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Home , Congenital cataract, Diabetic retinopathy, Esotropia

Br J Ophthalmol 1998;82:1215–1219 1215

LETTERS TO THE EDITOR Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from

Idiopathic in a patient with leucoma, atrophy and hypoplasia, and therapy. His past medical history was signifi- congenital rubella syndrome .2 Keratoconus and hydrops have cant for non- dependent melli- been reported in patients with CRS with a tus, systemic hypertension, hyperlipidaemia, EDITOR,—Keratoconus is very rarely found in history of mental retardation and eye and obesity. patients with congenital rubella syndrome rubbing.1 At presentation, the patient complained of (CRS). The only known aetiology of kerato- The microtrauma associated with eye rub- pain and redness in his left eye. conus associated with CRS is eye rubbing.1 bing is generally thought to be the aetiological was 20/25 in the right eye and light perception We report here a patient with CRS who devel- link between keratoconus and associated in the left eye. Examination of the right eye oped idiopathic keratoconus. systemic and ocular diseases.3-5 All four disclosed only mild diabetic . Slit patients with keratoconus and CRS described lamp examination of the left eye showed 1 CASE REPORT by Boger et al were retarded and vigorously hyperaemia of the , and an intense A 24 year old woman with CRS was examined rubbed and poked their eyes. These authors milky flare obstructing the view of the iris and for progressive visual blurring of 2 years’ postulated that keratoconus and acute corneal the (Fig 1). It was diYcult to detect the hydrops probably resulted from chronic trau- duration in her left eye. The diagnosis of CRS presence of cells in the anterior chamber matising mannerisms common in other men- was based on the maternal history of rash and because of the flare. There were neither tally retarded patients5 and is not specific to fever in the first trimester of , keratic precipitates nor or iris congenital rubella. Thus far, this is the only , and microphthalmia in the right eye, neovascularisation. Intraocular pressures in known aetiology of keratoconus associated bilateral rubella retinopathy, , deaf- both eyes were within normal limits. B-scan with CRS. ness, congenital heart disease. ultrasonography was unremarkable. In the case presented here the keratoconus Available medical records dated from the Results of laboratory tests showed hypergly- appeared to be idiopathic. Indeed, there was time she was 1 year old. At that time, caemia (24 mmol/l), a twofold increase in nor- no history of eye rubbing or atopy, or family extracapsular was performed mal cholesterol levels (15 mmol/l), and a history of keratoconus. To the best of our 22-fold increase in normal triglyceride levels in the right eye. She had in the right knowledge, this report is unique in as far as (38 mmol/l). Results of laboratory examina- eye and poor fixation. Apart from pigmentary idiopathic keratoconus has never been associ- tion including HLA typing, angiotensin con- changes in the fundus, the left eye was normal. ated with CRS. It is likely that in this case verting enzyme, serologies for herpes simplex A follow up examination when she was 15 there is no such association but merely an virus (HSV) and lues, purified protein deriva- documented nystagmus, esotropia, and micro- 67 overlap of two uncommon conditions occur- tive (PPD) skin test, and chest and sacroiliac phthalmia in her right eye, and bilateral ring in the same person. An association rays, were not contributory. A and pre- rubella retinopathy. Visual acuity was RE light x â â between idiopathic keratoconus and CRS may lipoproteinaemia pattern was established by perception; LE 6/9 (with −4.0 −1.0 c × 180). be determined only after other confirmatory serum electrophoresis. Analysis of the aque- Two other examinations at the ages of 18 and cases have been reported in the literature. ous humour of the left eye disclosed no cells, 21 gave similar results. and high levels of proteins (32 g/l), cholesterol On present examination, visual acuity with ANTONIO PINNA FRANCESCO CARTA (3.4 mmol/l), and triglycerides (4.9 mmol/l). spectacles was LE 6/60. revealed Visual acuity recovered at 20/50 and the high, irregular myopic with scis- Institute of , University of Sassari, Sassari, Italy