Eye (1989) 3, 791-802

Terrien's Disease: Clinical and Ultrastructural Studies, Five Case Reports

Y. POULIQUEN, P. DHERMY, G. RENARD, L. GOICHOT-BONNAT, G. FOSTER AND M. SAVOLDELLI Paris

Summary Terrien's disease occurs in middle-aged patients and is characterised by an insidious thinning of the cornea near the limbus. In most cases, this results in a peripheral ectasia associated with a severe degree of astigmatism. Inflammatory signs are rarely observed in this marginal corneal degeneration which is of unknown aetiology. Elec­ tron and light microscopic studies have been performed on five specimens from pen­ etrating keratoplasties. Anatomical and clinical correlations showed the marked marginal degenerations of the corneal stroma to be consistently associated with lipid deposits, but without inflammatory cell infiltrate. These changes are in agreement with previous reported pathological descriptions of Terrien's disease.

Terrien's disease is a rare corneal affection. It Sometimes, spontaneous perforation can was first described by Terrien1s is 1900 under necessitate urgent treatment. the name of 'Marginal, symmetrical, ectatic Previous electron microscopic studies have corneal dystrophy'. Clinical and histological been reported, but these have only been per­ descriptionss.6,7,10,17 made it possible to distin­ formed in lamellar corneal buttons.9 These guish several clinical varieties of the disease. studies attempt to subdivide the disease into Terrien's disease consists of a peripheral two groups: quiescent and inflammatory, corneal thinning which leads to ectasia after a however, the aetiology or pathogenicity of the prolonged interval. Several radial vessels are condition is still unknown. The five cases found in the thinned relatively transparent reported here have been subject to both light area, the anterior margin of which is marked and transmission electron microscopic stud­ with a dense yellowish area suggesting lipid ies: the diagnosis was made initially on Ter­ deposits. In most cases, the disease affects rien's described criteria. both corneae, is symmetrical and most often 60 observed in men over years old, the con­ Case Reports dition progresses very slowly, but the thinning Case No.1 1, and ectasia can still continue for several years (Fig. Panel I) MBG, a 42 years old, tool-maker, presented in and is often associated with an severe astig­ April 1983 with progressive visual loss. His visual matism. No inflammatory signs can be acuity was less than 1/10 in both eyes, N14 in the observed, but the pain and redness which right eye and N5 in the left eye. Both eyes had an have occasionally been reported have been against the rule astigmatism of more than six related to puberty and menstrual cycles. 1 dioptres: the acuity of the right eye could not be

Correspondence to: Department of Ophthalmology, Hotel-Dieu and INSERM Group U 86, Hotel-Dieu, 1 Place du Parvis Notre-Dame, 75004 Paris. 792 Y. POULIQUEN ET AL.

Figs. 1 and 2. Case 1: MBC: 42. Male, Right Eye, Flatteningof the corneal curvature in its vertical axis. Marked peripheral thinning is observed at 12 o'clock, leading to a crescent shaped ectasia. Deep corneal opacities can be seen in this ectatic area which is crossed over by fine vessels.

Figs. 3 and 4. Case 4: de St Mar: 77. Male, Advanced bilateral Terrien's disease. The central corneal protrusion is surrounded by severe perilimbal thinning. Its inner border is limited by a yellow ring, which corresponds to fatty deposits.

Panel I improved, but the left was improved to 4110 with penetrating keratoplasty, decentered at 12' o'clock -6 D.Sph, at 90°. Slit lamp examination revealed a and passing through the limbus to enclose the ecta­ flattening of the corneal curvature in its vertical ctic area. The specimen was fixed for pathological axis. A marked peripheral annular thinning was examination. There was no intra-operative compli­ observed at 12 o'clock leading to a crescent shaped cation. On April 27, 1985, the graft was transparent ectasia extending from three to nine o'clock. Deep after a period of post-operative oedema; the astig­ corneal opacities were observed in this area which matism was +3 D.sph at 90°, the visual acuity was was covered by fine vessels. 1110 and N2, the number of endothelial cells was The rest of the ocular examination was normal in 900 per square millimetre. The continuous suture this alcoholic patient. was removed because of loosened loops. The visual On September 5, 1984, we performed a 9 mm acuity was less than expected considering the clar- TERRIEN'S DISEASE 793 ity of the donor and button, An optic nerve neu­ which had been lost following injury at the age of 32 ropathy due to smoking and alcoholism may years. At 47, she felt the firstvisual signs in her right explain this poor visual result, eye. Corneal deformation developed progressively until 1981 when she was sent to our hospital. She Case No.2 was found to have a typical superior Terrien's mar­ Mrs 0 M, from Martinique was a 74 years old ginal ectasia characterised by a corneal thinning insulin dependent diabetic with only one eye, between three and nine o'clock, corneal vessels and

