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Year : 2017  |  Volume : 4  |  Issue : 2  |  Page : 147-149

Neuroretinitis with vitiligo: A rare case report

Department of Ophthalmology, Dr. Yashwant Singh Parmar Government Medical College, Sirmour, Himachal Pradesh, India

Date of Web Publication14-Mar-2017

Correspondence Address:
Dr. Anubhav Chauhan
Pine Castle, Near Mist Chamber, Khalini, Shimla - 171 002, Himachal Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/cjhr.cjhr_105_16

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Optic neuritis (ON) is an acute inflammatory disorder of the optic nerve and is characterized by a unilateral sudden visual loss in the affected eye, often accompanied by periocular pain. We report a classical case of neuroretinitis (a subtype of ON) with vitiligo in a 52-year-old female. Vitiligo is associated with a few ocular disorders, and neuroretinitis is not one of them. Autoimmune etiologies are associated with both the diseases. To the best of our knowledge and after extensive literature search, this is probably the first reported case of neuroretinitis with vitiligo.

Keywords: Neuroretinitis, steroid, vitiligo

How to cite this article:
Chauhan A, Gupta L. Neuroretinitis with vitiligo: A rare case report. CHRISMED J Health Res 2017;4:147-9

How to cite this URL:
Chauhan A, Gupta L. Neuroretinitis with vitiligo: A rare case report. CHRISMED J Health Res [serial online] 2017 [cited 2021 Jun 23];4:147-9. Available from: https://www.cjhr.org/text.asp?2017/4/2/147/201984

  Introduction Top

Optic neuritis (ON) is an inflammatory condition which affects the optic nerve.[1] ON is classified into three entities, namely, papillitis, retrobulbar neuritis, and neuroretinitis. While optic disc edema is present in papillitis and neuroretinitis, neuroretinitis in addition also has a “macular star” appearance which is hard exudates at the macula. Retrobulbar neuritis has a normal disc appearance. Multiple sclerosis (MS) is commonly associated with papillitis and retrobulbar neuritis while infectious and inflammatory disorders are more often related to neuroretinitis.[2] The disease commonly presents between 20 and 50 years of age. It is more common in females while its annual incidence has been reported to be between 1 and 5 per 100,000 in population-based studies in the United States.[3] We report a case of neuroretinitis with vitiligo in a 52-year-old female.

  Case Report Top

A 52-year-old female [Figure 1] presented to our institute with a history of sudden loss of vision in the right eye for 3 days. There was a mild pain on ocular movements. She was a known case of vitiligo but had not taken any medications for it since the past 5 years. There was no history of fever, diabetes mellitus, hypertension, any other systemic illness, headache, weakness of any part of the body, auditory or speech problems, recent drug intake or vaccination, animal bites or having any pets at home. There was no significant family history. She was farmer by occupation and lived in a nearby village.
Figure 1: Patients photo

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On examination, her speech, intelligence, language, hearing, and general physical examination were within normal limits. Her best-corrected visual acuity was 6/60 in the right eye and 6/6 in the left eye. The relative afferent pupillary defect was present in the right eye. Ocular movements, intraocular pressure, and gonioscopy were normal. Slit lamp examination revealed 1 + cells in the vitreous. Color vision tests using Ishihara charts revealed red-green color deficiency. Fundus examination revealed disc edema on the affected side with characteristic “Macular Star” finding which are hard exudates arranged in a radiating pattern at the macula [Figure 2]. Fundus examination of the left eye was normal [Figure 3]. Venous sheathing was also present. Visual field evaluation revealed generalized constriction of the fields. Ocular examination of the left eye was within normal limits. Based on the above findings, a diagnosis of ON (neuroretinitis) was made. As the etiology of this disease is wide, a battery of investigations was ordered. We carried out magnetic resonance imaging (MRI) of the brain and optic nerve (gadolinium enhancement and fat suppression), MRI spine, complete hemogram with erythrocyte sedimentation rate, blood sugar, C-reactive proteins, antinuclear antibodies, venereal disease research laboratories, serum angiotensin converting enzyme and calcium levels, lymes titer, X-ray chest, quantiferon tuberculosis gold test, blood culture, toxoplasma, rubella, cytomegalovirus, and herpes virus titers, stool examination, and viral markers (hepatitis A, B and human immunodeficiency virus) tests. All the tests were within normal limits. Visual evoked potential and serology for Bartonella were not be performed as they were not available at our institute and cerebrospinal fluid analysis for cells, protein, oligoclonal bands, culture, and aquaporins could not be carried out as the patient did not consent for the test and was apprehensive. We started the patient on intravenous methylprednisolone 1 g intravenous OD for 3 days followed by oral prednisolone 1 mg/kg for 11 days. She was advised regular follow-up. By the end of 2nd week, her best-corrected visual acuity in the affected eye had improved to 6/12, and we are awaiting the next follow-up from her.
Figure 2: Fundus photo (right) eye

