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Symblepharon in Sarcoidosis
SYMBLEPHARON IN SARCOIDOSIS ALLAN FLACH, San Francisco,
M.D.
California
Cicatricial involvement of the conjunc tiva including symblepharon is rare. The purpose of this case presentation is to document the association of symblepha ron with systematic sarcoidosis and, thus, add ocular sarcoidosis to the causes of cicatricial conjunctivitis. CASE REPORT A 38-year-old hlack man complained of shortness of breath and ephiphora of four months' duration. Five years previously a diagnosis of sarcoidosis had been confirmed by biopsy of a scrotal mass and nasal polyps. He complained then of nasal conges tion, painless swelling of the parotid glands, and a scrotal mass. An opthalmological evaluation had shown follicles involving the superior and inferior palpebral conjunctiva. The lacrimal glands were enlarged, visible, and associated with mild keratitis sicca. There was no uveal or retinal involvement. Biopsies of the left lacrimal gland and left inferior palpebral conjunctiva showed sarcoidosis. The pa tient was treated with artificial tears. The lacrimal gland biopsy did not appear to affect the progression of the keratitis sicca in the left eye. The patient developed skin lesions of the face, arms, and legs over the next five years. Biopsy of the lesions had shown sarcoid, and they were treated with intralesional injections of triamcinolone acetonide with good results. When I saw the patient, active skin lesions were confined to his arms and legs, and consisted of purplish papules which coalesced into welldemarcated, geographic areas. The lesions on the face (Fig. 1) appeared flat and were hyperpigmented from treatment with intralesional injections of tri amcinolone acetonide. The eyelids were thickened, and a cicatrical entropion with trichiasis of the lower eyelids was evident (Fig. 2). There was no evidence of cranial nerve abnormality. The best corrected visual acuity was R.E.: 6/12 (20/40), and L.E.: 6/6 (20/20). The conjunctiva re vealed follicles and cicatrization of both upper and lower palpebral conjunctivae of both eyes (Fig. 3).
From the University of California San Francisco School of Medicine, and Veterans Administration Hospital, San Francisco, California. Reprint requests to Allan Flach, M.D., Depart ment of Ophthalmology, Veterans Administration Hospital, 4150 Clement St., San Francisco, CA 94121. 210
Fig. 1 (Flach). Facial lesion after local corticosteroid injections.
Symblepharon was noted bilaterally, but was most prominent on the right eye (Fig. 4). The lacrimal glands were not visible. Examination of the cornea revealed a linear, blotchy epithelial keratitis involving the exposed area of the inferior cornea. An early arcus senilis was noted. There was no evidence of uveal or retinal involvement. Results of laboratory studies revealed mild eosinophilia, a mildly elevated sedimentation rate, and a slightly elevated serum protein. The patient showed anergy to tuberculin of both intermediate and full strength, but the skin test was reactive for dermatophytin. Chest roentgenograms revealed a hilar adenopathy without evidence of peripheral fibrosis, although results of pulmonary function studies were consistent with obstructive lung disease. The pa tient had a weakly positive rheumatoid factor, and a slightly elevated glucose tolerance curve. Results of the following laboratory tests were unremarkable: serologic tests for syphilis, lupus erythematosus cell preparation, antinuclear antibodies, serum creatinine, and bilirubin levels. An electrocardiogram showed sinus tachycardia, occasional premature ventricular contractions, and low QRS voltage. X-ray films of the upper gastrointestinal tract re vealed a gastric ulcer. Giemsa-stained conjunctival scrapings showed few eosinophils and rare Leber cells, but no evidence of epithelial inclusions. Re peat conjunctival scrapings on two subsequent occa sions failed to show eosinophils, but demonstrated a few polymorphonuclear leukocytes, and Staphylococcus aureus was cultured from the specimen. The patient was treated with artificial tears, soft contact lenses and excision of the symblepharon (which revealed only fibrosis); he then showed marked improvement in signs and symptoms (Fig. 5). The initial complaint of epiphora, which seemed
AMERICAN JOURNAL O F OPHTHALMOLOGY 85: 210-214, 1978
Fig. 2 (Flach). Right eye. Cicatricial entropion with trichiasis.
