BILATERAL N E C R O T I Z I N G SCLERITIS TAKA-AKI H A T S U D A , M.D., Kyoto,
Necrotizing inflammation of the sclera is a rare, severely destructive condition that sometimes leads to the loss of an eye after deteriorating vision and severe pain. The essential pathologic change consists of a central area of necrosis surrounded by a palisade of epithelioid and giant cells, which is most clearly seen in a sub cutaneous rheumatic nodule. There are four distinctive clinical entities that can be ascribed to the collagen diseases, par ticularly to rheumatoid arthritis: episcleral rheumatic nodules, seleromalaeia perforans, necrotizing nodular scleritis, and massive granuloma of the sclera. 1,2 We described herein a case of bilateral necrotizing scleritis, necrotizing nodular scleritis in the left eye and massive granu loma of the sclera in the right, observed in a woman whose history was noncontributory for rheumatoid arthritis or collagen disease. CASE REPORT A 53-year-old woman first came to our clinic in November 1974 complaining of painful red eyes with progressive loss of vision over the past several months. The ophthalmologic examination on Nov. 30 re vealed visual acuity of R.E.: 6/15 (20/50); L.E.: 6/120 (20/400). The eyes were deeply injected, espe cially in the left eye. Slit-lamp examination of the left eye showed a cloudy cornea, fine keratic precipi tates, flare and cells in the anterior chamber, almost complete posterior synechiae, and hazy media. The pupil was fixed and irregular. The right eye was similar to the left eye except for a transparent cornea, less posterior synechiae, and little haze in the media. The pupil could not be well dilated. Gonioscopy revealed open angles. The fundus of the left eye was From the Departments of Ophthalmology (Dr. Hatsuda) and Pathology (Dr. Tanaka), Kyoto Prefectural University of Medicine, Kawaramachi, Kyoto, Japan. Reprint requests to Taka-aki Hatsuda, M.D., De partment of Ophthalmology, Kyoto Prefectural Uni versity of Medicine, Kawaramachi, Kyoto 602, Ja pan. 710
AND J U N I C H I TANAKA,
M.D.
Japan invisible. The right fundus had a hyperemic disk, but was otherwise normal. Intraocular pressure was R.E.: 12 mm Hg; L.E.: 27 mm Hg. The physical examination revealed no other sig nificant abnormalities. Routine laboratory studies including a complete blood cell count, platelets, erythrocyte sedimentation rate, urinalysis, electro lytes, lupus erythematosus preparation, an antinuclear antibody titer, and a serologic test for syphilis gave normal results. The patient's history, repeated physical examina tion, and laboratory studies revealed no arthritis or collagen disease. A diagonosis of bilateral iridocyclitis was made. The eyes were treated with atropine and local and systemic corticosteroids. The eye disease showed several remissions and exacerbations during the following several months. In September 1975, the left eye became progressively injected and painful despite vigorous treatment. In the beginning of Novemrjer, a nodular lesion appeared on the scleral surface from 4 to 8 mm posterior to the corneoscleral limbus, at the 11 o'clock position. The visual acuity decreased to light perception. One week later a dark spot was discovered at the top of the lesion. At the end of the next week, a yellow center of necrosis developed in the nodule. Neighboring insertion of the superior rectus muscle and overlying conjuncti va became necrotic (Fig. 1). The visual acuity had decreased to no light perception, and intraocular pressure was 4 to 7 mm Hg. Because of intolerable pain in this blind eye, the eye was removed Dec. 1, 1975. No implant was used following the enuclea-
Fig. 1 (Hatsuda and Tanaka). Left eye with necro tizing nodular scleritis.