Fig. 1. Case 2: Perilimbal corneal thinning Fig. 3. Case 3: Soudan III staining. A positive (0.12 mm). The Epithelium is thick, Bowman's demonstration of lipidic deposits. membrane is missing. The corneal stroma is thin but quite normal in its lamellar organisation.

Fig. 2. Case 2: In a thicker zone: Vacuolated stroma, Fig. 4. Case 4: P.A.S. stammg. The stromal and thick Descemet's membrane. Endothelial cells are connective tissue and the vacuolated corneal cells take present. up the stain. Panel II 794 Y. POULIQUEN ET AL.

Fig. 1 (TEM x 2400). Case 1: Sub Epithelial zone. Some lymphocytes (Ly) are seen between basal epithelial cells (E) and are also present in the anterior stroma. Somme vessels (Vx) are running into this thin zone a/the perilimbal cornea. Fig. 2. (TEM x 31950). Case 1: Abnormal basal lamina: Duplicated, disrupted and abnormal collagen fibrils.

Panel III yellowish deposits. The centre of the cornea was transient, short lived episodic pain and redness of clear. A small circular descemetocele of 2 mm dia­ his right eye. Terrien's disease was diagnosed. The meter was located at six o'clock in the ectatic area. lesion was roughly symmetrical consisting of mar­ An against the rule astigmatism of three dioptres ginal corneal thinning of the inferior nasal area of was measured by keratometry. The visual acuity his right eye with some vascularisation. Severe was limited to counting finger at 1.5 m, the eye was astigmatism limited the visual acuity to counting otherwise normal except for a slight posterior cap­ fingers at one metre. There was an inferior thinning sular cataract. in the left eye with a minor deformation which per­ 26, On February 1981 a penetrating half circle mitted a visual acuity of 7/10. Two months later, superior corn eo-scleral keratoplasty was per­ although a penetrating keratoplasty had been pro­ formed between four and eight o'clock and the grammed for the right eye, a perforation took place resected specimen was fixed for pathological exam­ in the thinned zone. ination. The superior area of the transplant was A penetrating 9 mm keratoplasty was performed recovered with conjunctiva. The post-operative which was decentered towards the sclera to include course was unremarkable except for a slight inflam­ the perforated area. Six months later, the patient matory reaction of the anterior chamber during the had cataract surgery in the same eye, following first few days, which was accompanied by a tran­ which the corneal button became cloudy. The sient elevation of intraocular pressure. She left for patient has not been seen since. Martinique on April 10,1981. Her visual acuity was

unchanged for far vision because of her cataract, Case No. 4 (Figs. 3 and 4, Panel I) but she could read large characters for near vision. Mr De St, aged 77 years old, presented for the first The anterior chamber was quiet, the central cornea time in June 1985, with advanced bilateral Terrien's was clear, the corneal button well fitting and the disease. Both corneal protused a central area being ocular pressure was normal. The patient has not of normal thickness surrounded by severe peril­ been seen since. imbal thinning. This area was transluscent, mark­ Case No.3 edly thin, ectactic and was invaded by small vessels. Mr B A, 64 years old, complained 20 years ago of a Its inner border was limited by a yellowish ring. Different features of the corneal stroma lipid deposits. Fig. 3. (x 6140): Diffusevacuolization of the corneal lamellae. Fig.·4. (x 10740): Details of the vacuoles (V), some of them are surrounded by a thin dark membrane (arrows). Fig. 5. (x 13150): Lipidic coloration of the vacuolar deposits. Fig. 6. (x 24120): Vacuoles (V) crystal (C). Fig. 7. (x 2310): Lipid crystal (C). Fig. 8. (x 10740): Grouped vacuoles in the stroma. Note the keratocyte activity:mitochondriae (M) and glycogen (Gly) deposits, in the cytoplasm of the cell.

Panel IV 796 Y. POULIQUEN ET AL.