Fig. 3 (Flach). Left eye. Cicatrization of upper palpebral conjunctiva.
Fig. 4 (Flach). Right eye. Lower eyelid symblepharon.
Fig. 5 (Flach). Right eye. Partially excised lower eyelid symblepharon.
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SYMBLEPHARON IN SARCOIDOSIS
related to the associated mucus deficiency, disap peared. Glucocorticoids were administered systemically, which alleviated the patients shortness of breath. Antacids were given for the gastric ulcer. A repeat roentgenogram showed healing. DISCUSSION
As early as 1931 Blegvad 1 described cicatrizing conjunctivitis caused by sarcoidosis. Crick's 2 review of the ocular manifestations in sarcoidosis showed a photograph suggestive of cicatrization, but the caption described a mass of con fluent follicles. In the present case I found no evidence of a previous membranous, or pseudomembranous conjunctivitis, or chemical injury. 3 The left inferior palpebral conjunctival biopsy may have contributed to the conjunctival scarring on the left lower eyelid, but there was marked cicatricial involvement of all four eyelids. Further more, the greatest symblepharon forma tion was present on the right eye. The cicatrization was not suggestive of that seen in chlamydial infestations, nor could I find evidence of Chlamydia.4 There was no evidence of collagen disease such as scleroderma, systemic lupus erythematosis, or polyarteritis nodosa. The patient had no signs of related dermatologic prob lems such as dermatitis herpetiformis, epidermolysis bullosa, erythoderma ichthyosiforme, acne rosacea, or exfoliative dermatitis. The patient's age, the course of the conjunctival involvement, and the ab sence of dermatologic and oral involve ment, other than sarcoid lesions, are un like benign mucous membrane pemphigoid. 5 Mucus membrane pemphigoid is considered an antibody-dependent dis ease on the basis of constant, local eosinophilia. 7 In this case the single dem onstration of conjunctival eosinophils, unconfirmed by repeat scrapings, sug gests only intermittent eosinophilia, unlike mucus membrane pemphigoid. 6
213
The eosinophil is not specific for benign mucus membrane pemphigoid, but is characteristic of allergic inflammation and has been reported in association with Staphylococcus aureus.6 Although this patient lacked signs of staphylococcic blepharoconjunctivitis, 5. aureus was cultured from his eyelids and conjuncti va, and he may be in a carrier state. 8 Results of all laboratory studies, in cluding cultures and stains of all biopsy specimens and multiple sputum samples, revealed no evidence of tuberculosis. Ocular involvement occurs in 63% of histologically proven cases of sarcoid osis. 9 Keratoconjunctivitis sicca is fre quent. 1 0 1 1 This patient initially had Mikulicz's syndrome (enlargement of the lacrimal gland and parotid gland) 1 2 and early keratoconjunctivitis sicca. Although keratoconjunctivitis sicca and uveitis are the most frequent ocu lar findings in sarcoidosis, conjunctival involvement occurs in 2% to 25%. 2 * 3 Sarcoid nodules simulating follicle for mation in the conjunctiva are well doc umented, and biopsy of the involved area is a simple, relatively safe means of obtaining tissue for histologic diag nosis. 1 3 SUMMARY
A 38-year-old black man had developed sarcoidosis, confirmed by biopsy five years earlier. He then developed skin le sions and, at age 38, follicles and cicatri zation of the upper and lower palpebral conjunctivae of both eyes. This patient had keratoconjunctivitis sicca, lacrimal gland enlargement, and cicatrization of the conjunctiva with symblepharon. Bi opsies of the lacrimal gland, conjunctiva, skin, nasal polyps, and epididymis all showed sarcoidosis. REFERENCES 1. Blegvad, O.: Boeck's sarcoid der conjunctiva. Acta Ophthalmol. 9:180, 1931.
