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Conjunctival deposits as an initial manifestation of sarcoidosis
BRIEF REPORTS Conjunctival Deposits as an Initial Manifestation of Sarcoidosis Stefan Dithmar, MD, George O. Waring III, MD, FACS, FRCOphth, Todd A. Goldblum, MD, and Hans E. Grossniklaus, MD PURPOSE: To report conjunctival deposits as an initial manifestation of sarcoidosis. METHODS: Case reports. RESULTS: Two patients with bilateral bulbar conjunctival white deposits underwent conjunctival biopsy. The biopsy specimens showed noncaseating granulomas with prominent Schaumann bodies. Serum angiotensin-converting enzyme levels were increased in both patients. The first patient had abnormal results of pulmonary function tests. Sarcoidosis was diagnosed in both patients. CONCLUSIONS: Conjunctival deposits may represent the initial clinical manifestation of sarcoidosis. (Am J Ophthalmol 1999;128:361–362. © 1999 by Elsevier Science Inc. All rights reserved.)
S
performed. Histologic examination of the biopsy specimen showed scattered noncaseating granulomas consisting of epithelioid histiocytes, lymphocytes, and multinucleated giant cells. In many areas, the epithelioid histiocytes and giant cells surrounded Schaumann bodies (Figure 1, bottom). All tests were normal, except a pulmonary function test that showed decreased vital capacity and the serum angiotensin-converting enzyme level was increased. ● CASE 2:
An 8-year-old Hispanic girl was evaluated for bilateral, focal, multiple, white, discrete, breadcrumb-like, conjunctival deposits in her bulbar conjunctiva. Her remaining ophthalmic examination was normal, with no evidence of
ARCOIDOSIS IS AN INFLAMMATORY DISEASE OF UN-
known cause, characterized by noncaseating granulomas.1,2 Granulomatous inflammation of the conjunctiva constitutes the second most common ophthalmic finding in sarcoidosis.3 ● CASE 1:
A 19-year-old white woman was evaluated for bilateral conjunctival deposits. Slit-lamp microscopy showed multiple bilateral, focal, discrete, white, breadcrumb-like, bulbar conjunctival deposits that measured approximately 0.25 mm in diameter (Figure 1, top). The remaining ophthalmic examination was normal, and there was no evidence of uveitis or perivasculitis. A biopsy of the left conjunctiva was Accepted for publication March 30, 1999. From the Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia (S.D., G.O.W., H.E.G.), and Goldblum Family Eye Care Center, Albuquerque, New Mexico (T.A.G.). This work was supported in part by a grant from the Gertrud-Kusen Foundation, Hamburg, Germany (Di 98/99), an unrestricted departmental grant from Research to Prevent Blindness, Inc, New York, New York, and departmental core grant EY06030 from the National Eye Institute, National Institutes of Health, Bethesda, Maryland. Inquiries to Hans E. Grossniklaus, MD, LF Montgomery Ophthalmic Pathology Laboratory, Emory Eye Center, 1365 Clifton Rd NE, Atlanta, GA 30322; e-mail: [email protected] 0002-9394/99/$20.00
©
1999 BY
FIGURE 1. Case 1. (Top) Numerous white, breadcrumb-like deposits (arrows) are present in the bulbar conjunctiva. (Bottom) The conjunctival biopsy specimen contains laminated concretions (asterisk, Schaumann bodies) surrounded by epithelioid cells and giant cells (hematoxylin and eosin, ⴛ63 before 71% reduction).
ELSEVIER SCIENCE INC. ALL
RIGHTS RESERVED.