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tion because of widespread necrosis of the conjunc tiva and Tenon's capsule. The pain had disappeared after the enucleation. The socket healed nicely and a prosthesis was tolerable. The right eye had been relatively quiet except for several episodes of iridocyclitis. The visual acuity of the eye was 6/60 (20/200), on Jan. 12, 1976. The right eye had the first episode of scleritis Jan. 16, 1976. Scleritis was situated nasally, appeared to be deep, and extended from the corneoscleral limbus to the insertion of the medial rectus muscle. Moderate flare and cells were present in the anterior chamber. Intraocular pressure was 5 mm Hg. Increasing cata ract and medial haze obscured the fundus. The visual acuity had decreased to finger counting. The eye was treated mainly with indomethacin supposi tories and supplementarily with retrobulbar or sys temic corticosteroids. After several remissions, blu ish prominent lesions appeared at the insertion of the lateral rectus muscle and adjacent to the superior corneoscleral limbus in June 1976. The cornea be came entirely edematous and was neovascularized around its circumference. The vision decreased to blindness. Three months later two necrotic foci appeared, one superior to the insertion of the lateral rectus muscle and the other just lateral to the inser tion of the superior rectus muscle. The necrotic lesions grew and fused together (Fig. 2). The ocular disease rapidly progressed, with recurrent severe pain and increasing pericorneal injection. The eye exhibited extreme limitation of movement in all directions. The pain was intolerable, and the pa tient was anxious to have the eye removed. The eye was enucleated on Nov. 22, 1976. During surgery, a dense cartilage-like mass was found adherent to the globe and to the loose tissues of the orbit in an equatorial portion nasosuperiorly. The mass seemed to be an extension of inflammatory infiltrates into the loose tissue of the orbit. No implant was used after the enucleation because the indurated ocular socket would not tolerate it.
711
a hard and tender mass occupying the socket. We found a rapidly enlarged mass filling the empty socket. Because of increasing pain, the orbital mass was removed Jan. 10, 1977. The pain disappeared after the surgery. T h e patient had no symptoms of arthritis or any other general disease at her last examination July 2, 1977. RESULTS
Immediately after enucleation, the globes were fixed in 10% formalin solu tion and embedded in paraffin blocks. The left eye was of normal size (Fig. 3). The cornea was normal. The sclera super ior to the corneoscleral limbus surround ing the nodular lesion was three times the normal thickness, and the thickening ex tended to the equator. There was a large area of exudative retinal detachment. High-power microscopic examination of the thickened lesion revealed that it con sisted of a massive chronic inflammatory process, which had some of the character istics of a granulomatous inflammation. The lesion was composed of a central focus of necrosis surrounded by densely arranged lymphocytes, epithelioid cells, and multinucleated giant cells in some areas (Fig. 4). Extensive interruption and necrosis were observed in the scleral col lagen bundles. To a lesser extent, a simi-
A dull pain persisted after enucleation. One week postoperatively, the patient began having a sharp pain in her right ocular socket. Examination showed
Fig. 2 (Hatsuda and Tanaka). Right eye with extensive necrotizing nodular scleritis.
Fig. 3 (Hatsuda and Tanaka). Left eye. Lowpower view of equatorial section. Sclereal thicken ing extends superiorly from corneoscleral limbus to equator. Retina is detached with eosinophilic subretinal fluid. Lens has been removed before parrafin embedding (hematoxylin and eosin, x2).
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Fig. 4 (Hatsuda and Tanaka). Left eye. Highpower view of scleral granulomatous lesion. (L) Dense lymphocytic infiltration, (E) palisading ar rangement of epithelioid cells, (S) interrupted bun dles of scleral collagen, (G) multinucleated giant cell (hematoxylin and eosin, x 190).
lar process was discernible in the inferior sciera. The sciera posterior to the equator was involved, as evidenced by some focal collection of lymphocytes. Iris, ciliary body, and choroid involved in the area of the scleral granuloma showed a diffuse infiltration with lym phocytes and epithelioid cells. Some of the epithelioid cells contained pigment granules in the cytoplasm. Remaining portions of the choroid showed only dif fuse infiltration of lymphocytes. The reti na was completely detached, contained eosinophilic subretinal fluid, and was mildly involved in the inflammatory pro cess. The pigment epithelium was de stroyed in the area of scleral granuloma. The optic nerve was somewhat atrophic and associated with slight gliosis. The right eye was phthitic. The cor nea was involved with superficial and midstromal neovascularization and perivascular lymphocytic infiltration, most marked at the corneoscleral limbus (Fig. 5). The sciera had extensive thick ening and formed a tumor-like mass that occupied the vitreous cavity. High-power microscopic examination of the mass revealed it to be composed of loose scar tissue with massive lymphocytic in-
NOVEMBER, 1978
Fig. 5 (Hatsuda and Tanaka). Right eye. Lowpower view of section of whole eye. Granulamatous mass occupied vitreous cavity. Episclera and cho roid are markedly involved by inflammatory process (hematoxylin and eosin, x2).
filtration, in which epithelioid cells and occasional giant cells were scattered (Fig. 6). Most of the collagen bundles of the sciera were distorted and necrotic. There was little vascularized infiltration in the sciera. Iris, ciliary body, and choroid were disintegrated and diffusely infiltrated by lymphocytes and scattered epithelioid cells. Occasional phagocytosis of pigment granules occurred in the epithelioid and giant cells (Fig. 7). The retina was atroph ic. The optic nerve was also atrophic and gliotic.