Fig. 9. (x 39460): Vacuolar diffuse infiltration of the stroma. Collagen fibrillar degneration (arrows). Fig. 10. (x 39460): Grouped microfibrils, dispersed collagenfibrills whose diameter is heterogenous. Fig. 11. (x 39460): Fibrinoid degeneration of the collagen fibrills around the lipid vacuoles. Fig. 12. (x 24120): In the neighbouring of the cholesterol crystals collagen fibrills are modified. Fig. 13. (x 394690): I}lastin deposits in the stroma. Fig. 14. (x 39460): Corneal scar tissue formation. Fig. IS. (x 24120): Long period collagen formation in the thinned part of the cornea.

Panel V TERRIEN'S DISEASE 797

Table I

Corneal thickness Case 2 Case 4 (mm) Case 1 blocks a, b, c Case3 blocks a, b, c Case 5

<= Full 0,29 0,20 0,16 0,12 .8 0,34 0,28 0,175 0,28 "" Epithelium 0,015 0,06 0,05 0,05 .... 0,105 0,090 0,D75 0,065 Descemet + 0,0075 0,D35 0,03 0,D25 ..8.... 0,06 0,06 0,04 0,010

Normal thickness for central cornea is 0,45 mm, and peripheral cornea 0,65, after

The astigmatism was so severe that it could not be six o'clock (the specimen was fixed forpathological measured, the visual acuity was 1120, reading was examination). One year later, the visual acuity was impossible. The marginal deformation was more 5/10 and p2 despite the presence of a slight corneal marked in the left eye than in the right. A moderate oedema in the inferior half of the corneal button. nuclear cataract was severe in both eyes. The intra­ Local corticotherapy was maintained continuously. ocular pressure was normal. On October 11, 1985, a penetrating keratoplasty Materials and Methods of 11 mm was performed on the right eye. After a All surgical specimens had the same treat­ perilimbal conjunctival dissection, the trephination ment: half the tissue fixed in a Bouin's solu­ allowed removal of the central cornea with its tion, embedded in paraffin and stained with thinned periphery. The patient was treated with hematoxoline-eosine for light microscopic dexamethasone and cyclosporin A. Six months examination the other half was treated for later the corneal button was still clear. The visual acuity was 2/10 and N4 despite the presence of an electron microscopic examination. This was against the rule astigmatism of six dioptries. In fixed in a glutaraldehyde solution (2,5%) for April 1986, there was a small allograft reaction two hours, washed out in a Sorensen buffer which lead to an opacification of the graft. solution pH 7.4, dehydrated in alcohol baths In May 1986, a penetrating keratoplasty of of increasing concentrations, embedded in 11 mm was performed on the left eye, combined epon, and cut in medium and ultra thin sec­ with an extracapsular cataract extraction with a tions. Cases 4 and 5 specimens were placed in posterior intraocular lens implantation. The cor­ formol for lipidic study and treated in a similar 18 neal graft remained clear for months under a fashion for lipid electron microscopic regimen of dexamethasone and cyclosporin A. examination. Later, this also became cloudy after an allograft reaction. The patient's vision was still 1110 in the right eye which permitted him to move around. The Results two corneas were subjected to a light and electron microscopic study. Light microscopic examination (Table I, Figs. 1,2,3,4, Panel II) Case No.5 This confirmeda severe thinning of the peri­ Mr B J, 36 years old, presented with progressive astigmatism leading to severe cornea plana in the limbal area. This thinning was different from vertical axis of the left eye such that the vision could one case to another and from one area to not be improved. A similar though less severe con­ another in the same specimen. Epithelial dition affected the right eye, in which visual acuity changes and thickness irregularities were was 5/10. The left eye which was seriously affected, noticed in the four non perforated cases: epi­ presented with a typical appearance with marked thelium was normal (case 1),thickened (cases thinning of the cornea between six and nine 4 and 5), or much thinned (case 2). The cells o'clock. This thinning was discontinuous consisting were stratified and normal except in certain of an apposition of small thinned areas surrounded areas where rare inflammatory cells were by yellowislideposits and normal limbus areas. The found. In these same areas, Bowman's mem­ patient's history revealed episodic discrete redness which did not require any anti-inflammatory treat­ brane was absent,fragmented or broken. The ment. Otherwise, the patient was in good health. underlying corneal stroma was modifiedin all In July 1986, a penetrating keratoplasty of 9 mm its layers (cases 4, 5) and especially in its was performed in the left eye, it was decentered at anterior (case 1) or posterior (case 2) layers. 798 Y. POULIQUEN ET AL.