flare resolved after lipidaemia and diabetes soring of the reflex. Central keratometry had been controlled by oral fibrates and insu- showed irregular mires which could not be Correspondence to: Dr Antonio Pinna, Istituto di Clinica Oculistica, Universita degli Studi di Sassari, lin, respectively. Fundus examination of the superimposed; 4 dioptre irregular astigmatism left eye showed mild was found. A conical reflection on the nasal Viale San Pietro 43 A, 07100 Sassari, Italy. Accepted for publication 21 April 1998 including microaneurisms, hard exudates, and http://bjo.bmj.com/ was obtained by shining a penlight many paravascular retinal haemorrhages. Six from the temporal side (Rizzuti’s sign). weeks later, fundus examination of the left eye Bulging of the lower on downgaze was 1 Boger WP III, Petersen RA, Robb RM. Kerato- showed evidence of central retinal venous conus and acute hydrops in mentally retarded also observed (Munson’s sign). occlusion (CRVO). examination revealed an inferiorly located patients with congenital rubella syndrome. Am J Ophthalmol 1981;91:231–3. ectatic protrusion of the central cornea with 2 Wolf SM. The ocular manifestations of congeni- COMMENT reticular anterior stromal scars. As a result, the tal rubella. J Pediatr Ophthalmol 1973;10:101– diagnosis of mild keratoconus was made. 41. Although clinical presentation including pain, 3 Karseras AG, Ruben M. Aetiology of kerato- perilimbal conjunctival hyperaemia, and ante- Treatment with a Softperm contact was on October 2, 2021 by guest. Protected copyright. conus. Br J Ophthalmol 1976;60:522–5. rior chamber flare, was consistent with acute successful and visual acuity was again LE 6/9. 4 Rahi A, Davies P, Ruben M, et al. Keratoconus All other ocular findings were the same as in and coexisting atopic disease. Br J Ophthalmol anterior , many features favoured a earlier observations. 1977;61:761–4. strong relation between the metabolic disor- 5 Pierce C, Eustace P. Acute keratoconus in mon- ders and the occurrence of the flare. These The patient attended a special school for gols. Br J Ophthalmol 1971;55:50–4. the deaf and dumb, learning sign language 6 Feder RS. Noninflammatory ectatic disorders. clues included the milky appearance of the flare, the results of the anterior chamber para- and to read and write. There was no history of In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. St Louis: Mosby-Year Book, 1996: centesis, the lack of response to topical eye rubbing or atopy, or a family history of 1091–106. keratoconus. Serum total IgE (34 IU/ml) and 7 Centers for Disease Control. Rubella and eosinophils (1.1%) were normal. congenital rubella—United States, 1984–1986. MMWR 1987;36:664–75.

COMMENT Congenital rubella produces a spectrum of Pseudouveitis as a manifestation of ocular and systemic abnormalities. hyperlipidaemia during the first trimester of pregnancy is more serious, with the virus disrupting organogen- EDITOR,—We report a case of unilateral esis and diVerentiation. Many fetal tissues are anterior pseudouveitis in a diabetic retino- susceptible to infection leading to the abnor- pathic eye, as a manifestation of hyperlipidae- malities found in the CRS—deafness, cardiac mia. High levels of were detected in the malformations, bone and dental abnormali- aqueous humour and the anterior flare ties, ocular malformations, and mental retar- resolved only after successful control of dation. diabetes and hyperlipidaemia was obtained. “Salt and pepper” retinopathy is the most common ocular disorder; other ocular mani- CASE REPORT Figure 1 Slit lamp photograph of the left eye festations include microphthalmia, strabis- A 44 year old man was referred for anterior showing an intense milky flare in the anterior mus, , transient corneal uveitis not responsive toa5daytopical steroid chamber. 1216 Letters