Fig. 6 (Hatsuda and Tanaka). Right eye. Highpower view of scleral granuloma. Scleral collagen bundles are distorted and almost missing. Massive lymphocytic infiltration and palisade arrangement of epithelioid cells are seen. No giant cells are present in this area. There is little vascularization (hematoxylin and eosin, x 190).
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Fig. 7 (Hatsuda and Tanaka). Right eye. Highpower view of scleral granuloma near ciliary body. Note epithelioid cells and giant cell containing pigment granules (hematoxylin and eosin, X380).
The cartilage-like mass attached to the sclera and orbital adnexae was histologically confirmed to be composed of fibroadipose tissue markedly infiltrated by granuloma, indicating intraorbital in volvement of the scleral lesion (Fig. 8). DISCUSSION
Necrotizing granulamatous scleritis is a rare ocular disease; necrotizing nodular scleritis, a violent inflammatory reaction usually associated with the somewhat loosely linked group of collagen dis eases, particularly rheumatoid arthritis,
Fig. 8 (Hatsuda and Tanaka). High-power view of intraorbital mass developing among orbital adnexae after enucleation of right eye. It consists of flbroadipose tissue mainly involved by diffuse lymphocytic infiltration (hematoxylin and eosin, x 190).
713
is even rarer. The characteristic histologic appearance of nodular granulomatous scleritis is a central area of necrosis surrounded by a palisading zone of epi thelioid and multinucleated giant cells, a granulomatous lesion most typically seen in a subcutaneous rheumatic nodule. The etiology of the condition is unknown. In this case the pathologic findings were necrotizing nodular scleritis in the left eye and massive granuloma of the sclera in the right eye. Scleral lesions of both eyes began with nodular scleritis, which developed necrosis resembling scleromalacia perforans, but with violent inflammatory symptoms. This strongly suggests that massive granuloma of the sclera is the severe condition of necrotiz ing granulomatous scleritis. Wolter and Bentley, 2 who reported two cases of a combination of scleromalacia perforans and massive granuloma of the sclera, stat ed that differences in the degree of necro sis and scar formation as well as location in the sclera distinguished three condi tions: scleromalacia perforans, necrotiz ing nodular scleritis, and massive granu loma of the sclera. Distinguishing between the three con ditions by their histologic features is difficult. Franceschetti and Bischler 3 sug gested that the term "scleromalacia per forans" be reserved for cases showing indolent necrotic sloughing and a quiet clinical course associated with rheuma toid arthritis, and that the expression "necrotizing nodular scleritis" should be used to designate cases exhibiting a vio lent inflammatory process, generally not associated with rheumatoid arthritis. Sev eral researchers 4 - 6 have supported this suggested differentiation between sclero malacia perforans and necrotizing nodu lar scleritis. Van der Hoeve, 7 who was the first to characterize this dramatic syn drome as a clinical entity and named it scleromalacia perforans, suggested that the cases he described might belong to
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AMERICAN JOURNAL OF OPHTHALMOLOGY
two categories. Our case had no history of rheumatoid arthritis or other collagen dis ease. One of us (T. H.) had encountered a case of scleromalacia perforans in a 37year-old woman with rheumatoid arthritis of six years' duration, whose left eye developed four necrotic nodules, which were resolved in four months. Hogan and Zimmerman 8 did not distinguish between rheumatoid lesions occurring in the sclera with or without a history of rheumatoid arthritis or related disease because of sim ilarity in the histologic features. There may be some exceptions, consisting of a combined occurrence of scleromalacia perforans and massive granuloma of the sclera, as in the cases reported by Wolter and Bentley. 2 However, from our own experience, we prefer to divide necrotizing granulomatous scleritis into sclero malacia perforans and necrotizing nodu lar scleritis. SUMMARY