Changes were also noticed in the shape of the spaces which were not surrounded by any cell fibrocytes which were distended or modified membrane. In case 4, membrane residues adjacent to the anomalies of the collagen were found at a distance from the epithelium. fibres. They also conserved their vacuoled The thinned corneal stroma presented cytoplasm. Few inflammatory cells were numerous modifications; such as a lamellar found in the stroma in all the cases. structural disorganisation. Certain lamellae Blood vessels were constantly observed. had a good collagen fibre arrangement while Descemet's membrane was either normal or others were totally disorganised, their colla­ slightly altered being thickened with posterior gen fibres being scattered in different tuberosities (case 2), sometimes it was broken directions. or healed by posterior fibrous tissue (case 4). Generally, the stroma/lamellae were dis­ A few endothelial cells were always present in organised with small cavities, optically clear, the posterior surface of Descemet's mem­ circular or oval shaped. Some of the vacuoles brane. The perforated cornea in case 3 did not seemed to have no membrane, while others differ from the others especially in central seemed to be lined with a discontinuous dense area: cholesterol crystals were particularly material, others also were delimited by a con­ evident there, being scattered within the stro­ tinuous cytoplasmic cellular-like membrane. mal lamella and around 'spumous' cells sur­ Some of the vacuoles contained more or less rounded by inflammatory c.ells. Soudan III confluent pieces of membrane. staining used in cases 4 and 5 revealed a dense In case 3 these vacuoles, similar to those lipidic infiltration. observed above, were scattered within large lipidic crystals with which they were some­ Electron microscopic examination times in contact and were often associated The epithelium present in the thinned area with electron dense deposits. These could be was never completely normal. In cases 1, 3 arranged in rings around the vacuoles, gath­ and 4 it was stratifiedwith basal,intermediate ered in small island at a distance,or even scat­ and superficial cells. The interstitial spaces tered within the lamella (Figs 3-8, Panel IV). were dilated and filled with a granular The fine granular material in these dense material which was more or less dense to elec­ deposits was often in close contact with the trons (cases 1 and 3). Sometimes, optically lipid crystal in case 3. In case 1, the represent clear vacuoles were found in basal and inter­ the most important anomaly. mediate cells (case 3). In case 2, the epithe­ Scattered elastin and fibrin fibres were lium in certain areas was thinned and found in the stroma of cases 1 and 4. In case 4, infiltrated with inflammatory cells (cases 2 irregular patches of long periodicity collagen and 3). Some of these cells also contained fibreswere scattered within the fibrocytesand large, numerous, irregular mitochondria. the fragment of Descemet's membrane. The basal lamina which was always altered, Certain areas in the stroma showed signs of thickened, discontinuous, broken or dupli­ healing reconstruction which consisted of a cated was lying on an altered stroma. Most of heterogenicity of the collagen fibres. Else­ the time, hemidesmosomes adhesions were where collagen fibres degeneration was observed (Figs. 1,2, Panel III). characterised by the absence of contact Bowman's membrane was either broken or between fibrillar and interfibrillar space or absent. Some of its fragments could be identi­ deposits. This degeneration is usually accom­ fied in the anterior altered layers of the cor­ panied by fibrinoid collagen degeneration neal tissue (cases 2, 3, 4 and 5). In cases 2 and (Figs. 9-15, Panel V). 5, Bowman's membrane was present, in con­ Stroma fibroblasts cells had different tact with the epithelium, but, presented some appearances according to the area examined. important modifications: fragments of basal They were usually abnormal, often inten­ membrane were superimposed and interlaced sively activated, or even subjected to severe with small microfibrillar islands. Sometimes changes (case 1) such as cellular necrosis. The these fragments were condensed into dark severity of the changes was proportional to irregular patches containing clear vacuolated corneal vacularisation. TERRIEN'S DISEASE 799