361
2. Muller-Quernheim J. Sarcoidosis: clinical manifestations, staging and therapy (part II). Respir Med 1998;92:140 –149. 3. Kataria S, Trevathan GE, Holland JE, Kataria YP. Ocular presentation of sarcoidosis in children. Clin Pediatr 1983;22: 793–797. 4. Margo CE, Grossniklaus HE. Granulomatous conjunctivitis. In: Margo CE, Grossniklaus HE, editors. Ocular histopathology: a guide to differential diagnosis. Philadelphia: WB Saunders, 1991:8 –15. 5. Kirkpatrick CJ, Curry A, Bisset DL. Light and electron microscopic studies on multinucleated giant cells in sarcoid granuloma: new aspects of asteroid and Schaumann bodies. Ultrastruct Pathol 1988;12:581–597.
Oral Cimetidine (Tagamet) for Recalcitrant, Diffuse Conjunctival Papillomatosis
FIGURE 2. Case 2. The conjunctival biopsy specimen displays Schaumann bodies (asterisk) surrounded by epithelioid histiocytes and giant cells (hematoxylin and eosin, ⴛ63 before 71% reduction).
Carol L. Shields, MD, Margaret R. Lally, MD, Arun D. Singh, MD, Jerry A. Shields, MD, and Thaddeus Nowinski, MD
uveitis or perivasculitis. A conjunctival biopsy was performed, and histologic examination showed focal epithelioid histiocytes and multinucleated giant cells surrounding Schaumann bodies (Figure 2). The specimen was interpreted to represent noncaseating granulomatous inflammation with associated Schaumann bodies. A systemic evaluation was normal except the patient had an increased serum angiotensin-converting enzyme level. Sarcoidosis was diagnosed. Multiple, translucent, pale yellow conjunctival nodules have frequently been described as the first clinical sign of sarcoidosis.3 These nodules represent noncaseating granulomas. Our patients had discrete, white, breadcrumb-like deposits scattered over the bulbar conjunctiva of both eyes. The presence of giant cells in granulomas associated with sarcoidosis is variable. Giant cells may contain Schaumann bodies, asteroid bodies, or both in sarcoidosis and other disease processes.4 Schaumann bodies are concentrically arranged lamellae of basophilic, electron-dense material that form within the giant cell and are eventually extruded.5 Schaumann bodies measure approximately 100 m in diameter, contain iron and calcium, and are found in approximately 4% of noncaseating granulomas in sarcoidosis.5 Schaumann bodies accounted for the white conjunctival deposits in our patients. Both of our patients had normal chest x-rays. Our first patient had abnormal pulmonary function tests consistent with sarcoidosis. An increased serum angiotensin-converting enzyme level in both of our patients supports the diagnosis of sarcoidosis. Our patients demonstrate that white conjunctival deposits may represent the initial clinical manifestation of sarcoidosis.
PURPOSE:
To report the efficacy of cimetidine for treatment of conjunctival papillomatosis. METHODS: An 11-year-old boy had an 8-year history of diffuse conjunctival papillomas, treated previously with standard measures of excisional biopsy and cryotherapy. He developed spontaneous conjunctival bleeding and diffuse tumor recurrence over the entire conjunctival surface. Oral cimetidine liquid (30 mg/kg/day) was prescribed. RESULTS: Within 2 months, dramatic tumor regression was noted, with nearly complete resolution by 4 months. The patient had no local or systemic side effects from the medication. CONCLUSIONS: Oral cimetidine possesses immune modulation properties and may be useful in some cases of virus-induced conjunctival papillomatosis. (Am J Ophthalmol 1999;128:362–364. © 1999 by Elsevier Science Inc. All rights reserved.)
C
Accepted for publication July 16, 1999. From the Ocular Oncology Service (C.L.S., A.D.S., J.A.S.) and the Oculoplastics Department (T.N.), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, and St Margaret’s Memorial Hospital (Dermatology) (M.R.L.), Pittsburgh, Pennsylvania. This work was supported by the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (J.A.S.), the Macula Foundation, New York, New York (C.L.S.), and the Eye Tumor Research Foundation, Philadelphia, Pennsylvania. Data from this paper were presented at the International Congress of Ocular Oncology, May 2, 1999, Philadelphia, Pennsylvania. Inquiries to Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.