steroids, and the resolution of the flare only lymphocytic pleocytosis (proteins 0.37 g/l, and Asian indians) and is uncommon in Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from after the control of hyperlipidaemia. white cells 77×106/l, lymphocytes 96%), with- whites.1 Our patient was Portuguese and had To our knowledge, only one case of out oligoclonal bands on electrophoresis. A these clinical features. Most patients are in pseudoendophthalmitis related to hyperlipi- magnetic resonance cerebral scan was normal. their second to fourth decade of life at onset of daemia has been published.1 Both eyes were Suspecting a herpetic infection, aciclovir (900 the disease; nevertheless, VKH syndrome has involved in a patient with bilateral proliferative mg/8 hours), topical steroids, and prednisone also been reported in children and young diabetic retinopathy. Blood-aqueous barrier (1 mg/kg) were administered on the day of adults.1 breakdown has been demonstrated in diabetic admission. To date no certain aetiological factors has patients, especially those presenting with Further investigations revealed that serol- been reported, but taking into account that diabetic retinopathy,2 and in patients with ogy ( (CMV), Epstein–Barr this illness aVects various organs (skin, eye, CRVO.3 In the present case, an underlying virus, herpes simplex I/II, varicella zoster central nervous system) a common aetiologi- mild diabetic retinopathy was diagnosed in virus, rubella virus, , Borrelia cal factor seems to be involved in the both eyes. The unilateral leakage of lipids in burgdorferi, Treponema pallidum, and HIV) was pathogenesis. Among the hypotheses that have the anterior chamber may be related to ipsilat- positive for anti-CMV IgM (ratio: 0.7, normal been formulated, the role of genetic constitu- eral blood-aqueous barrier breakdown follow- 0.6) and IgG (521 units of IgG antibody anti- tion is important. Ohno first discovered, ing an undiagnosed partial vein occlusion or CMV per ml (UA/ml), normal 15) and early among the Japanese population, a link an episode of transient iridocyclitis rapidly CMV antigen was detected in the urine. An between VKH syndrome and cured by topical steroid therapy. anterior chamber tap was performed (the day HLA-DR4/Dw53.2 Other authors have re- THANH HOANG-XUAN of admission, before starting antiviral and ported the presence of antiretinal antibodies DANIELE HANNOUCHE anti-inflammatory therapy); results were posi- directed against photoreceptors and Müller HERVÉ ROBIN tive for a local production of anti-CMV cells in the sera of patients with a VKH MARGOT VAN DER DONK immunoglobulins; the Goldmann–Witmer syndrome.3 Moreover, other studies have JEAN-FRANÇOIS KOROBELNIK coeYcient was 4.47 for CMV, 0.56 for herpes demonstrated the presence of a close relation Department of Ophthalmology, simplex virus, and not calculable for varicella between melanocytes and the presence of Bichat-Claude Bernard Hospital, zoster virus and for Epstein–Barr virus. Viral lymphocytes directed against these cells. Paris, France serologies in the cerebrospinal fluid were These findings tend to suggest the presence of Correspondence to: Thanh Hoang-Xuan, MD, negative. Tests for sarcoidosis and for connec- an autoimmune response and/or hypersensi- Department of Ophthalmology, Bichat-Claude tive tissue disorders were negative. Immu- tivity against melanocytes, leading to the Bernard Hospital, 46, rue Henri Huchard, 75018 noglobulin electrophoresis, quantitative im- development of an inflammation aVecting the Paris, France. munoglobulin levels, PPD skin test, CD4- , the skin, and the meninges resulting Accepted for publication 15 April 1998 CD8 lymphocytes count, C3-C4, and CH50 from a break of tolerance to self antigens. examination were within the normal range. However, the initiating factor responsible of the autoimmune process is still unknown. 1 Robertson DM, Misch DM. Pseudo- Based on the clinical findings, by the caused by intravitreal positive CMV serology, the presence of early Autoimmune disorders are characterised by transudation in association with proliferative CMV antigen in the urine and by the result of the breaking of immunological tolerance to diabetic retinopathy and hyperlipidemia. the anterior chamber tap an acute CMV self antigens after the activation of lym- 1986;6:73–6. phocytes. The exact mechanism by which 2 Moriarty AP, Spalton DJ, Moriarty BJ, et al. infection was diagnosed; aciclovir was discon- Studies of the blood-aqueous barrier in diabetes tinued after 4 days, and we administered the there is a loss of tolerance to self antigens is mellitus. Am J Ophthalmol 1994;117:768–71. more specific drug foscarnet (180 mg/kg) for mostly unknown. Viruses have been impli- 3 Miyake K, Miyake T, Kayazawa F. Blood- 21 days. Topical steroids and oral prednisone cated, as triggers, of this mechanism together aqueous barrier in