They were generally activated with a glyco­ the rule astigmatism. Systemic investigations gen rich cytoplasm and developed organelles, revealed diabetes in two cases and an alcohol! Numerous cells possessed optically clear tobacco intoxication in one case. There were vacuoles. no signs of any rheumatic or collagen dis­ In case 3, numerous macrophages were eases, nor any signs of an acquired immune grouped within lipid crystals in infiltrated syndrome. There was no special ocular areas where many spumous cells were found. history in these five cases. All the patients It was among these cells that plasma cells were conformed to the classical description of Ter­ found. Specificlipidic staining was positive in rien's disease as discussed by Austin et al. 1 electron microscopic specimens of cases 4 and Although Terrien's disease can be identi­ 5. fiedas a clear entity,its nature is still obscure. Numerous vacuole cells containing micro­ It is a slowly progressive disease lasting for as fibrillar material were observed in case 4 as long as 30 years in some patients. It is rarely well as some polymorphonuclear cells. associated with inflammatoryepisodes except Inflammatory cells were rare, but were found when associated with limbal vasculitis,. 9 particularly in two specimens. In case 3, they All histological examinations showed cor­ were located near the perforation and areas neal stromal thinning, an altered basal lamina with intensive lipid infiltrate; in case 4 they and Bowman's membrane, a disorganised were found near the residue of Descemet's corneal stroma with interstitial vascularisa­ membrane and under the epithelium. tion, intense lipid infiltration, a thickened Numerous blood vessels and nerve fibres Descemet's membrane and few inflammatory were found in all the specimens. Descemet's cell infiltrates. membrane was either broken (case 3) or frag­ Terrien's disease would be much better mented with different pieces scattered within understood if it showed a specific ultrastruc­ the ectatic stroma (case 4). It was thickened in tural abnormality as a specificconfirmation of all cases. It was also partially or totally infil­ abnormalities. trated by microvacuoles scattered within is These 5 cases reports are unusual because collagen nodules. Endothelial cells when they have been treated by six penetrating ker­ present were quite normal except in case 4 atoplasties. This has permitted a study of the where they were noted to have abnormally lesion throughout the full thickness and over dilated mitochondrias. the thinned ectatic periphery. The most important corneal change is the Discussion peripheral thinning which reduces it to half The five reported cases had the same clinical the normal thickness. This is not a specific appearance which consisted of progressive characteristic as thinning occurs in marginal marginal corneal thinning and ectasia. There keratoconus, keratoglobus and Mooren's were no inflammatory signs in three cases. ulcer. Nevertheless,ultrastructural anomalies Repeated episodes of inflammation led to can clearly differentiate these three limbic perforation in case 3 while in case 5 some epi­ conditions from Terrien's marginal ectasia. sodic ocular redness was observed. Clinical Marginal keratoconus has the same structure observation of these cases by the same phys­ as classical keratoconus; the stroma is ician made it possible to distinguish Terrien's thinned, but the lamellar structure is easily disease from the other three corneal marginal recognised. Even if they are full of finedense conditions with which it could be confused: deposits, they never present any vacuoles as marginal keratoconus (marginal pellucid was reported in the above cases. Further­ degeneration), keratoglobus and Mooren's more, Bowman's membrane is characteristi­ ulcer. cally broken, but Descemet's membrane is All cases had paralimbal corneal thinning almost always normal inflammatorycell reac­ with more or less ectasia, interstitial vascular­ tion is encountered only when the cornea has isation, annular lipid accumulation in the perforated. In keratoglobus,protrusion of the inner ring of the thinning and distortion of the entire corneal surface is also characterised by corneal curvature producing severe against the absence of inflammatory cells. 800 Y. POULIQUEN ET AL.