A 53-year-old woman whose history was noncontributory for rheumatoid ar thritis and collagen disease had bilateral necrotizing inflammation of the sclera. The left eye had necrotizing nodular scle ritis and the right eye, massive granuloma
NOVEMBER, 1978
of the sclera. In the right eye the orbital adnexae were also involved by granulom atous infiltration and developed a mass of fibroadipose tissue simulating an orbital tumor within a week after enucleation. REFERENCES 1. Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 8, p . 2. Lon don, Henry Kimpton, 1965, p. 1036. 2. Wolter J. R., and Bentley, M. D.: Scleromalacia perforans and massive granuloma of the sclera. A report of an unusual combination of ocular patholo gy in rheumatoid arthritis. Am. J. Ophthalmol. 51: 71, 1961. 3. Franceschetti, A., and Bischler, V.: La sclerite nodulaire necrosante et ses rapports avec la scleromalacie. Ann. Oculistique 183:737, 1950. 4. Ashton, N., and Hobbs, H. E.: Effect of corti sone on rheumatoid nodules of the sclera (scleroma lacia perforans). Br. J. Ophthalmol. 36:373, 1952. 5. Anderson, B., and Margolis, G.: Scleromalacia. Clinical and pathologic study of a case with consid eration of differential diagnosis, relationship of col lagen disease, and effect of ACTH and cortisone therapy. Am. J. Ophthalmol. 35:917, 1952. 6. Francois, J., Victoria-Troncoso, V., Hanssens, M., and Bacskulin, J.: Scleromalacie perforante et collagenose. Ophthalmologica 159:71, 1969. 7. van der Hoeve, cited by Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthal mology, vol. 8, pt. 2. London, Henry Kimpton, 1965, p. 1037. 8. Hogan, M. J., and Zimmerman, L. E.: Ophthalmic Pathology, 2nd ed. Philadelphia, Saunders, 1962, p. 337.
Necrotizing inflammation of the sclera is a rare, severely destructive condition that sometimes leads to the loss of an eye after deteriorating vision and severe pain. The essential pathologic change consists of a central area of necrosis surrounded by a palisade of epithelioid and giant cells, which is most clearly seen in a sub cutaneous rheumatic nodule. There are four distinctive clinical entities that can be ascribed to the collagen diseases, par ticularly to rheumatoid arthritis: episcleral rheumatic nodules, seleromalaeia perforans, necrotizing nodular scleritis, and massive granuloma of the sclera. 1,2 We described herein a case of bilateral necrotizing scleritis, necrotizing nodular scleritis in the left eye and massive granu loma of the sclera in the right, observed in a woman whose history was noncontributory for rheumatoid arthritis or collagen disease. CASE REPORT A 53-year-old woman first came to our clinic in November 1974 complaining of painful red eyes with progressive loss of vision over the past several months. The ophthalmologic examination on Nov. 30 re vealed visual acuity of R.E.: 6/15 (20/50); L.E.: 6/120 (20/400). The eyes were deeply injected, espe cially in the left eye. Slit-lamp examination of the left eye showed a cloudy cornea, fine keratic precipi tates, flare and cells in the anterior chamber, almost complete posterior synechiae, and hazy media. The pupil was fixed and irregular. The right eye was similar to the left eye except for a transparent cornea, less posterior synechiae, and little haze in the media. The pupil could not be well dilated. Gonioscopy revealed open angles. The fundus of the left eye was From the Departments of Ophthalmology (Dr. Hatsuda) and Pathology (Dr. Tanaka), Kyoto Prefectural University of Medicine, Kawaramachi, Kyoto, Japan. Reprint requests to Taka-aki Hatsuda, M.D., De partment of Ophthalmology, Kyoto Prefectural Uni versity of Medicine, Kawaramachi, Kyoto 602, Ja pan. 710
AND J U N I C H I TANAKA,
M.D.
