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   Table of Contents      
BRIEF REPORT
Year : 2001  |  Volume : 49  |  Issue : 1  |  Page : 53-55

Vogt-koyanagi-harada syndrome following Injury-induced progressive vitiligo


1 MS. Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 MD. Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 MS. Department Internal Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Vishali Gupta
MS. Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

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Source of Support: None, Conflict of Interest: None


PMID: 15887718

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How to cite this article:
Gupta V, Gupta A, Bambery P, Radotra B D, Panday S S. Vogt-koyanagi-harada syndrome following Injury-induced progressive vitiligo. Indian J Ophthalmol 2001;49:53-5

How to cite this URL:
Gupta V, Gupta A, Bambery P, Radotra B D, Panday S S. Vogt-koyanagi-harada syndrome following Injury-induced progressive vitiligo. Indian J Ophthalmol [serial online] 2001 [cited 2020 Apr 4];49:53-5. Available from: http://www.ijo.in/text.asp?2001/49/1/53/22667

We report a case of Vogt-Koyanagi-Harada (VKH), Syndrome wherein the patient developed ocular symptoms following injury-induced progressive vitiligo with immunologic evidence from the skin biopsy specimen of the vitiligo. This case supports the hypothesis that the cell-mediated immune process against a common melanocyte antigen plays an important role in the development and progression of the disease.

Vogt-Koyanagi-Harada-Syndrome comprises uveitis with associated extraocular manifestations, namely vitiligo or central nervous system disorders. A cell-mediated immune response directed against an antigen that is common to the uveal, dermal and meningeal melanocytes is believed to play an important role.[1] Recently, the development of VKH Syndrome following cutaneous injury has been reported where the affected areas became vitiliginous on healing and simultaneous with onset of ocular symptoms.[2] We report a patient who developed bilateral VKH syndrome following injury-induced progressive vitiligo to further support the association with additional immunopathologic evidence from the skin biopsy specimen of the vitiligo.


  Case report Top


A 48-year-old woman presented with a history of gradual loss of vision in both eyes associated with recurrent attacks of pain, redness and headache of 3 years' duration. A diagnosis of intraocular inflammation with complicated cataract had been made elsewhere. She had undergone cataract extraction with a posterior chamber intraocular lens (IOL) implant in the left eye eight months prior to presentation to us. Thus the IOL implantation had taken place 2 years and 4 months after the episode of bilateral ocular inflammation. She did not receive systemic corticosteroids at the time of surgery. However, at the time of our examination she was receiving oral prednisolone 30mg/day and azathioprine 50mg twice daily for postoperative worsening of her symptoms without any relief. She had a history of roadside accident 15 years earlier, involving injury to both hands that had left behind depigmented scars. These depigmented patches did not show any change till 3 years ago, when she noted an increase in the size of the patch on the right hand [Figure - 1] along with appearance of new depigmented patches elsewhere on the body including on the left hand. This coincided with the onset of her ocular symptoms.

Systemic examination revealed extensive patches of vitiligo on her hands, feet and back. The best corrected visual acuity was 6/12 in the right eye and counting fingers close to face in the left eye. Intraocular pressure was 12 mm Hg in the right eye and 4 mm Hg in the left eye. External examination showed vitiligo over both upper eyelids and forehead with poliosis in both eyes. Slitlamp examination of the right eye showed broad-based posterior synechiae with a complicated posterior subcapsular cataract, but no signs of active inflammation. The left eye showed moderate anterior chamber flare and cells, with iridocapsular adhesions and a posterior chamber IOL. Sugiura's sign (perilimbal vitiligo) was absent in both eyes. Fundus examination showed widespread atrophy of the retinal pigment epithelium with sunset glow in both eyes. In addition her left eye also showed optic disc oedema, tortuous veins, and choroidal folds [Figure:2a] and [Figure:2b]. B-Scan ultrasonogram was normal in the right eye and showed retinochoroidal thickening of the posterior pole in the left eye. There were no auditory symptoms and audiometry was normal. Laboratory investigations, including a skin testing with PPD, X-ray chest and syphilis serologies were negative. Skin biopsy from the vitiligo patch on the right hand showed loss of pigment and presence of melanocytes in the basal cell layer of epidermis associated with focal areas of perivascular collection of lymphocytes.The infiltrated lymphocytes consisted of T lymphocytes that were predominantly positive for CD8+ markers on immunohistochemistry [Figure - 3] with few CD4+ cells.The patient was diagnosed as having VKH Syndrome and treated with topical corticosteroids and cycloplegics alongwith oral corticosteroids lmg/kg/day tapered off over a period of 9 months and oral azathioprine 50 mg. twice daily for 12 months. There has been no recurrence over a follow up of 12 months (when last seen) and the final visual acuity was 6/9 in right eye and counting fingers half metre in the left eye. The visual acuity in the left eye was less due to hypotonic maculopathy.


