = 0.15) as heparan sulphate proteoglycans described in the basement membrane of the EHS "sarcoma" (Hassell, J. R., P. G. Robey, H. J. Barrach, J. Wilczek, S. I. Rennard, and G. R. Martin, 1980, Proc. Natl. Acad. Sci. USA, 77:4494-4498) and of the mouse mammary epithelium (David, G., and M. Bernfield, 1981, J. Cell Biol., 91:281-286). Its presence in the culture medium was unanticipated but may be explained by the inability of these cultures to deposit a basement membrane when grown on a plastic surface. The relationship of this heparan sulphate proteoglycan to the lens epithelial basement membrane is the subject of the following paper.
Rabbit lens epithelial cells display a similar "cobblestone" morphology and produce the same complement of sulphated macromolecules (also see Heathcote, J.G., and R.W. Orkin, 1984, J. Cell Biol., 99:852-860) whether grown on plastic or glass, dried films of gelatin or type IV collagen with laminin, or on gels of type I collagen. There was no evidence of basement membrane formation by these cells when they were grown on plastic, glass, or dried films. In contrast, cultures that had been grown on gels deposited a discrete basement membrane that followed the contours of the basal surfaces of the cells and in addition, they secreted amorphous basement membrane-like material that diffused into the interstices of the gel and associated with the collagen fibrils of the gel. A significant proportion (approximately 70%) of the heparan sulphate proteoglycan fraction that was secreted into the culture medium (fraction MI) when the cells were grown on plastic became associated with the cell-gel layer in the gel cultures. Further, when basement membrane was isolated by detergent extraction, greater than 90% of the 35S-labeled material present was in this heparan sulphate proteoglycan.
In controlled clinical studies of adult diabetics a 42% incidence of signs of Dupuytren's disease was found. The incidence was highest in the older patients with a longer history of diabetes, but was not related to the severity of the diabetes. The features of Dupuytren's disease in the diabetics has a distinctive pattern, being more severe in men than women and, compared with controls, having a radial shift towards the middle finger. The disease was mild and of benign prognosis, rarely needing operation. In a further study, 13% of patients with Dupuytren's disease were found to have a raised blood glucose level. The question is posed as to whether the biochemical disturbance causes the Dupuytren's disease or whether the pattern of inheritance predisposes to both Dupuytren's disease and diabetes.
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