Japan invisible. The right fundus had a hyperemic disk, but was otherwise normal. Intraocular pressure was R.E.: 12 mm Hg; L.E.: 27 mm Hg. The physical examination revealed no other sig nificant abnormalities. Routine laboratory studies including a complete blood cell count, platelets, erythrocyte sedimentation rate, urinalysis, electro lytes, lupus erythematosus preparation, an antinuclear antibody titer, and a serologic test for syphilis gave normal results. The patient's history, repeated physical examina tion, and laboratory studies revealed no arthritis or collagen disease. A diagonosis of bilateral iridocyclitis was made. The eyes were treated with atropine and local and systemic corticosteroids. The eye disease showed several remissions and exacerbations during the following several months. In September 1975, the left eye became progressively injected and painful despite vigorous treatment. In the beginning of Novemrjer, a nodular lesion appeared on the scleral surface from 4 to 8 mm posterior to the corneoscleral limbus, at the 11 o'clock position. The visual acuity decreased to light perception. One week later a dark spot was discovered at the top of the lesion. At the end of the next week, a yellow center of necrosis developed in the nodule. Neighboring insertion of the superior rectus muscle and overlying conjuncti va became necrotic (Fig. 1). The visual acuity had decreased to no light perception, and intraocular pressure was 4 to 7 mm Hg. Because of intolerable pain in this blind eye, the eye was removed Dec. 1, 1975. No implant was used following the enuclea-
Fig. 1 (Hatsuda and Tanaka). Left eye with necro tizing nodular scleritis.
AMERICAN JOURNAL O F OPHTHALMOLOGY 86:710-714, 1978
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BILATERAL NECROTIZING SCLERITIS
tion because of widespread necrosis of the conjunc tiva and Tenon's capsule. The pain had disappeared after the enucleation. The socket healed nicely and a prosthesis was tolerable. The right eye had been relatively quiet except for several episodes of iridocyclitis. The visual acuity of the eye was 6/60 (20/200), on Jan. 12, 1976. The right eye had the first episode of scleritis Jan. 16, 1976. Scleritis was situated nasally, appeared to be deep, and extended from the corneoscleral limbus to the insertion of the medial rectus muscle. Moderate flare and cells were present in the anterior chamber. Intraocular pressure was 5 mm Hg. Increasing cata ract and medial haze obscured the fundus. The visual acuity had decreased to finger counting. The eye was treated mainly with indomethacin supposi tories and supplementarily with retrobulbar or sys temic corticosteroids. After several remissions, blu ish prominent lesions appeared at the insertion of the lateral rectus muscle and adjacent to the superior corneoscleral limbus in June 1976. The cornea be came entirely edematous and was neovascularized around its circumference. The vision decreased to blindness. Three months later two necrotic foci appeared, one superior to the insertion of the lateral rectus muscle and the other just lateral to the inser tion of the superior rectus muscle. The necrotic lesions grew and fused together (Fig. 2). The ocular disease rapidly progressed, with recurrent severe pain and increasing pericorneal injection. The eye exhibited extreme limitation of movement in all directions. The pain was intolerable, and the pa tient was anxious to have the eye removed. The eye was enucleated on Nov. 22, 1976. During surgery, a dense cartilage-like mass was found adherent to the globe and to the loose tissues of the orbit in an equatorial portion nasosuperiorly. The mass seemed to be an extension of inflammatory infiltrates into the loose tissue of the orbit. No implant was used after the enucleation because the indurated ocular socket would not tolerate it.
711
a hard and tender mass occupying the socket. We found a rapidly enlarged mass filling the empty socket. Because of increasing pain, the orbital mass was removed Jan. 10, 1977. The pain disappeared after the surgery. T h e patient had no symptoms of arthritis or any other general disease at her last examination July 2, 1977. RESULTS
Immediately after enucleation, the globes were fixed in 10% formalin solu tion and embedded in paraffin blocks. The left eye was of normal size (Fig. 3). The cornea was normal. The sclera super ior to the corneoscleral limbus surround ing the nodular lesion was three times the normal thickness, and the thickening ex tended to the equator. There was a large area of exudative retinal detachment. High-power microscopic examination of the thickened lesion revealed that it con sisted of a massive chronic inflammatory process, which had some of the character istics of a granulomatous inflammation. The lesion was composed of a central focus of necrosis surrounded by densely arranged lymphocytes, epithelioid cells, and multinucleated giant cells in some areas (Fig. 4). Extensive interruption and necrosis were observed in the scleral col lagen bundles. To a lesser extent, a simi-
A dull pain persisted after enucleation. One week postoperatively, the patient began having a sharp pain in her right ocular socket. Examination showed
Fig. 2 (Hatsuda and Tanaka). Right eye with extensive necrotizing nodular scleritis.