  Discussion Top


Our patient developed bilateral panuveitis following injury induced progressive vitiligo. The possibilities of intraocular tuberculosis and syphilis were ruled out by investigations. The association of vitiligo with VKH syndrome is well described. [3,4] However, VKH after skin trauma appears uncommon. Rathinam et al[2] have recently reported three patients who developed VKH syndrome shortly following cutaneous injury. The affected areas in each case became vitiliginous on healing, simultaneous with onset of ocular symptoms.

On immunohistochemistry the vitiligo patches showed the predominant presence of CD8+ cells indicating a chronic phase of T-cells-mediated delayed hypersensitivity response. Sakamoto et al[5] have demonstrated that the choroidal infiltrates are predominantly composed of T lymphocytes; most of which were CD4+ cells, with CD8+ being relatively low in density. In contrast, results from the skin biopsy, showed the predominant presence of CD8+ cells.

It is now firmly believed that VKH syndrome results from a T-cells-mediated delayed hypersensitivity response to an antigen shared by melanocytes in the skin, the meninges, the inner ear and the choroid.[1] However, the trigger for this response not yet known. Recently, Bessou et al[6] have emphasized the need for an extrinsic factor to trigger the epidermal vitiligo. Our patient had a clinical picture that closely resembled Vogt-Koyanagi-Harada Syndrome. The American Uveitis Society in 1978 adopted the following criteria for the diagnosis of VKH syndrome:[7] (1) the patient should have no history of ocular trauma or surgery; (2) at least three of the following four signs should be present: (a) bilateral chronic iridocyclitis; (b) posterior uveitis, including exudative retinal detachment, forme fruste of exudative retinal detachment and "Sunset glow" fundus; (c) neurologic and (d) cutaneous signs.

Our patient did not have any history of ocular trauma or surgery prior to the onset of inflammation. The cataract extraction in the left eye was done 2 years and 4 months after the onset of ocular symptoms. She had bilateral chronic iridocyclitis, sunset glow fundus and cutaneous signs, thus fulfilling the criteria of VKH syndrome. Histologic evidence of delayed T-cell mediated hypersensitivity reaction in the progressive vitiligo patch coincided with the occurrence of VKH syndrome in our patient. This supports the hypothesis that a cell-mediated immunological reaction against a common melanocyte antigen is involved in the pathogenesis of VKH Syndrome.


  Acknowledgement Top


We are thankful to Prof. N.A.Rao Doheny Eye Institute, University of Southern California for helping us carry out the immunohistochemical staining of skin biopsy specimen.



 
  References Top

1.
Moorthy RS, Inomata H, Rao NA. Vogt-Koyanagi-Harada Syndrome.Surv Ophthalmol1995;39:265-92.  Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.
Rathinam SR, Nampenumalsamy P, Nozik RA, Cunningham ET. Vogt-Koyanagi-Harada Syndrome after cutaneous injury. Ophthalmology 1999;106:635-38.  Back to cited text no. 2
    
3.
Wagoner MD, Albert DM, LernerAB, Kirtwood J, Forget BM, Norlund JJ. New observations on vitiligo and ocular disease.Am J Ophthalmol1983;96:16-26.  Back to cited text no. 3
    
4.
Albert DM, Wagoner MD, Pruett RC, Norlund JJ, Lerner AB. Vitiligo and disorders of the retinal pigment epithelium.Br J Ophthalmol1983;67:153-56.  Back to cited text no. 4
    
5.
Sakamoto T, Murata T, Inomata H. Class II Major Histocompatibility complex on melanocytes of Vogt-Koyanagi-Harada disease.Arch Ophthalmol1991;109:1270-74.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.
Bessou S, Gauther Y, Surleu Bazeille JE, Taileb A. Epidermal reconstructs in vitiligo: An extrinsic factor is needed to trigger the disease.BrJDermatol1997;137:890-97.  Back to cited text no. 6
    
7.
Snyder DA, Tessler HH. Vogt-Koyanagi-Harada Syndrome.Am J Ophthalmol1980;90:69-75.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]


This article has been cited by
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Andrade, A., Pithon, M.
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Chen, L.-F., Xu, G.-Y., Tong, Q.-L.
Chinese Journal of Contemporary Neurology and Neurosurgery. 2008; 8(1): 56-58
[Pubmed]



 

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