eyes with retinal vein with other mechanisms such as the presence occlusion. Ophthalmology 1992;99:906–10. (1 mg/kg) were also continued for 1 month at tapering doses. After 3 weeks of treatment, of a genetic predisposition or through the acti- visual acuity returned to 20/20 in both eyes. vation of the cytokine network. Detection of anticytomegalovirus Papilloedema disappeared in both eyes. Two The induction and the development of the antibody synthesis in the anterior months later, viral titres revealed a decrease in autoimmune process may be explained by the chamber in Vogt–Koyanagi–Harada CMV IgM (ratio: 0.5) and an increase of the exposition of ocular tissue antigens to the syndrome CMV IgG to 707 U/ml. Early CMV Ag was immune system after a break of the blood- http://bjo.bmj.com/ no longer detectable in the urine. retinal barrier. It is conceivable that a tissue EDITOR,—Vogt–Koyanagi–Harada (VKH) After 3 months, the patient developed damage, by infectious agents, may render for- syndrome has long been suspected to be the poliosis of her , eyebrows, and scalp eign some non-immunogenic ocular antigens. consequence of autoimmunity or hypersensi- hair. A vitiligo appeared. Fundus examination tivity against melanocytes. Nevertheless, to revealed a diVuse depigmentation aVecting date no certain aetiological factors has been both posterior segments, and areas of chorio- reported. retinal atrophy (Dalen–Fuchs nodules) in the periphery of both fundi (Fig 2A, B). Tests on October 2, 2021 by guest. Protected copyright. CASE REPORT seeking to establish a hearing loss were A previously healthy 30 year old Portuguese unremarkable. HLA typing was positive for woman was admitted complaining of progres- the HLA-DRB1*0701/08. On the basis of the sive visual loss in both eyes, over 2 weeks, clinical evolution, the presence of integumen- associated with headaches and pain on eye tary manifestations and the results of the cer- movements and meningismus. Symptoms ebrospinal tap a VKH syndrome was diag- appeared 24 hours after the beginning of a nosed. flu-like syndrome. The patient had no past history of ocular trauma or surgery. On exam- COMMENT ination, the visual acuity was 20/100 in both VKH syndrome is a bilateral panuveitis eyes. Slit-lamp examination revealed 2+ cells associated with vitiligo, poliosis, auditory signs in both anterior chambers with non- and neurological manifestations (cerebrospi- granulomatous keratic precipitates. Intraocu- nal fluid lymphocytic pleocytosis, meningo- lar pressure was 10 mm Hg in both eyes. encephalitis, hemiparesis). Typically, the pres- There were 1+ cells in the anterior vitreous of ence of these features is variable and might both eyes. Fundus examination revealed a depend on the race of the patient. Clinically, bilateral papilloedema and the fluorescein this syndrome is characterised by a prodromal angiography showed focal areas of leakage at stage (headache, fever) and followed by a the level of the retinal pigment epithelium and panuveitis with retinal oedema and/or multi- staining of the (Fig 1A, B). ple retinal serous detachments. After several The initial clinical examination revealed an weeks of evolution, a depigmentation of the erythrocyte sedimentation rate of 10 mm in integuments and appear. Moreover, Figure 1 Late phase of the fluorescein the first hour (normal range 1–12), a white most of the patients relapse and develop a angiogram, right eye (A) and left eye (B). Focal blood count of 4.2 g/l (normal range 4–11 g/l) chronic recurrent phase of the disease. VKH areas of leakage at the level of the retinal with a normal diVerential count. Chest x ray syndrome has a predilection for darkly pig- pigment epithelium (arrows) and staining of the was normal. A lumbar puncture showed a mented races (Asian, Hispanics, American optic disc. Letters 1217

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VKH syndrome, although it is diYcult to Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from establish the exact role of ubiquitous viruses in specific diseases.10 Our patient had an anti- CMV antibody synthesis in the anterior chamber and an acute CMV infection. This case illustrates the possible implication of CMV in the aetiology of VKH syndrome; nevertheless, other known or unknown viruses may be involved in the pathogenesis of this syndrome in predisposed individuals.