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AMERICAN JOURNAL OF OPHTHALMOLOGY
2. Crick, R. P., Hoyle, C , and Smellie, H.: The eyes in sarcoidosis. Br. J. Ophthalmol. 45:461, 1961. 3. Hogan, M. J.: Conjunctivitis with membrane formation. Am. J. Ophthalmol. 30:1495, 1947. 4. Mordhorst, C. H. and Dawson, C : Sequelae of neonatal inclusion conjunctivitis and associated dis ease in parents. Am. J. Ophthalmol. 71:861, 1971. 5. Chalkley, T. H. F.: Chronic cicatricial conjunc tivitis. Am. J. Ophthalmol. 67:526, 1969. 6. Thygeson, P.: The cytology of conjunctival exudates. Am. J. Ophthalmol. 29.1499, 1946. 7. Allansmith, M. R., and O'Connor, G. R.: Immunoglobulins: Structure function and relation to the eye. Survey Ophthalmol. 14:367, 1970. 8. Thygeson, P., and Kimura, S. J.: Chronic con
FEBRUARY 1978
junctivitis. Trans. Am. Acad. Ophthalmol. Otolarygol. 67:4: 494, 1963. 9. James, D. G.: Ocular sarcoidosis. Acta Med. Scand. 176:203, 1964. 10. Jones, B. R., and Stevenson, C. J.: Keratoconjunctivitis sicca due to sarcoidosis. Br. J. Ophthal mol. 41:153, 1957. 11. Sjcjgren, H. and Block, K. J.: Keratoconjunctivitis sicca and the Sjogren syndrome. Survey Oph thalmol. 16:145, 1971. 12. Mikulicz, J.: Concerning a peculiar symmetri cal disease of the lacrimal and salivary glands. Med. Classics 2:137, 1937. 13. Crick, R. P., Hoyle, C , and Mather, G.: Conjunctival biopsy in sarcoidosis. Br. Med. J. 4: 1180, 1955.
OPHTHALMIC MINIATURE
In Sushruta's classic Hindu treatise, the operation for cataract is given, including the advise to pierce the right eye with the left hand, and vice versa. If my tentative dates are right, this was an Indian, not an Alexandrian discovery. Guido Majno, The Healing Hand Cambridge, Mass., Harvard University Press, 1975
M.D.
California
Cicatricial involvement of the conjunc tiva including symblepharon is rare. The purpose of this case presentation is to document the association of symblepha ron with systematic sarcoidosis and, thus, add ocular sarcoidosis to the causes of cicatricial conjunctivitis. CASE REPORT A 38-year-old hlack man complained of shortness of breath and ephiphora of four months' duration. Five years previously a diagnosis of sarcoidosis had been confirmed by biopsy of a scrotal mass and nasal polyps. He complained then of nasal conges tion, painless swelling of the parotid glands, and a scrotal mass. An opthalmological evaluation had shown follicles involving the superior and inferior palpebral conjunctiva. The lacrimal glands were enlarged, visible, and associated with mild keratitis sicca. There was no uveal or retinal involvement. Biopsies of the left lacrimal gland and left inferior palpebral conjunctiva showed sarcoidosis. The pa tient was treated with artificial tears. The lacrimal gland biopsy did not appear to affect the progression of the keratitis sicca in the left eye. The patient developed skin lesions of the face, arms, and legs over the next five years. Biopsy of the lesions had shown sarcoid, and they were treated with intralesional injections of triamcinolone acetonide with good results. When I saw the patient, active skin lesions were confined to his arms and legs, and consisted of purplish papules which coalesced into welldemarcated, geographic areas. The lesions on the face (Fig. 1) appeared flat and were hyperpigmented from treatment with intralesional injections of tri amcinolone acetonide. The eyelids were thickened, and a cicatrical entropion with trichiasis of the lower eyelids was evident (Fig. 2). There was no evidence of cranial nerve abnormality. The best corrected visual acuity was R.E.: 6/12 (20/40), and L.E.: 6/6 (20/20). The conjunctiva re vealed follicles and cicatrization of both upper and lower palpebral conjunctivae of both eyes (Fig. 3).
