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Show Journal of Neiim- Ophthalmology 18( 4): 268- 269, 1998. & 1998 Lippincolt Williams & Wilkins, Philadelphia The Many Faces of Sarcoidosis James D. Izer, B. S., Andrew G. Lee, M. D., Ramon L. Font, M. D., and James R. Patrinely, M. D. A 42- year- old African- American woman saw her internist in December 1996 with symptoms of binocular vertical diplopia, headache, and a bulging, painful right eye ( RE). Two weeks before, she had undergone an uneventful left lower jaw root canal. Examination revealed proptosis RE and a moderate abduction deficit RE. Complete blood count, serum chemistry, thyroid function studies, antinuclear antibody, and rheumatoid factor were normal. A magnetic resonance ( MR) scan of the head and orbits revealed a soft tissue density behind the right globe, involving the medial rectus muscle RE. She was diagnosed with orbital pseudotumor and treated with 60 mg oral prednisone daily. The diplopia, headache, and eye pain improved but did not resolve. The patient was referred to the neuro- ophthalmology service in February 1997. Visual acuity was 20/ 20 in both eyes. The pupils were isocoric and reacted normally to light with no relative afferent pupillary defect. Hertel exophthalmometry measured 21 mm in both eyes. Motility examination showed a moderate abduction deficit RE, and there was a 10- prism diopter esotropia in primary gaze. Slit lamp examination showed conjunctival injection and an inferior corneal micropannus RE. The remainder of the ocular examination was unremarkable. An erythematous, indurated papular lesion with a central umbilication on the right malar eminence was present ( Fig. 1). A second MR scan of the orbits revealed soft tissue density within the orbital fat surrounding the globes bilaterally, with inhomogeneous enhancement after administration of gadolinium- diethylenetriaminepentaacetic acid ( DTPA). Angiotensin- converting enzyme level, Lyme titer, syphilis serology results, antineutrophil cytoplasmic antibody levels, and chest radiograph were normal. A biopsy specimen obtained from the malar skin Manuscript received February 10, 1998; accepted June 12, 1998. From Baylor College of Medicine, ( J. D. I.), and the Departments of Pathology ( R. L. F.), Ophthalmology ( R. L. F., J. R. P, A. G. L), Neurology ( A. G. L.), and Neurosurgery ( A. G. L.), Baylor College of Medicine; and the Division of Neurosurgery, M. D. Anderson Cancer Center, Houston, Texas, U. S. A. Supported in part by a grant from Research to Prevent Blindness, New York, New York, U. S. A. Address correspondence and reprint requests to Andrew G. Lee, M. D., 6565 Fannin St. NC- 205, Baylor College of Medicine, Department of Ophthalmology, Houston, TX 77030, U. S. A. FIG. 1. An indurated lesion is present on the right malar eminence. lesion showed confluent epithelioid granulomas with multinucleated giant cells and scattered lymphoplasma-cytic infiltrates ( Fig. 2). Small foci of fibrinoid necrosis were present. A gallium scan showed abnormal uptake in the lacrimal glands bilaterally. Systemic sarcoidosis has many different skin manifestations. The papular form ( also known as miliary sarcoidosis) has symptoms of small papules on the face, eyelids, and neck, often with lichenification and pitting. Lupus pernio displays violaceous, smooth, shiny plaques FIG. 2. Skin specimen obtained in biopsy of the right malar lesion showing multiple noncaseating granulomas and numerous multinucleated giant cells. 268 SARCOIDOSIS 269 on the ears, forehead, nose, and digits. Erythema nodosum exhibits erythematous, warm, tender nodules up to 5 cm in diameter, usually appearing on the shins and the face. Other skin forms include hypopigmentation, psoriasiform, and ulcerative sarcoid ( 1). The incidence of skin involvement in sarcoidosis has been reported to be 25% ( 2,3) and ocular involvement occurs in 27% to 40% of patients ( 2- 4). As many as 54% of ocular sarcoid patients have skin involvement at the time of diagnosis, usually manifested as erythema nodosum or skin plaques ( 5). This case is unusual because the diagnosis of sarcoid with presumed orbital involvement was made on analysis of a skin biopsy specimen despite a normal angiotensin-converting enzyme level and chest radiograph. Ophthalmologists should be aware of the dermatologic manifestations of sarcoid and the utility of skin biopsy in diagnosis. REFERENCES 1. Arnold HL, Odom RB, James WD. Sarcoidosis. In: Lamsback W, ed. Andrews' diseases of the skin. 8th ed. Philadelphia: WB Saunders, 1990: 840- 46. 2. James DG, Sharma OP. Extralhoracic sarcoidosis. Proc R Soc Med 1967; 60: 992- 4. 3. Obenauf CD, Shaw HE, Sydnor CF, Klintworth GK. Sarcoidosis and its ophthalmic manifestations. Am J Ophthalmol 1978; 86: 648- 55. 4. Nielsen RH. Ocular sarcoidosis. Arch Ophthalmol I959; 61: 657- 63. 5. James DG. The diagnosis and treatment of ocular sarcoidosis. Acta Med Scand Sitppl 1964: 425: 203- 08. J Neum- Oplitlwlnml, Vol. 18, No. 4. 1998 |