Presented at the Fourth International Symposium on Uveitis, October 1997, Yokohama, Japan. EDOARDO BAGLIVO BÉATRICE ROSSILLION PHILIPPE DE GOTTRAU PETER LEUENBERGER Department of Ophthalmology, Geneva University Hospital Switzerland Correspondence to: Dr Edoardo Baglivo, Clinique d’Ophtalmologie, Hôpital Cantonal Universitaire, Rue Alcide-Jentzer, 22, CH-1205 Geneve. Accepted for publication 21 April 1998

1 Moorthy RS, Inomata H, Rao NA. Vogt- Koyanagi-Harada syndrome. Surv Ophthalmol 1995;39:265–92. 2 Ohno S. Immunological aspects of Behcet’s and Vogt-Koyanagi-Harada’s diseases. Trans Oph- thalmol Soc UK 1981;101(Pt 3):335–41. 3 Chan CC, Palestine AG, Nussenblatt RB, et al. Anti-retinal auto-antibodies in Vogt-Koyanagi- Harada syndrome, Behcet’s disease, and sympa- thetic ophthalmia. Ophthalmology 1985;92: 1025–8. 4 Cohen IR, Young DB. Autoimmunity, microbial immunity and the immunological homunculus. Immunol Today 1991;12:105–10. 5 Singh VK, Yamaki K, Donoso LA, et al. Sequence homology between yeast histone H3 and uveitopathogenic site of S-Antigen: lym- phocyte cross-reaction and adoptive transfer of the disease. J Immunol 1989;142:1512–17. 6 Singh VK, Kalra K, Yamaki K, et al. Molecular mimicry between a uveitopathogenic site of S-antigen and viral peptides. Induction of experimental autoimmune uveitis in Lewis rats. J Immunol 1990;144:1282–7. 7 Zinkernagel R. Immunology taught by viruses. Science 1996;271:173–8. 8 Usui M, Usui N, Goto H. Detection of Epstein- Barr virus by polymerase chain reaction in cerebrospinal fluid from patients with Vogt- Koyanagi-Harada disease. IOVS (suppl) 1991; 32:807. http://bjo.bmj.com/ 9 Bassili SS, Peyman GA, Gebhardt BM, et al. Detection of Epstein-Barr virus DNA by polymerase chain reaction in the vitreous from a patient with Vogt-Koyanagi-Harada syndrome. Retina 1996;16:160–1. 10 Chodosh J, Gan Y, Sixbey J. Detection of Epstein-Barr virus genome in ocular tissues. Ophthalmology 1996;103:687–90.

Bilateral small retinal infiltrates during on October 2, 2021 by guest. Protected copyright. rickettsial infection

EDITOR,—Rickettsiae may cause heterogene- ous retinal manifestations, the most character- istic of which are vascular lesions, periphlebi- tis, retinal haemorrhages and oedema, central retinal thrombosis, and papilloedema.1-5 We present the first case in which rickettsiae led to small retinal white infiltrates. Figure 2 Colour picture of right eye (A) and left eye (B). DiVuse depigmentation aVecting both posterior segments, and areas of chorioretinal atrophy (Dalen–Fuchs nodules) in the periphery of both CASE REPORT fundi. A previously healthy 25 year old woman had a fever up to 40°C of unknown origin during a Another mechanism by which micro- foreign antigens called “molecular mimicry”, trip to Queensland, Australia in August. Five organisms can induce ocular autoimmunity is may induce a cross reaction with one or more days after the onset of fever a generalised by the presence of homology between some self antigens sharing determinants with the maculopapular rash and a black lesion on the sequences of their proteins and the sequence foreign agents; this suggests that infectious forefoot (eschar) were visible, without a of uveitogenic determinants of ocular anti- agents can act as a trigger of organ specific known history of a tick bite. The patient was gens. Recently, it has been shown that there is autoimmunity in predisposed individuals.7 transferred to our hospital with the signs of an amino acid sequence homology between Among presumed triggers that could initiate general illness. Extensive examination for certain bacterial antigens and some autoanti- the immune process, viruses may play a role in infectious disease including gens and between sequences in retinal the pathogenesis of VKH syndrome. Indeed, was initiated. S-antigen and protein from yeast histone H3 various authors have reported the presence of Visual acuity was 20/25 in both eyes. Slit and Escherichia coli, as well as with certain viral Epstein–Barr virus genome in the cerebrospi- lamp examination revealed bilateral conjunc- peptides.4–6 This similarity between self and nal fluid or in the vitreous of patients with tival hyperaemia. Moderate inflammation of 1218 Letters

