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Show J. Clill. Ncuro-opiltilllllllo/. 5: 177-179,1985 Il) 1985 Raven Press, New York Retinopathy of Dermatomyositis BRUCE H. COHEN, M.D. LYN A. SEDWICK, M.D. RONALD M. BURDE, M.D. Abstract A recent case of childhood dermatomyositis with striking retinopathy is reported. The patient's vision was initially at the 20/80 to 20/100 level with associated macular edema and extensive cottonwool infarction of the retina. Visual recovery to the level of 20/20 in both eyes was achieved following treatment with high-dose corticosteroids. .Dermat.omyositis is a systemic inflammatory disease ot unknown etiology affecting primarily striated muscle, skin, and various connective tissues in the body. Weakness of proximal limb, shoulder, and pelvic girdle muscles is characteristic of the disease and, without skin changes, constitutes polymyositis. Dermatomyositis encompasses polymyositis associated with dermatologic manifestations such as localized or diffuse erythema, maculopapular rash, scaling, eczemoid dermatitis, and occasionally exfoliative dermatitis. I - 3 One of the most striking features of dermatomyositis is a violaceous or heliotrope eruption of the eyelids, skin of the bridge of the nose, cheeks, or forehead. Rheumatoid arthritis, rheumatic fever, scleroderma, or systemic lupus erythematosus may be present conjointly in patients with dermatomyositis or polymyositis, and associated malignancy has been found in 5-8% of cases in a large series. 3 Approximately 8-22% of patients with dermatomyositis are children. 3 Childhood dermatomyositis is characterized by a Widespread vasculitis and late development of calcinosis (subcutaneous calcification).1.3 Features of collagen vascular disease like arthropathy and Raynaud's phenomenon are seen infrequently, and associated malignancy is rare in children. 3 From the Departments of Ophthalmology (B.H.C.. L.AS, R.M.B.) and Neurology and Neurological Surgery (R.M.B.), Washington University School of Medicine, SI. Louis, Missouri. Dr. Sedwick is currently in private practice in Orlando, Florida. Write for reprints to: R. M. Burde, M.D., Department of Ophthalmology-Box 8096, 660 South Euclid Avenue, SI. Louis, MO 63110, U.S.A. September 1985 Ophthalmologic manifestations of dermatomyositis have been reported. 4- 11 Retinopathy characteristically includes diffuse cotton-wool spots and deep and superficial retinal hemorrhages. 5•7- Y Postinflammatory optic atrophy, choroidal infarction, uveitis, secondary glaucoma, extraocular muscle dysfunction, scleritis, and episcleritis have been reported. 5.6,1O.11 A recently observed case of childhood dermatomyositis with neurologic signs and decreased vision is reported here . Case Report A 5-year, lO-month-old boy was in his usual state of good health until 1 month prior to admission when he was noted to be "sleepy" all the time. Two and one-half weeks prior to admission he complained of leg pain and refused to walk up and down stair,:,. He had decreased appetite with weight loss of 6 Ib and appeared depressed and withdrawn. By the time of admission to St. Louis Children's Hospital on January 31, 1983, he had stopped ambulating. His past medical history was remarkable for five episodes of pneumonia, the last one in January 1982. He had a history of mumps 1 year prior to admission. The patient was a product of a normal delivery, and his early development was normal. The family history revealed endogenous depression but was negative for neuromuscular disease. The patient was taking no medications. The general physical examination revealed decreased strength in the legs, distally more than proximally, and minimal hypertrophy of the calves without muscle tenderness. The admission laboratory workup included an erythrocyte sedimentation rate of 25 mm/h, hem"atocrit of 42.3%, and white blood cell count of 14,800 with 91 polymorphonuclear cells and 5 bands. Urinalysis showed a specific gravity of 1.023 with trace protein. Ophthalmologic consultation was obtained on February 3, 1983. His best-corrected visual acuity was 20/80 in the right eye and 20/100 in the left eye. External examination showed a violaceous hue to the left upper eyelid. He had no afferent pupillary defect, and visual fields 177 Retinopathy of Dermatomyositis were full to confrontation. Examination of the ocular fundi revealed numerous cotton-wool spots ,lnd rare retinal hemorrhages especially prnmilll'nt in the posterior poll' of both eyes, as well as ll1acubr edema (Fig. I). Subsequent laboratory values included a creatinine plHlsphokinase of 2,84lJ lUlL, a negative antinuclear antibody, slightly decreased serum protein of 4.6 g/dL, normal immunoglobulin kVt'ls, negative anti-DNA, negative RA latex, normal Raji cell complexes, complement C-3 slightly low at 89 mg%, C-4 normal at 36 mg%, and total complement (CH-50) normal at 177 UI mL. Electromyography was consistent with a myositis. Muscle biopsy revealed