Fig. 3 (Hatsuda and Tanaka). Left eye. Lowpower view of equatorial section. Sclereal thicken ing extends superiorly from corneoscleral limbus to equator. Retina is detached with eosinophilic subretinal fluid. Lens has been removed before parrafin embedding (hematoxylin and eosin, x2).
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Fig. 4 (Hatsuda and Tanaka). Left eye. Highpower view of scleral granulomatous lesion. (L) Dense lymphocytic infiltration, (E) palisading ar rangement of epithelioid cells, (S) interrupted bun dles of scleral collagen, (G) multinucleated giant cell (hematoxylin and eosin, x 190).
lar process was discernible in the inferior sciera. The sciera posterior to the equator was involved, as evidenced by some focal collection of lymphocytes. Iris, ciliary body, and choroid involved in the area of the scleral granuloma showed a diffuse infiltration with lym phocytes and epithelioid cells. Some of the epithelioid cells contained pigment granules in the cytoplasm. Remaining portions of the choroid showed only dif fuse infiltration of lymphocytes. The reti na was completely detached, contained eosinophilic subretinal fluid, and was mildly involved in the inflammatory pro cess. The pigment epithelium was de stroyed in the area of scleral granuloma. The optic nerve was somewhat atrophic and associated with slight gliosis. The right eye was phthitic. The cor nea was involved with superficial and midstromal neovascularization and perivascular lymphocytic infiltration, most marked at the corneoscleral limbus (Fig. 5). The sciera had extensive thick ening and formed a tumor-like mass that occupied the vitreous cavity. High-power microscopic examination of the mass revealed it to be composed of loose scar tissue with massive lymphocytic in-
NOVEMBER, 1978
Fig. 5 (Hatsuda and Tanaka). Right eye. Lowpower view of section of whole eye. Granulamatous mass occupied vitreous cavity. Episclera and cho roid are markedly involved by inflammatory process (hematoxylin and eosin, x2).
filtration, in which epithelioid cells and occasional giant cells were scattered (Fig. 6). Most of the collagen bundles of the sciera were distorted and necrotic. There was little vascularized infiltration in the sciera. Iris, ciliary body, and choroid were disintegrated and diffusely infiltrated by lymphocytes and scattered epithelioid cells. Occasional phagocytosis of pigment granules occurred in the epithelioid and giant cells (Fig. 7). The retina was atroph ic. The optic nerve was also atrophic and gliotic.
Fig. 6 (Hatsuda and Tanaka). Right eye. Highpower view of scleral granuloma. Scleral collagen bundles are distorted and almost missing. Massive lymphocytic infiltration and palisade arrangement of epithelioid cells are seen. No giant cells are present in this area. There is little vascularization (hematoxylin and eosin, x 190).
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Fig. 7 (Hatsuda and Tanaka). Right eye. Highpower view of scleral granuloma near ciliary body. Note epithelioid cells and giant cell containing pigment granules (hematoxylin and eosin, X380).
The cartilage-like mass attached to the sclera and orbital adnexae was histologically confirmed to be composed of fibroadipose tissue markedly infiltrated by granuloma, indicating intraorbital in volvement of the scleral lesion (Fig. 8). DISCUSSION
Necrotizing granulamatous scleritis is a rare ocular disease; necrotizing nodular scleritis, a violent inflammatory reaction usually associated with the somewhat loosely linked group of collagen dis eases, particularly rheumatoid arthritis,
Fig. 8 (Hatsuda and Tanaka). High-power view of intraorbital mass developing among orbital adnexae after enucleation of right eye. It consists of flbroadipose tissue mainly involved by diffuse lymphocytic infiltration (hematoxylin and eosin, x 190).