7 Saah AJ. Rickettsiosis. In: Mandell GL, Douglas the vitreous in both eyes was visible. Fundus thalmic re-examination and angiography 3 Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from examination disclosed bilateral small white weeks after initial presentation revealed im- RG, Bennett JE, eds. Principles and practice of infectious diseases. 3rd ed. New York: Churchill infiltrates within the neurosensory retina, provement; 3 weeks later there was a complete Livingstone 1990:1463–82. practically without involvement of the resolution of retinal dots, and 6 months later 8 Sexton DJ, Dwyer B, Kemp R, et al. Spotted fever choroid, mainly in the posterior pole (Fig 1). findings were normal with no chorioretinal group rickettsial in Australia. Rev Infect Dis 1991;13:876–86. In the right eye, a tiny well defined haemor- scars. 9 Bradford WD, Crocker BP, Tisher CC. Kidney rhage near the temporal inferior artery was lesions in Rocky mountain spotted fever. Am J 1979; :381–92. seen. angiography of both eyes COMMENT Pathol 97 showed punctuate hypofluorescence corre- Based on patient history, clinical signs, and sponding to the white infiltrates (Fig 2), stain- serology we concluded that the illness pre- Progressive outer retinal necrosis in a ing of the optic discs, but no signs of vasculitis. sented was associated with rickettsiae. Within Indocyanine green (ICG) angiogram, visual patient with cutaneous non-Hodgkin’s the genus Rickettsia, small obligate intracellu- field examination, and colour tests were unre- T cell lymphoma (Sézary syndrome) lar bacteria, three groups of antigenically markable in both eyes. related organisms are known: spotted fever, Results of the following laboratory tests EDITOR,—The progressive retinal outer necro- typhus, and scrub typhus.67 Travel history, were pathologic: erythrocyte sedimentation sis syndrome (PORN) is a recognised variant incubation time, and the marked titre increase rate (86 mm in the first hour), platelets (135 of necrotising herpetic retinopathy, presum- of OX19 and OX2 indicate that the infectious 12 g/l), CRP (15 mg/dl), GOT (165 U/l), GPT ably caused by the varicella zoster virus. The agent was of the spotted fever group, most (127 U/l), GGT (49 U/l), and LDH (527 U/l). syndrome is characterised by minimal in- likely which occurs in Abdominal ultrasound sonography revealed Rickettsia australis traocular inflammation, rapidly spreading Queensland, Australia. This rickettsia causes an enlarged spleen (160 mm). The main sero- multifocal deep retinal lesions without vascu- the Queensland tick typhus and its most logical finding was a definite increase of the lar involvement (until late in the disease), prevalent vector is ixodic tick (Ixodes holocy- bilateral involvement in the majority of the Weil–Felix reaction from 1:160 (OX19, OX 8 2), 1:40 (OX K) to 1:1280 (OX 19, OX2) and clus, Ixodes tasmani). A bite by Ixodes tasmani cases, and poor visual prognosis irrespective of 2 1:160 (OX K) within 1 week. Serology for may have been overlooked in the case treatment. So far, the majority of the presented, as, contrary to , the brucella, salmonella, Yersiniaenterocolica, Liste- Ixodes holocyclus described cases were related to HIV disease. former is less apparent during feeding.8 Addi- ria monocytogenes, Mycoplasma pneumoniae, We present a HIV seronegative immunocom- tional tests to specify the infectious agent Francisella tularensis, Mycobacterium tuberculo- promised patient with normal CD4 counts, unfortunately did not reveal any further clari- sis, Coxiella burnetti, Borrelia burgdorferi, who developed PORN and responded well to fication. Treponema pallidum, hepatitis (A, B, C), HIV, aciclovir. rubeola virus, rubella virus, Epstein–Barr In general, rickettsiae invade endothelial virus, cytomegalovirus, herpes simplex I and cells, resulting in destruction of smooth mus- CASE REPORT II, , , cle fibres, thrombosis, and extravasation of A 29 year old man with cutaneous non- Candida albicans Cryptococcus neoformans 1-9 Aspergillus, toxoplasmosis, and chlamydia blood into the tissues. The papilloedema, Hodgkin’s T cell lymphoma (Sézary syn- were all negative. also observed in our patient, is supposedly drome) for 4 years, developed a progressive 1 During antibiotic treatment with doxycy- based on microvessel destruction. Further, loss