From the University of California San Francisco School of Medicine, and Veterans Administration Hospital, San Francisco, California. Reprint requests to Allan Flach, M.D., Depart ment of Ophthalmology, Veterans Administration Hospital, 4150 Clement St., San Francisco, CA 94121. 210
Fig. 1 (Flach). Facial lesion after local corticosteroid injections.
Symblepharon was noted bilaterally, but was most prominent on the right eye (Fig. 4). The lacrimal glands were not visible. Examination of the cornea revealed a linear, blotchy epithelial keratitis involving the exposed area of the inferior cornea. An early arcus senilis was noted. There was no evidence of uveal or retinal involvement. Results of laboratory studies revealed mild eosinophilia, a mildly elevated sedimentation rate, and a slightly elevated serum protein. The patient showed anergy to tuberculin of both intermediate and full strength, but the skin test was reactive for dermatophytin. Chest roentgenograms revealed a hilar adenopathy without evidence of peripheral fibrosis, although results of pulmonary function studies were consistent with obstructive lung disease. The pa tient had a weakly positive rheumatoid factor, and a slightly elevated glucose tolerance curve. Results of the following laboratory tests were unremarkable: serologic tests for syphilis, lupus erythematosus cell preparation, antinuclear antibodies, serum creatinine, and bilirubin levels. An electrocardiogram showed sinus tachycardia, occasional premature ventricular contractions, and low QRS voltage. X-ray films of the upper gastrointestinal tract re vealed a gastric ulcer. Giemsa-stained conjunctival scrapings showed few eosinophils and rare Leber cells, but no evidence of epithelial inclusions. Re peat conjunctival scrapings on two subsequent occa sions failed to show eosinophils, but demonstrated a few polymorphonuclear leukocytes, and Staphylococcus aureus was cultured from the specimen. The patient was treated with artificial tears, soft contact lenses and excision of the symblepharon (which revealed only fibrosis); he then showed marked improvement in signs and symptoms (Fig. 5). The initial complaint of epiphora, which seemed
AMERICAN JOURNAL O F OPHTHALMOLOGY 85: 210-214, 1978
Fig. 2 (Flach). Right eye. Cicatricial entropion with trichiasis.
Fig. 3 (Flach). Left eye. Cicatrization of upper palpebral conjunctiva.
Fig. 4 (Flach). Right eye. Lower eyelid symblepharon.
Fig. 5 (Flach). Right eye. Partially excised lower eyelid symblepharon.
VOL. 85, NO. 2
SYMBLEPHARON IN SARCOIDOSIS
related to the associated mucus deficiency, disap peared. Glucocorticoids were administered systemically, which alleviated the patients shortness of breath. Antacids were given for the gastric ulcer. A repeat roentgenogram showed healing. DISCUSSION
As early as 1931 Blegvad 1 described cicatrizing conjunctivitis caused by sarcoidosis. Crick's 2 review of the ocular manifestations in sarcoidosis showed a photograph suggestive of cicatrization, but the caption described a mass of con fluent follicles. In the present case I found no evidence of a previous membranous, or pseudomembranous conjunctivitis, or chemical injury. 3 The left inferior palpebral conjunctival biopsy may have contributed to the conjunctival scarring on the left lower eyelid, but there was marked cicatricial involvement of all four eyelids. Further more, the greatest symblepharon forma tion was present on the right eye. The cicatrization was not suggestive of that seen in chlamydial infestations, nor could I find evidence of Chlamydia.4 There was no evidence of collagen disease such as scleroderma, systemic lupus erythematosis, or polyarteritis nodosa. The patient had no signs of related dermatologic prob lems such as dermatitis herpetiformis, epidermolysis bullosa, erythoderma ichthyosiforme, acne rosacea, or exfoliative dermatitis. The patient's age, the course of the conjunctival involvement, and the ab sence of dermatologic and oral involve ment, other than sarcoid lesions, are un like benign mucous membrane pemphigoid. 5 Mucus membrane pemphigoid is considered an antibody-dependent dis ease on the basis of constant, local eosinophilia. 7 In this case the single dem onstration of conjunctival eosinophils, unconfirmed by repeat scrapings, sug gests only intermittent eosinophilia, unlike mucus membrane pemphigoid. 6
