Short Communication Ophthalmic Res 2008;40:101–104 DOI: 10.1159/000113889
Received: September 29, 2007 Accepted after revision: June 25, 2007 Published online: January 25, 2008
Choroidal Sclerosis in Localized Scleroderma (Morphea en Plaque) S. Milenkovic a L. Petrovic c D. Risimic a N. Kosanovic-Jakovic a V. Jaksic d Z. Djakovic b M. Stojkovic a D. Risovic c Lj. Ivankovic a M. Ivancevic-Milenkovic a Institutes of a Ophthalmology and b Dermatovenereology, University Clinical Center of Serbia, c Department of Ophthalmology, Clinical Hospital Center Zvezdara, Belgrade, d Department of Ophthalmology, Medical Faculty, Kosovska Mitrovica, Serbia
Key Words Morphea ⴢ Morphea en plaque ⴢ Localized scleroderma ⴢ Choroidal sclerosis
Abstract Plaque morphea is a superficial type of morphea (localized scleroderma) which is characterized by various fibrotic areas of the dermis without systemic features. We present a 63year-old man with morphea en plaque. The skin on his forearms and feet was taut, thickened and hidebound with scattered telangiectatic changes. Autoantibody profile was obtained and only ANA were positive (1:80). The patient had a decreased vision in the only functional, left eye. Our case is specific because the patient negated any kind of health problem, meaning the morphea and visual deterioration were of outstanding importance for him. Choroidal sclerosis and fundus appearance was extremely impressive and, to our knowledge, this is the first report of such unique case of ocular involvement in the literature. Copyright © 2008 S. Karger AG, Basel
Scleroderma (systemic sclerosis) is a multiorganic, multistage disease of unknown etiology [1]. The clinical picture is dominated by vascular insufficiency (abnormality in small arterioles and capillaries) [2]. Skin changes (dermal sclerosis) are frequently accompanied by vis© 2008 S. Karger AG, Basel 0030–3747/08/0402–0101$24.50/0 Fax +41 61 306 12 34 E-Mail
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ceral involvement (kidneys, lungs, digestive tract, heart) so the disease can be fatal (progressive fibrosis in multiple organs) [1]. Localized fibrosing disorder – morphea – is a rare condition that frequently begins in childhood, and is characterized by various fibrotic areas of the dermis, subcutis or underlying tissue and bone, similar to the skin involvement of systemic sclerosis, but without systemic features. The clinical course of the disease is usually benign at first, but sometimes significant complications occur, especially in disseminated morphea [3]. Case Report A 63-year-old man was referred to us due to decreased vision in the only functional, left eye, which lasted more than several months. The other eye was not functional and irrelevant for observation due to the retinal detachment surgery that had been performed 10 years ago after a severe injury. An examination showed that visual acuity in the left eye was 6/12 (with +3.50 Dsph) and intraocular pressure was 16 mm Hg. The patient had localized scleroderma – morphea en plaque type – according to Mayo Clinic classification system of morphea [3] from early childhood. Raynaud’s phenomenon was present. The skin on the forearms and feet was taut, thickened and hidebound with scattered telangiectatic changes (fig. 1). His mouth aperture was slightly narrower than normal. Clinical findings and investigations on lungs, heart, kidneys, digestive tract, etc. were within normal limits. As it was reported in the recent publications, neurologic involvement is one of the most frequent ex-
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tracutaneous manifestations of localized scleroderma, specifically in the childhood [4]. Cerebral MRI, which was also performed to exclude underlying neurological conditions, was normal. Autoantibody profile was obtained and only ANA were positive (1:80). Antibodies to single-stranded DNA, histones, anticentromere and anti-SCL-7o, as markers of systemic sclerosis [4], were negative. It is important that autoantibodies, particularly ANA are seen frequently in localized scleroderma. Some observation suggest that ANA may be a prognostic indicator of the progression of localized forms of scleroderma to systemic disease [5]. Ophthalmological examinations of the left eye demonstrated no eyelid, conjunctival or corneal involvement, with normal tear production, but a discrete cortical cataract was observed. Fundus examination showed large sharply demarcated fields of choroidal sclerosis (fig. 2). Visual field defects corresponded with fundal changes, dark adaptation was relatively unimpaired, but an electroretinogram was subnormal. Fluorescein angiography was specific (fig. 3), demonstrating the large fields of choroidal sclerosis, along with capillary nonperfusion on retinal circulation. Ocular ultrasound (fig. 4) and retinal optical coherent tomography were also performed and suggested retinal and choroidal thinning (sclerosis; fig. 5).
Discussion
Fig. 1. The skin on forearms and feet is taut, thickened and hidebound with scattered telangiectatic changes.
Retinal and choroidal vasculature would be ideal for the observation of generalized arteriolar and capillary pathology in scleroderma. The retinal and choroidal microvascular changes are important because systemic sclerosis is practically a disease of the small arteries [6–10]. A
Fig. 2. Fundus examination shows large sharply demarcated fields of choroidal sclero-
sis.
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Fig. 3. A fluorescein angiogram of the left eye shows sharply circumscribed areas of choroidal sclerosis. Marked areas of capillary nonperfusion on retinal circulation are also visible.
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Fig. 5. Retinal optical coherent tomography showing retinal and choroidal thinning (sclerosis).