of vision of his right eye within 5 days. cline 200 mg once daily for 14 days and clari- rickettsiae may cause round cell infiltration in Five weeks previously, he had had a thoracic thromycin 500 mg twice daily for 5 days, all perivascular spaces in lungs, kidneys, brain, zoster that responded well to orally adminis- 69 symptoms subsided and the Weil–Felix reac- and adrenals. This is the first report to dem- tered aciclovir (5 × 800 mg per day). On tion decreased to 1:640 (OX 19, OX2) and onstrate small retinal infiltrates. The diVeren- admission, the visual acuity of the right eye 1:80 (OX K). The visual acuity increased to tial diagnosis of the ocular findings included was 0.4 and a central subcapsular posterior 25/20 in both eyes. Four weeks after this treat- many diseases which were excluded by cataract was present. Intraocular cells were ment all laboratory tests were normal. Oph- serology. The complete resolution without any absent, and remained absent thereafter. Ex- residual scars and normal ICG angiograms tensive central and peripheral oedematous practically excluded . Further, and white necrotic lesions, including the this did not meet the fluorescence- macula were observed with sparing of the angiographic criteria of white dot syndrome, perivascular region (Fig 1). Slight optic http://bjo.bmj.com/ since no hyperfluorescence was observed. In oedema and a few scattered retinal haemor- conclusion, rickettsiae may lead to white dots rhages were also found. The left eye exhibited in the retina and, thus, should be considered no abnormalities. A diagnostic aqueous tap in the diVerential diagnosis. was performed and intravenous aciclovir, 10 JULIUS R LUKAS mg/kg body weight three times per day, was STEFAN EGGER immediately initiated. Treatment with chlo- Department of Ophthalmology and Optometry, rambucil 4 mg and prednisone 22.5 mg daily University Hospital Vienna, Austria for the non-Hodgkin’s lymphoma was contin- 6 BERNHARD PARSCHALK ued. CD4 counts were normal (390×10 /l, on October 2, 2021 by guest. Protected copyright. Department of General Medicine I, Section of Infectious reference range 350–1500×106/l), CD8 Diseases, University Hospital Vienna, Austria counts, however, were low (14.8×106/l) result- MICHAEL STUR ing in high CD4/CD8 ratio (26, reference Figure 1 Fundus photograph of the left eye Department of Ophthalmology and Optometry, range 1.1–2.5). Microparticle enzyme immu- demonstrating multiple white spots in the University Hospital Vienna, Austria noassay (IMX 8B3220, Abbott, CA, USA) posterior pole which were mainly confined to the demonstrated HIV seronegativity. neuroretina without any involvement of the Correspondence to: J R Lukas, Department of choroid. Ophthalmology and Optometry, University of Vienna, A-1090 Vienna, Waehringer Guertel 18-20, Austria. Accepted for publication 15 April 1998

1 Presley GB. Fundus changes in Rocky Mountain spotted fever. Am J Ophthalmol 1969;67:263–7. 2 Francois J. Rickettsiae in ophthalmology. Oph- thalmologica 1968;156:459–72. 3 Donegan EA. Ocular findings in tropical typhus (Tsutsugamushi or Japanese river fever). Br J Ophthalmol 1946;30:11–19. 4 Chamberlain WP. Ocular findings in scrub typhus. Arch Ophthalmol 1952;48:313–32. 5 Font-Creus B, Bella-Cueto F, Espejo-Arenas E, et al. Mediterranean spotted fever: a cooperative study of 227 cases. Rev Infect Dis 1985;7:635– Figure 2 In the fluorescein angiogram these 42. neuroretinal infiltrates blocked the background 6 Berman SJ. Scrub typhus. In: Hoeprich PD, Figure 1 Posterior pole of patient’s right eye fluorescence throughout the whole phase of dye Colin J, Allan R, eds. Infectious diseases. 5th ed. showing multifocal infiltrates 3 days after transit. (left eye, mid-phase, scanning laser Philadelphia: JB Lippincott 1984; chapter 113, starting intravenous aciclovir treatment (10 ophthalmoscope). 983–5. mg/kg body weight). Letters 1219