no perivascular infiltrate, but scattered necrotic fibers were present consistent with an inflammatory myopathy. The patient was started on 250 mg of intra-venous methylprednisolone every 12 h for 5 days and then was switched to oral prednisone. The patient had gradual resolution of his fundus abnormalities with subsequent increase in his vision by March 3, 1983, to 20/20 in both eyes. There was no evidence of optic atrophy or uveitis (Fig. 2). This patient's striking ischemic retinopathy led to his extensive workup for an underlying collagen vascular disease. The diagnosis of dermatomyositis was most consistent with the clinical and laboratory findings. Discussion First noted by Bruce,4 in 1938, retinal involvement in dermatomyositis was reviewed by Harrison and associates. J Children are more likely than adults to develop retinal manifestations of Cbl Figure 1. Fundus photugr.Jphs obt.Jin~d Juring lh~ .Jeule phase ot the illness a" , . . capillary telangiectasia. and multiple cotton-wool spots b. M 't" d' 'f: Right tundus demonstrating diSC edema, . ., . agm Ie View a nght macula d . uI . 1 cotton-wool spots, a single hemorrhage, and areas of resum . .'. r area emonstrating m tip e d: similar findings in the left eye. ' p. ed axoplasmlc staSIS at the temporal border of the disc. c and 178 Journal of Clinical Neuro-ophthalmology Cohen et al. (al (b) Figure 2. Fundu~ phl'tl'~r"rh~ l,bt,lin..d I month following tilt' initiation of curtico~t..roid th..rapy. a: Right fundus demllO~ trating r"~l,lutillO l,f di~c ..dema. coltl'"-wool ~pot~. and a,onal ~tasi~. b: Similar findings in the left eye. dermatom\'ositis because of the systemic vasculitis ass~Kiated with the childhood form. 3.'i Bitnum and co-\\'orkersl~ emphasized the frequency of vascular lesions in children, finding that one-fourth of the children die with gastrointestinal hemorrhage and perforation. 3.1~ Systemic manifestations of dermatomyositis frequently appear to respond to high-dose corticosteroid therapy. However, there have been no controlled studies that demonstrate the efficacy of corticosteroids. ~.3 Immunosuppressive agents, particularly methotrexate and azathioprine, are used in patients whose response to corticosteroid therapy is inadequate.~ Muscle biopsies in patients with childhood dermatomyositis show widespread endothelial damage and degeneration in arterioles, capillaries, and veins with associated platelet thrombi. Similar events occurring in the arterioles and capillaries of the optic disc, retina, and choriocapillaris explain the clinical findings of ischemic infarction of the ganglion cell layer (cotton-wool spots and disc swelling) and of the retinal pigment epithelium (macula: pigmentary changes and Elschnig's spots).' Interestingly, arteriolar-venular shunts have been noted as a late consequence of childhood dermatomyositis. 5 Visual recovery is variable and may be limited by factors such as postinflammatory optic atrophy, residual mascular pigmentary changes, and ischemic infarction of the choriocapillaris. 13 Acknowledgment This work was supported in part by a grant from Research to Prevent Blindness, Inc., New York, New York (Department of Ophthalmology). September 1985 References 1. Bohan, A., and Peter, J.: Polymyositis and dermatomyositis. First of two parts. N. Ellg/. f. Med. 292: 344-347, 1975. 2. Bohan, A., and Peter, J.: Polymyositis and dermatomyositis. Second of two parts. N. Ellg/. I Mt'd. 292: 403-407, 1975. 3. Bradley, W.: Inflammatory disease of muscle. In Tt!xt/Jook of Rht!ll/1wtO/Ogy, Kelly, W., Harris, E., Rudley, S., and Sledge, c., Eds. W.B. Saunders, Philadelphia, 1981, pp. 1255-1276. 4. Bruce, G. M.: Retinitis and dermatomyositis. TrailS. Am. Opht/lIl/mo/. Soc. 36: 282-297, 1938. 5. Harrison, S. M., FrenkeL M., Grossman, B., and Matalon, R.: Retinopathv in childhood dermatomyositis. Am. f. Ophtha/mo/. 76: 786-790, 1973. 6. Hollenhorst, R. W., and Henderson, J. W.: The ocular manifestations of the difiuse collagen diseases. Am. f. Med. Sci. 221: 211-222,19:;1. 7. Liebman,S., and Cook, c.: Retinopathy with dermatomyositis. Arch. 0l'hlh'1/1/1l1/. 74: 70ol-705. 19b5 H. Klien, B. A.: Comments on colton-wool lesilll1 lli the retina. Am. ,. Opht/wII/1l1/. 59: 17-23. 1%5. 9. Lisman, J. V.: DermatonWllsitis with rl'tiI1llpathy. Arch. Ophtlwlml1/. 37: 155-159. lYol7. 10. Munro, 5.: Fundus appearance in a case oi acute dermatomyositis. Br. ,. 0p!lth,I/I/II)/' 013: 5olS-558. 195<). 1I. TUlwinen. E., .1I1d R,llId.1S0j,1. R.: P,'ik.ilt,dermalomyositis with relin,11 hl'l11l1rrhages and second: uv glallclll11,l. At'I,l l )phth'111I/t1/. -13: 6b9- b72, 1965. 12. Bitnum, 5., D.1l'schnl'r. C. W., Jr., Travis, L. B., Dodgl', W. r .. ,lI1d Hl1pps, H. c.. Dermatomyl) sitis. ,. rediatl'. 64: 101-131, 19M. 13. Crawford, J. 13.: Pathologic correlates in ophthalmoscl1py. In Clillical Ophthll/molosy, 'lO/. 3, Duane, T. D., and Jaeger, E. A., Eds. Harper & Row, Philadelphia, 1982, Chap. 7, p. 3. 179 |