In addition acquired and congenital ker­ particular attention to the relationship atoglobus have different specificsigns. II Con­ between the agllutination around the vacu­ genital keratoglobus has obvious anomalies of oles. This change suggests a possible disorder the stromal structure: absence of Bowman's which may precede vascularisation, intersti­ membrane, quantitative modifications in the tial necrosis or even a degenerative process of ratio of collagen to matrix and Descemet's collagen. This relation, we noticed in membrane is profoundly altered. The ana to­ Schnyder cristalline dystrophy,8 these infil­ mopathogical changes in Mooren's ulcer, in trates and lipid crystals insinuate themselves its acute phase, cannot be confused with Ter­ through the structure of or collect around col­ rien's disease because even healed quiescent lagen fibrils. Mooren's ulcers show an acute reaction in All of these stromal modifications (vacu­ which inflammatory cells prevail. olar infiltrations, dense micro granular The five cases reported here make it poss­ deposits, collagen fibrils agglutination or dis­ ible to differentiate Terrien's disease from sociation, fibrinoid degeneration), suggests other limbal conditions. an organised degeneration process surround­ The stromal changes found in Terrien's ing the lipidic infiltrates. In our fivecases, the disease consist of dense dispersed deposits process is associated with a mild inflammatory which are scattered or grouped within the cell infiltration. lamella,and which are found in the peripheral Stromal inflammatory cells were particu­ stromal lamella, these vacuoles are an impor­ larly observed in case 4 which was excessively tant ultrastructural element which is specific evoluted and thinned presenting scattered of this condition. They can alsQ be found in polynuclear cells within its stroma. It was only the anterior layers of a normal Descemet's in case 3 that we observed numerous macro­ membrane or in its fragments in the posterior phages and 'spumous' cells surrounding lipid stromal layers. These multiple sometimes crystals. It was within these areas that we confluent deposits are dissolved by fixation found some plasma cells. are associated with cholesterol lipidic crystals, In the other cases, only few lymphocytes (in case 3) suggesting a lipid nature. were found in the epithelium. In fact, in none Soudan III staining was positive in cases 4 of our five cases could we detect a severe and 5 and examination of the firstthree cases inflammatory cell reaction. by electron microscopy confirms the first Healing and degenerative signs coexist. description made by Fine et al. 4 A histochemi­ They are characterised by fibrocytic activity cal study was performed which revealed the (dense glycogen cytoplasmic aggretates). lipid nature of these lesions. In cases 4 and 5, Corneal scarring represented by either het­ specific lipid staining for electron microscopy erogeneous or long periodic collagen fibres. confirmed the lipid nature of the deposits, Vessels were numerous, but the blood vessels which confirmsthe clinical appearance, How­ were not surrounded by numerous inflamma­ ever, this is not enough to explain the entire tory cells. paralimbic degeneration of Terrien's disease. Are these anomalies sufficient to explain It is possible that this disease,like some pri­ the development of Terrien's marginal ecta­ marily lipidic degeneration consist of a magni­ sia? It seems unlikely because rings are fre­ fication of the degenerative process which quently observed in moderately altered leads to arcus senilis. However,Iwamoto et at" peripheral cornea, in which dense irregular showed in their lamellar specimen study that infiltratedeveloped an arcus senilis where the lipid infiltration occurs together with other stroma is modified or the corneal epithelial is lesions and that histochemistry also shows the thickened above without any thinning of the presence of mucopolysaccharide acid cornea. These individuals do not show any deposits14 a substance which is highly eosino­ ectasia or any particular clinical or anatomical phlic, PAS positive or intensively stained by evolution. PTH,suggesting a fibrinoidcollag en degener­ Could the lipidic deposits by the starting ation.9 These substances were found in the point of a slow degenerative process leading five cases reported here. Iwamoto et at. pay to subsequent modificationsor could there be TERRIEN'S DISEASE 801 additional factors leading to the modifications we have one patient who developed kerotoso­ mentioned above? Berkowitz et al.2 tried to nus and Terrien's disease. The role of the lipid answer these questions by searching for circu­ is uncertain. Most authors believe that the lating immune complexes in peripheral cor­ excess lipid is only secondary to the interstitial neal diseases; Mooren's ulcer, Terrien's vacuolisation of the thinned area. It is usually disease and corneal