REFERENCES
1. Forrester JV. Sarcoidosis and inflammatory eye disease. Br J Ophthalmol 1992;76:193–194.
362
AMERICAN JOURNAL
ONJUNCTIVAL PAPILLOMA IS AN ACQUIRED TUMOR OF
the surface epithelium of the eye. In children, it is often caused by human papillomavirus infection1 that occurs by mother-to-infant transmission at birth through an infected vagina. Some children manifest the tumors
OF
OPHTHALMOLOGY
SEPTEMBER 1999
S
performed. Histologic examination of the biopsy specimen showed scattered noncaseating granulomas consisting of epithelioid histiocytes, lymphocytes, and multinucleated giant cells. In many areas, the epithelioid histiocytes and giant cells surrounded Schaumann bodies (Figure 1, bottom). All tests were normal, except a pulmonary function test that showed decreased vital capacity and the serum angiotensin-converting enzyme level was increased. ● CASE 2:
An 8-year-old Hispanic girl was evaluated for bilateral, focal, multiple, white, discrete, breadcrumb-like, conjunctival deposits in her bulbar conjunctiva. Her remaining ophthalmic examination was normal, with no evidence of
ARCOIDOSIS IS AN INFLAMMATORY DISEASE OF UN-
known cause, characterized by noncaseating granulomas.1,2 Granulomatous inflammation of the conjunctiva constitutes the second most common ophthalmic finding in sarcoidosis.3 ● CASE 1:
A 19-year-old white woman was evaluated for bilateral conjunctival deposits. Slit-lamp microscopy showed multiple bilateral, focal, discrete, white, breadcrumb-like, bulbar conjunctival deposits that measured approximately 0.25 mm in diameter (Figure 1, top). The remaining ophthalmic examination was normal, and there was no evidence of uveitis or perivasculitis. A biopsy of the left conjunctiva was Accepted for publication March 30, 1999. From the Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia (S.D., G.O.W., H.E.G.), and Goldblum Family Eye Care Center, Albuquerque, New Mexico (T.A.G.). This work was supported in part by a grant from the Gertrud-Kusen Foundation, Hamburg, Germany (Di 98/99), an unrestricted departmental grant from Research to Prevent Blindness, Inc, New York, New York, and departmental core grant EY06030 from the National Eye Institute, National Institutes of Health, Bethesda, Maryland. Inquiries to Hans E. Grossniklaus, MD, LF Montgomery Ophthalmic Pathology Laboratory, Emory Eye Center, 1365 Clifton Rd NE, Atlanta, GA 30322; e-mail: [email protected] 0002-9394/99/$20.00
©
1999 BY
FIGURE 1. Case 1. (Top) Numerous white, breadcrumb-like deposits (arrows) are present in the bulbar conjunctiva. (Bottom) The conjunctival biopsy specimen contains laminated concretions (asterisk, Schaumann bodies) surrounded by epithelioid cells and giant cells (hematoxylin and eosin, ⴛ63 before 71% reduction).
ELSEVIER SCIENCE INC. ALL
RIGHTS RESERVED.
361
2. Muller-Quernheim J. Sarcoidosis: clinical manifestations, staging and therapy (part II). Respir Med 1998;92:140 –149. 3. Kataria S, Trevathan GE, Holland JE, Kataria YP. Ocular presentation of sarcoidosis in children. Clin Pediatr 1983;22: 793–797. 4. Margo CE, Grossniklaus HE. Granulomatous conjunctivitis. In: Margo CE, Grossniklaus HE, editors. Ocular histopathology: a guide to differential diagnosis. Philadelphia: WB Saunders, 1991:8 –15. 5. Kirkpatrick CJ, Curry A, Bisset DL. Light and electron microscopic studies on multinucleated giant cells in sarcoid granuloma: new aspects of asteroid and Schaumann bodies. Ultrastruct Pathol 1988;12:581–597.