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Figure 3: Fundus photo (left) eye

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  Discussion Top

Neuroretinitis presents as a sudden unilateral visual loss with optic disc edema and “macular star” configuration. It is thought to be an infectious/immune-mediated process and various etiological agents implicated are cat scratch disease, hepatitis B, syphilis, Lyme disease, toxoplasmosis, toxocariasis, etc., The mechanism of its pathogenesis is the infectious/inflammatory process which affects the optic nerve along with edema and exudation of fluid.[4]

In addition to the visual loss, periocular pain with ocular movements is often seen and is thought to occur as a result of stretching of the inflamed optic nerve with eyeball movement. Other positive findings include decreased color vision, visual field loss, and a relative afferent pupillary defect present in the affected eye. Differential diagnosis includes anterior ischemic optic neuropathy (AION)[5] and central retinal vein occlusion (CRVO).[4] Although AION also presents as sudden unilateral loss of vision in patients more than 50 years of age along with optic disc swelling, periocular pain on eye movements is not seen as well as there is no female preponderance. Segment retinal hemorrhages are also seen in AION.[5] CRVO presents with multiple dot and flame-shaped hemorrhages in all four retinal quadrants, tortuous retinal veins with soft exudates present more often.[6] Our patient was a 52-year-old female with periocular pain and characteristic “macular star” configuration without segmental retinal hemorrhages.

Although the diagnosis is mainly clinical, the tests carried out are MRI brain (gadolinium-enhanced) and spine to determine the risk for developing MS. Tests are done to determine the etiological agents implicated, and the various tests carried out are serology for syphilis, Bartonella and toxoplasmosis; Mantoux test, blood culture; cerebrospinal fluid examination, etc.[7] All the investigations in our patient were within normal limits and hence it was the idiopathic form of the disease. Treatment modalities include intravenous dexamethasone 200 mg OD for 3 days or intravenous methylprednisolone sodium succinate 250 mg every 6 h for 3 days followed by oral prednisone, 1 mg/kg/day for 11 days; intravenous immunoglobulin, plasma exchange, disease modifying drugs such as interferon #-1a, interferon #-1b, and glatiramer acetate.[8]

Vitiligo is a skin disorder, and ocular disorders such as Vogt Koyanagi Harada syndrome, sympathetic ophthalmia, Alezzandrini's syndrome, Waardenburg syndrome, uveitis, iris pigmentary abnormalities, and subclinical inflammatory fundus depigmentation association with it is known.[9] Association of vitiligo with neuroretinitis is not known, but autoimmune etiology is common to both. In our case, the association of neuroretinitis and vitiligo may be coincidental or may have some immune phenomenon linked to both. Further studies and research are hence warranted. Improvement in vision starts within 2–3 weeks in 80% of the patients and continues to improve for up to 1 year.[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


We would like to thank our patient.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Lim SA, Goh KY, Tow S, Fu E, Wong TY, Seah A, et al. Optic neuritis in Singapore. Singapore Med J 2008;49:667-71.  Back to cited text no. 1
Pau D, Al Zubidi N, Yalamanchili S, Plant GT, Lee AG. Optic neuritis. Eye (Lond) 2011;25:833-42.  Back to cited text no. 2
Gal RL, Vedula SS, Beck R. Corticosteroids for treating optic neuritis. Cochrane Database Syst Rev 2015:CD001430.  Back to cited text no. 3
Narayan SK, Kaliaperumal S, Srinivasan R. Neuroretinitis, a great mimicker. Ann Indian Acad Neurol 2008;11:109-13.  Back to cited text no. 4
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Dooley MC, Foroozan R. Optic neuritis. J Ophthalmic Vis Res 2010;5:182-7.  Back to cited text no. 5
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The Central Vein Occlusion Study Group. Natural history and clinical management of central retinal vein occlusion. Arch Ophthalmol 1997;115:486-91.  Back to cited text no. 6
Menon V, Saxena R, Misra R, Phuljhele S. Management of optic neuritis. Indian J Ophthalmol 2011;59:117-22.  Back to cited text no. 7
[PUBMED]  [Full text]  
Hoorbakht H, Bagherkashi F. Optic neuritis, its differential diagnosis and management. Open Ophthalmol J 2012;6:65-72.  Back to cited text no. 8
Gopal KV, Rama Rao GR, Kumar YH, Appa Rao MV, Vasudev P; Srikant. Vitiligo: A part of a systemic autoimmune process. Indian J Dermatol Venereol Leprol 2007;73:162-5.  Back to cited text no. 9
[PUBMED]  [Full text]  
Shams PN, Plant GT. Optic neuritis: A review. Int MS J 2009;16:82-9.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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