213
The eosinophil is not specific for benign mucus membrane pemphigoid, but is characteristic of allergic inflammation and has been reported in association with Staphylococcus aureus.6 Although this patient lacked signs of staphylococcic blepharoconjunctivitis, 5. aureus was cultured from his eyelids and conjuncti va, and he may be in a carrier state. 8 Results of all laboratory studies, in cluding cultures and stains of all biopsy specimens and multiple sputum samples, revealed no evidence of tuberculosis. Ocular involvement occurs in 63% of histologically proven cases of sarcoid osis. 9 Keratoconjunctivitis sicca is fre quent. 1 0 1 1 This patient initially had Mikulicz's syndrome (enlargement of the lacrimal gland and parotid gland) 1 2 and early keratoconjunctivitis sicca. Although keratoconjunctivitis sicca and uveitis are the most frequent ocu lar findings in sarcoidosis, conjunctival involvement occurs in 2% to 25%. 2 * 3 Sarcoid nodules simulating follicle for mation in the conjunctiva are well doc umented, and biopsy of the involved area is a simple, relatively safe means of obtaining tissue for histologic diag nosis. 1 3 SUMMARY
A 38-year-old black man had developed sarcoidosis, confirmed by biopsy five years earlier. He then developed skin le sions and, at age 38, follicles and cicatri zation of the upper and lower palpebral conjunctivae of both eyes. This patient had keratoconjunctivitis sicca, lacrimal gland enlargement, and cicatrization of the conjunctiva with symblepharon. Bi opsies of the lacrimal gland, conjunctiva, skin, nasal polyps, and epididymis all showed sarcoidosis. REFERENCES 1. Blegvad, O.: Boeck's sarcoid der conjunctiva. Acta Ophthalmol. 9:180, 1931.
214
AMERICAN JOURNAL OF OPHTHALMOLOGY
2. Crick, R. P., Hoyle, C , and Smellie, H.: The eyes in sarcoidosis. Br. J. Ophthalmol. 45:461, 1961. 3. Hogan, M. J.: Conjunctivitis with membrane formation. Am. J. Ophthalmol. 30:1495, 1947. 4. Mordhorst, C. H. and Dawson, C : Sequelae of neonatal inclusion conjunctivitis and associated dis ease in parents. Am. J. Ophthalmol. 71:861, 1971. 5. Chalkley, T. H. F.: Chronic cicatricial conjunc tivitis. Am. J. Ophthalmol. 67:526, 1969. 6. Thygeson, P.: The cytology of conjunctival exudates. Am. J. Ophthalmol. 29.1499, 1946. 7. Allansmith, M. R., and O'Connor, G. R.: Immunoglobulins: Structure function and relation to the eye. Survey Ophthalmol. 14:367, 1970. 8. Thygeson, P., and Kimura, S. J.: Chronic con
FEBRUARY 1978
junctivitis. Trans. Am. Acad. Ophthalmol. Otolarygol. 67:4: 494, 1963. 9. James, D. G.: Ocular sarcoidosis. Acta Med. Scand. 176:203, 1964. 10. Jones, B. R., and Stevenson, C. J.: Keratoconjunctivitis sicca due to sarcoidosis. Br. J. Ophthal mol. 41:153, 1957. 11. Sjcjgren, H. and Block, K. J.: Keratoconjunctivitis sicca and the Sjogren syndrome. Survey Oph thalmol. 16:145, 1971. 12. Mikulicz, J.: Concerning a peculiar symmetri cal disease of the lacrimal and salivary glands. Med. Classics 2:137, 1937. 13. Crick, R. P., Hoyle, C , and Mather, G.: Conjunctival biopsy in sarcoidosis. Br. Med. J. 4: 1180, 1955.
OPHTHALMIC MINIATURE
In Sushruta's classic Hindu treatise, the operation for cataract is given, including the advise to pierce the right eye with the left hand, and vice versa. If my tentative dates are right, this was an Indian, not an Alexandrian discovery. Guido Majno, The Healing Hand Cambridge, Mass., Harvard University Press, 1975