ulcers associated with present in arcus senilis but here it is not associ­ connective tissue diseases. ated with a corneal thinning. However, it is These authors found no difference in any possible that it is one of the promoting factors circulating immune complex between normal of lysis and disorganisation of corneal con­ patients and patients suffering from Terrien's junctival tissue leading to corneal peripheral disease. These studies showed that an thinning. Lipid deposition is a constant fea­ immune reaction type III could not play any ture of Terrien's disease and its presence in part in the pathogenesis of the disease. This excess cannot be ignored. test suggests that it is possible to differentiate At present it appears that the ectactic Mooren's ulcers fromTerrien's degeneration. changes in Terrien's disease are the terminal However, not everyone agrees with this clear phase of a condition characterised by struc­ differentation between the two conditions, tural weakness of the peripheral corneal Austin! stated that inflammatoryepisodes can stroma which is associated with mild inflam­ be encountered during the evolution of Ter­ matory episodes and the secondary desposi­ rien's degeneration and Binder et al.3 tion of lipid in the adjacent cornea and reported a case of bilateral marginal ulcer conjuctiva. which could be easily confused with Terrien's References disease. However this case of marginal ectasia 1 Austin P and Brown SI: Inflammatory Terrien's mar­ was quite different from the five cases ginal corneal disease. Am J Ophthalmol1981, 92: reported in this paper. Its anatomico-path­ 189-92 . ological description shows an inflammatory 2Berkowitz PJ, Arenssen JJ, Felberg NT, Laibson PR: Presence of circulating immune complexes in necrosis of the superficiallayers of the cornea patients with peripheral corneal disease. Arch while the posterior layers as well as Desce­ Ophthalmol1983, 101: 242-5 . 3 met's membrane were normal. This is like a Binder P, Zavala EY, Stainer GA: Non-infectious Mooren's ulcer and is clearly different from peripheral corneal ulceration: Mooren's ulcer or Terrien's marginal degeneration. Ann Ophthal­ the changes we and others have found in Ter­ mol 1982, 14: 425-532 . rien's disease. Nevertheless, it is interesting to 4 Fine BS , Thownsend WM, Zimmerman LE , consider the views of Binder et al. which refer Lashkari MH: Primary lipidal degeneration of the to a possible similarity between the mech­ cornea. Am J Ophthalmol1974, 78: 12-23 . 5 anisms which cause the lesions observed in Fran<;ois J: La degenerescence marginale de la cor­ nee. Arch Ophthalmol1936, 53: 616 . these two non-infectious affections of the cor­ 6 Fuchs E: Ueber Randsklerose und Randa trophie neal limbus. Although the clinical appear­ der Hornhant. Albrecht v Graefe's Arch Klin ances are similar they cannot be the result of OphthalmoI1901, 52: 317 . the same disease. In Mooren's ulcer, the pres­ 7 Fuchs E: Ueber senile Randatrophie der Hornhant. Albrecht v Graefe's Arch Klin Ophthalmol1915, ence of immunoglobulins, circulating anti­ 89: 386 . bodies and immune complex leads to an active 8 Hoang-Xuan T, Pouliquen Y, Savoldelli M, Gasteau inflammatory lesion. All of these manifesta­ J: Dystrophie cristalline de Schnyder. J Fr tions are absent in Terrien's disease, which in Ophtalmol1985, 8: 735-42. 9 Iwamoto T, De Voe AG, Farris F: Electron micro­ typical form is slowly progressive and ectatic scopy in cases of marginal degeneration of the giving rise to slow interstitial necrosis of the cornea. Invest OphthalmoI1972, 11: 4, 241-57 . disorganised corneal stroma resulting in per­ 10 Lauber H: Ueber periphere Hornhant ektasie. Klin ipheral thinning. Monatsbl Augenheilkd 1905, 43: 382 . 11 Pouliquen Y, Espinasse MA, Dhermy P, Savoldelli However, this process is similar to that M: Le keratoglobe. J Fr Ophtalmol 1985, 8: observed in. certain corneal inflammatory 43-54. lesions, especially herpes, in which there is 12 Pouliquen Y, Renard G, Savoldelli M: Keratoconus sometimes stromal thinning. We have found associated with Terrien's marginal degeneration. Acta Ophthalmologica 1988 (sous presse). lymphocytes and plasma albumin in our cases 3 1 Savino DF, Fine BS, Alldredge OC: Primary lipidic so it is possible that inflammatory episodes degeneration of the cornea. Cornea 1988, 5: could play a part in the necrosis particularly as 191-8 . 802 Y. POULlQUEN ET AL.

14 Suveges L, Levai G, Alberth B: Pathology of Ter­ 16 Terrien F: La dystrophie marginale ectatique de la rien's disease. Histochemical and electron micro­ cornec. In Semeiologie oculaire: la calotte corneo­ scopic study. Am ] Ophthalmol 19 72, 74: sclerale. Masson, pp 167-9 , 19 23 . 1191-200. 17 Trantas A: Ectasie peripherique de la cornee de Ter­ 15 Terrien F: Dystrophie marginale symetrique des rien (keratoleptensis marginal). Clin Ophtalmol deux cornees. Arch Ophtalmol1900, 20: 12 . 1925 , 14: 621 .