Oral Cimetidine (Tagamet) for Recalcitrant, Diffuse Conjunctival Papillomatosis
FIGURE 2. Case 2. The conjunctival biopsy specimen displays Schaumann bodies (asterisk) surrounded by epithelioid histiocytes and giant cells (hematoxylin and eosin, ⴛ63 before 71% reduction).
Carol L. Shields, MD, Margaret R. Lally, MD, Arun D. Singh, MD, Jerry A. Shields, MD, and Thaddeus Nowinski, MD
uveitis or perivasculitis. A conjunctival biopsy was performed, and histologic examination showed focal epithelioid histiocytes and multinucleated giant cells surrounding Schaumann bodies (Figure 2). The specimen was interpreted to represent noncaseating granulomatous inflammation with associated Schaumann bodies. A systemic evaluation was normal except the patient had an increased serum angiotensin-converting enzyme level. Sarcoidosis was diagnosed. Multiple, translucent, pale yellow conjunctival nodules have frequently been described as the first clinical sign of sarcoidosis.3 These nodules represent noncaseating granulomas. Our patients had discrete, white, breadcrumb-like deposits scattered over the bulbar conjunctiva of both eyes. The presence of giant cells in granulomas associated with sarcoidosis is variable. Giant cells may contain Schaumann bodies, asteroid bodies, or both in sarcoidosis and other disease processes.4 Schaumann bodies are concentrically arranged lamellae of basophilic, electron-dense material that form within the giant cell and are eventually extruded.5 Schaumann bodies measure approximately 100 m in diameter, contain iron and calcium, and are found in approximately 4% of noncaseating granulomas in sarcoidosis.5 Schaumann bodies accounted for the white conjunctival deposits in our patients. Both of our patients had normal chest x-rays. Our first patient had abnormal pulmonary function tests consistent with sarcoidosis. An increased serum angiotensin-converting enzyme level in both of our patients supports the diagnosis of sarcoidosis. Our patients demonstrate that white conjunctival deposits may represent the initial clinical manifestation of sarcoidosis.
PURPOSE:
To report the efficacy of cimetidine for treatment of conjunctival papillomatosis. METHODS: An 11-year-old boy had an 8-year history of diffuse conjunctival papillomas, treated previously with standard measures of excisional biopsy and cryotherapy. He developed spontaneous conjunctival bleeding and diffuse tumor recurrence over the entire conjunctival surface. Oral cimetidine liquid (30 mg/kg/day) was prescribed. RESULTS: Within 2 months, dramatic tumor regression was noted, with nearly complete resolution by 4 months. The patient had no local or systemic side effects from the medication. CONCLUSIONS: Oral cimetidine possesses immune modulation properties and may be useful in some cases of virus-induced conjunctival papillomatosis. (Am J Ophthalmol 1999;128:362–364. © 1999 by Elsevier Science Inc. All rights reserved.)
C
Accepted for publication July 16, 1999. From the Ocular Oncology Service (C.L.S., A.D.S., J.A.S.) and the Oculoplastics Department (T.N.), Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania, and St Margaret’s Memorial Hospital (Dermatology) (M.R.L.), Pittsburgh, Pennsylvania. This work was supported by the Paul Kayser International Award of Merit in Retina Research, Houston, Texas (J.A.S.), the Macula Foundation, New York, New York (C.L.S.), and the Eye Tumor Research Foundation, Philadelphia, Pennsylvania. Data from this paper were presented at the International Congress of Ocular Oncology, May 2, 1999, Philadelphia, Pennsylvania. Inquiries to Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.
REFERENCES
1. Forrester JV. Sarcoidosis and inflammatory eye disease. Br J Ophthalmol 1992;76:193–194.
362
AMERICAN JOURNAL
ONJUNCTIVAL PAPILLOMA IS AN ACQUIRED TUMOR OF
the surface epithelium of the eye. In children, it is often caused by human papillomavirus infection1 that occurs by mother-to-infant transmission at birth through an infected vagina. Some children manifest the tumors
OF
OPHTHALMOLOGY
SEPTEMBER 1999