mained uninvolved until his death (due to res- negative patient following bone marrow trans- Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from piratory failure) at 18 months’ follow up. plantation with poor visual outcome despite Aqueous humour analysis revealed low preventive antiviral treatment; however, this Goldmann–Witmer coeYcients (<3.0) and no patient had low CD4 counts.6 DNA of varicella zoster virus (VZV), herpes Our case demonstrates that PORN may simplex virus, and cytomegalovirus (polymer- develop in HIV seronegative, immunocom- ase chain reaction negative) was detected. promised patients with normal CD4 but low High serum IgG levels (1:8192) against VZV CD8 counts. might have contributed to the low Goldmann– Witmer coeYcients. Sponsors/grants: none. FEY LIENG NJOO COMMENT ANIKI ROTHOVA Our patient was immunocompromised by the ALLEGONDA VAN DER LELIJ cutaneous non-Hodgkin’s lymphoma and the F C Donders Institute, Department of Ophthalmology, accompanying therapy, but in contrast with University Hospital Utrecht, Utrecht, Netherlands Figure 2 Fundus photograph of patient’s right AIDS patients CD4 counts were, albeit low, eye 2.5 weeks after initiation of intravenously Correspondence to: F L Njoo, MD, F C Donders administered aciclovir showing optic atrophy, within the normal limits. Characteristically for Institute, Department of Ophthalmology, University resolution of infiltrates, and attenuation of the Sézary syndrome however, the CD4/CD8 Hospital Utrecht, PO Box 85500, 3508 GA vessels. Poor contrast quality is caused by ratio was elevated due to low counts of CD8 Utrecht, Netherlands. associated cataract; the vitreous was clear. cells. Compared with HIV seropositive pa- Accepted for publication 21 April 1998 2 After 3 days, resolution of the peripheral tients or AIDS patients with PORN, our 1 Forster DJ, Dugel PU, Frangieh GT, . lesions started but progression into the poste- patient responded well to aciclovir treatment. et al Furthermore, notwithstanding the optic atro- Rapidly progressive outer retinal necrosis in the rior pole occurred. acquired immunodeficiency syndrome. Am J disclosed retinal necrotic lesions with perivas- phy, he retained useful visual acuity and Ophthalmol 1990;110:341–8. cular sparing and areas of resolution with did not develop. As 2 Engstrom RE, Holland GN, Margolis TP, et al. suggested earlier, the degree1 and probably the The progressive outer retinal necrosis syn- arteriolar attenuation. Because of the develop- drome. A variant of necrotizing herpetic retin- ment of phlebitis along the cannula intrave- type of immune deficiency might be responsi- opathy in patients with AIDS. Ophthalmology nously administered aciclovir was temporarily ble for the diVerences in clinical picture, and 1994;101:1488–502. × therapy outcome. 3 Duker JS, Shakin EP. Rapidly progressive outer switched to oral aciclovir (5 800 mg per day) retinal necrosis in the acquired immunodefi- from day 8 until day 13. Nevertheless, resolu- Five cases with PORN in HIV seronegative, ciency syndrome. Am J Ophthalmol 1991;111: tion of the necrotic lesions continued, result- but otherwise immunocompromised, indi- 255–6. ing in opaque chorioretinal scarring and optic viduals were reported. In contrast with our 4 Rochat C, Herbort CP. Syndrome de nécrose rétinienne aiguë (ARN/BARN/PORN). Casuis- atrophy by the end of the second week (Fig 2). patient, none of these cases displayed all typi- tique lausannoise, revue de la littérature et nou- After a month patient was discharged with a cal features of PORN as described originally velle hypothèse physiopathogénique. Klin maintenance therapy (5 × 800 mg aciclovir in AIDS patients1: two patients had moderate Monatsbl Augenheilkd 1994;204:440–9. or pronounced intraocular inflammation,34 5 Feinman MC, Friedberg MA, Schwartz JC, et al. orally per day). Visual acuity in the right eye Central nervous system herpesvirus infection in stabilised at 0.2 for the following 11 months; and the third patient showed ophthalmologi- systemic lupus erythematosus: diagnosis by however, the patient developed a mature cata- cal lesions resembling bilateral acute retinal endoretinal biopsy (case report). J Rheumatol ract, leading to light perception only. Retinal necrosis syndrome.5 Two other cases were 1993;20:1058–61. 4 6 Lewis JM, Nagae Y, Tano Y. Progressive outer detachment did not occur (shown by ultra- only briefly mentioned but not described. retinal necrosis after bone marrow transplanta- sound biomicroscopy) and the left eye re- Recently, PORN was described in a HIV sero- tion. Am J Ophthalmol 1996;122:892–5. http://bjo.bmj.com/ on October 2, 2021 by guest. Protected copyright.