Fig. 4. A and B ultrasonography showing choroidal sclerosis.
basic question remains after numerous studies. Is the small choroidal and retinal arterial system involved in systemic sclerosis since the small vessels of other organs are involved [1]? We present the unusual but very suggestive existence of choroidal sclerosis along with capillary nonperfusion on retinal circulation in a middle-aged patient with localized scleroderma (morphea en plaque). Reported retinal manifestations of scleroderma from the former literature usually included hemorrhages, localized edema, hard exudates, cotton wool spots or vascular occlusions [8–10]. The retinal vascular changes were similar to those in malignant hypertension. Fluorescein angiography in previous reports revealed only mild retinal hyperfluorescence in isolated cases [10]. Our case is specific because the paChoroidal Sclerosis in Localized Scleroderma
tient negated any kind of health problem, meaning the morphea and visual deterioration were of outstanding importance for him. Choroidal sclerosis and fundus appearance was extremely impressive and, to our knowledge, this is the first report of such a unique case of ocular involvement in the literature. In addition, in previous reports the most common ocular findings were inflammatory changes (uveitis, episcleritis, keratitis), glaucoma, xerophthalmia (due to a fibrotic process involving the anterior segment and lacrymal gland) [4], or restrictive ophthalmopathy [11], ptosis, pseudo-oculomotor palsy and retrobulbar pain [12]. Such ocular manifestations were mostly associated with the linear type of localized scleroderma. According to our knowledge, there are no published data on the en plaque type of morphea with associated ocular involvement, such as choroidal sclerosis. The problem with the left eye started only several months before the patient decided to be examined by the ophthalmologist. Differential diagnosis of other possible conditions leading to choroidal sclerosis is complex. We excluded the possibility of disease from the group known as white dot chorioretinal inflammatory syndromes: acute multifocal placoid pigment epitheliopaty (AMPPE), serpiginous choroiditis, multifocal choroididis with panuveitis, birdshot retinochoroidopathy (vitiliginous choOphthalmic Res 2008;40:101–104
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rioretinitis) multiple evanescent white dot syndrome (MEWDS), punctate inner choroidopathy and subretinal fibrosis syndrome. Although there is a similarity in the fundus appearance of these conditions, fluorescein angiography clearly and precisely leads to correct diagnosis. There are many other important facts to be considered: AMPPE affects young people, it is commonly bilateral, the lesions are characteristic, mainly at the level of the retinal pigment epithelium. Serpiginous choroidopathy usually extends from the edge of the optic disc and leads to choroidal scars and atrophy (not sclerosis!). Multifocal uveitis with panuveitis occurs mostly in women and the lesions are ‘punched out’ grey-white in color with sharp margins. Uveitis is always present. MEWDS affects mostly young female patients and specific small lesions are like
photocoagulation scars. Birdshot retinochoroidopathy affects middle-aged women with large, broad lesions which evolve into yellowish scars. Vitreous inflammation is severe. Finally, diffuse subretinal fibrosis affects young women and recurrent inflammation is very severe with massive submacular fibrous scars at the end. Sympathetic ophthalmia was excluded because inflammatory signs were absent. The picture of circumscribed sharply demarcated zones of choroidal sclerosis without inflammatory signs is so unusual and typical that possibly represents the manifestation of localized scleroderma. We consider, from an ophthalmologic point of view, that this case might contribute to a further understanding of the enormously complicated mosaic of systemic sclerosis.
References 1 Schwab IR: Scleroderma; in Gold DH, Weingeist TA (eds): The Eye in Systemic Disease. Philadelphia, JB Lippincott, 1990, pp 67–69. 2 Campbell PM, LeRoy EC: Pathogenesis of systemic sclerosis: a vascular hypothesis. Sem Arthritis Rheum 1975;4:351–368. 3 Peterson LS, Nelson AM, Su WP: Classification of morphea (localized scleroderma). Mayo Clin Proc 1995;70:1068–1076. 4 Zulian F, Vallongo C, Woo P, et al: Localized scleroderma in childhood is not just a skin disease. Arthritis Rheum 2005; 52: 2873– 2881.
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5 Birdi N, Laxer RM, Thorner P, Fritzler MJ, Silverman ED: Localized scleroderma progressing to systemic disease. Case report and review of the literature. Arthritis Rheum 1993;36:410–415. 6 Artlett CM, Smith JB, Jimenez SA: New perspectives on the etiology of systemic sclerosis. Mol Med Today 1999;5:74–78. 7 Connolly MK: Scleroderma. Dermatol Ther 2001;14:81–94. 8 Grennan DM, Forrester J: Involvement of the eye in SLE and scleroderma. A study using fluorescein angiography in addition to clinical ophthalmic assessment. Ann Rheum Dis 1977;36:152–156.
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9 West RH, Barnett AJ: Ocular involvement in scleroderma. Br J Ophthalmol 1979;63: 845– 847. 10 Serup L, Serup J, Hagdrup H: Fundus fluorescein angiography in generalised scleroderma. Ophthalmic Res 1987;19:303–308. 11 Campbell WW, Bajandas FJ: Restrictive ophthalmopathy associated with linear scleroderma. J Neuroophthalmol 1995: 15:95–97. 12 Obermoser G, Pfausler BE, Linder DM, Sepp NT: Scleroderma en coup de sabre with central nervous system and ophthalmologic involvement: treatment of ocular symptoms with interferon gamma. J Am Acad Dermatol 2003;49:543–546.
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