713
is even rarer. The characteristic histologic appearance of nodular granulomatous scleritis is a central area of necrosis surrounded by a palisading zone of epi thelioid and multinucleated giant cells, a granulomatous lesion most typically seen in a subcutaneous rheumatic nodule. The etiology of the condition is unknown. In this case the pathologic findings were necrotizing nodular scleritis in the left eye and massive granuloma of the sclera in the right eye. Scleral lesions of both eyes began with nodular scleritis, which developed necrosis resembling scleromalacia perforans, but with violent inflammatory symptoms. This strongly suggests that massive granuloma of the sclera is the severe condition of necrotiz ing granulomatous scleritis. Wolter and Bentley, 2 who reported two cases of a combination of scleromalacia perforans and massive granuloma of the sclera, stat ed that differences in the degree of necro sis and scar formation as well as location in the sclera distinguished three condi tions: scleromalacia perforans, necrotiz ing nodular scleritis, and massive granu loma of the sclera. Distinguishing between the three con ditions by their histologic features is difficult. Franceschetti and Bischler 3 sug gested that the term "scleromalacia per forans" be reserved for cases showing indolent necrotic sloughing and a quiet clinical course associated with rheuma toid arthritis, and that the expression "necrotizing nodular scleritis" should be used to designate cases exhibiting a vio lent inflammatory process, generally not associated with rheumatoid arthritis. Sev eral researchers 4 - 6 have supported this suggested differentiation between sclero malacia perforans and necrotizing nodu lar scleritis. Van der Hoeve, 7 who was the first to characterize this dramatic syn drome as a clinical entity and named it scleromalacia perforans, suggested that the cases he described might belong to
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AMERICAN JOURNAL OF OPHTHALMOLOGY
two categories. Our case had no history of rheumatoid arthritis or other collagen dis ease. One of us (T. H.) had encountered a case of scleromalacia perforans in a 37year-old woman with rheumatoid arthritis of six years' duration, whose left eye developed four necrotic nodules, which were resolved in four months. Hogan and Zimmerman 8 did not distinguish between rheumatoid lesions occurring in the sclera with or without a history of rheumatoid arthritis or related disease because of sim ilarity in the histologic features. There may be some exceptions, consisting of a combined occurrence of scleromalacia perforans and massive granuloma of the sclera, as in the cases reported by Wolter and Bentley. 2 However, from our own experience, we prefer to divide necrotizing granulomatous scleritis into sclero malacia perforans and necrotizing nodu lar scleritis. SUMMARY
A 53-year-old woman whose history was noncontributory for rheumatoid ar thritis and collagen disease had bilateral necrotizing inflammation of the sclera. The left eye had necrotizing nodular scle ritis and the right eye, massive granuloma
NOVEMBER, 1978
of the sclera. In the right eye the orbital adnexae were also involved by granulom atous infiltration and developed a mass of fibroadipose tissue simulating an orbital tumor within a week after enucleation. REFERENCES 1. Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthalmology, vol. 8, p . 2. Lon don, Henry Kimpton, 1965, p. 1036. 2. Wolter J. R., and Bentley, M. D.: Scleromalacia perforans and massive granuloma of the sclera. A report of an unusual combination of ocular patholo gy in rheumatoid arthritis. Am. J. Ophthalmol. 51: 71, 1961. 3. Franceschetti, A., and Bischler, V.: La sclerite nodulaire necrosante et ses rapports avec la scleromalacie. Ann. Oculistique 183:737, 1950. 4. Ashton, N., and Hobbs, H. E.: Effect of corti sone on rheumatoid nodules of the sclera (scleroma lacia perforans). Br. J. Ophthalmol. 36:373, 1952. 5. Anderson, B., and Margolis, G.: Scleromalacia. Clinical and pathologic study of a case with consid eration of differential diagnosis, relationship of col lagen disease, and effect of ACTH and cortisone therapy. Am. J. Ophthalmol. 35:917, 1952. 6. Francois, J., Victoria-Troncoso, V., Hanssens, M., and Bacskulin, J.: Scleromalacie perforante et collagenose. Ophthalmologica 159:71, 1969. 7. van der Hoeve, cited by Duke-Elder, S., and Leigh, A. G.: Diseases of the Outer Eye. Cornea and Sclera. In Duke-Elder, S. (ed.): System of Ophthal mology, vol. 8, pt. 2. London, Henry Kimpton, 1965, p. 1037. 8. Hogan, M. J., and Zimmerman, L. E.: Ophthalmic Pathology, 2nd ed. Philadelphia, Saunders, 1962, p. 337.
