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BRIEF COMMUNICATION
Year : 2019  |  Volume : 7  |  Issue : 3  |  Page : 122-123

Bilateral conjunctival melanocytosis and retinitis pigmentosa


Department of Ophthalmology, Vasantrao Naik Government Medical College, Nagpur, Maharashtra, India

Date of Submission16-Jan-2018
Date of Acceptance07-Jun-2019
Date of Web Publication11-Dec-2019

Correspondence Address:
Rajesh Subhash Joshi
77, Panchatara Housing Society, Manish Nagar, Somalwada, Nagpur - 440 015, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcor.jcor_11_18

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  Abstract 


Conjunctival pigmented lesions have varied appearances. It arises from the melanocytic or nonmelanocytes. It is rare to find conjunctival melanocytosis associated with retinal pigmentary dystrophy. We report a case of a 69-year-old male with bilateral typical retinitis pigmentosa with conjunctival melanocytosis. The excessive pigmentation of conjunctiva was recognized when the patient came for cataract surgery. The conjunctival pigment was identified during slit lamp examination. Histopathological section of superior conjunctiva revealed stromal mononuclear infiltrate with melanin pigment in the basal cells. To the best of our knowledge, such an association has not previously been described. Such an association needs to be studied in a large case series. The present of conjunctival pigments may alert the eye surgeon to look for pigmentary disorder of the retina.

Keywords: Conjunctival melanocytosis, conjunctival pigmentation, retinitis pigmentosa


How to cite this article:
Joshi RS, Hussain Bombaywala IZ. Bilateral conjunctival melanocytosis and retinitis pigmentosa. J Clin Ophthalmol Res 2019;7:122-3

How to cite this URL:
Joshi RS, Hussain Bombaywala IZ. Bilateral conjunctival melanocytosis and retinitis pigmentosa. J Clin Ophthalmol Res [serial online] 2019 [cited 2023 Mar 23];7:122-3. Available from: https://www.jcor.in/text.asp?2019/7/3/122/272705



Retinitis pigmentosa (RP) is a group of inherited retinal disorders characterized by night blindness and progressive visual field loss. The essential features consist of arteriolar narrowing, waxy disc pallor, and bony spicule pigmentary changes. Most of the features are limited to the retina and optic nerve. However, there are reports of oculodermal pigments in patients with RP.[1] Conjunctival pigments in RP have not been reported yet. Pigmented conjunctival lesions have varied differential diagnosis. They arise either from melanocyte or nonmelanocyte cells. Pigmentary conjunctival lesions include racial melanosis, nevus, primary acquired melanosis, and melanoma.[2],[3] Silver and iron deposits do give rise to the similar appearance.[4],[5]

The purpose of reporting this case is to describe an uncommon conjunctival pigment which was bilateral with entirely different appearance and an association with RP.


  Case Report Top


A 69-year-old male presented with complaints of diminished vision in both eyes since 1 year. The patient was a known case of RP [Figure 1]. On examination, his visual acuity was 20/200 in both eyes. The conjunctival pigment was identified during slit lamp examination. Discrete black-pigmented lesions were seen all over inferior and superior conjunctiva of variable sizes extending till fornices [Figure 2]a, [Figure 2]b, [Figure 2]c. However, tarsal conjunctiva was not affected. Similar lesions were seen in the right eye also. Slit lamp examination also showed semi dilated sluggishly reacting pupil with Grade-4 nuclear cataract according to Lens Opacities Classification System III. Fundus evaluation on indirect ophthalmoscopy was done. It showed hazy media due to cataract. Optic disc and retinal vessels were faintly seen, and details were not commentable. Discrete bony spicule pigments were seen scattered all over the retina. The diagnosis of conjunctival pigments with RP and cataract was kept in both eyes. The patient underwent left eye cataract surgery with implantation of intraocular lens. During the surgical procedure, 4 × 4 superior conjunctiva including Tenon's capsule was sent for the histopathology. Biopsy of conjunctiva was done to know the nature of pigments. The histopathological examination showed unremarkable stratified squamous epithelium with basal cells containing brown-black, nonrefractile, and finely granular pigments suggestive of melanin [Figure 3]. To confirm the nature of pigment a piece of conjunctiva was kept in a diluted solution of potassium permanganate, which lead to melanin to bleach.
Figure 1: Retinal pigment in retinitis pigmentosa

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Figure 2: (a-c) Distribution of conjunctival pigment superior, inferior and lateral side of conjunctiva

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Figure 3: Histopathological section of conjunctiva showing basal cells laden with melanin pigment

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  Discussion Top


The exact etiology of conjunctival pigment in RP could not be ascertained. Histopathology of conjunctival pigment was planned to know the nature of pigment and to find out any association and resemblance with the retinal pigments. The absence of inflammatory signs in conjunctiva clinically and histopathologically ruled out inflammatory nature of the pigment. Conjunctival pigmentation has been reported in  Stevens-Johnson syndrome More Details triggered by sulfasalazine,[6] minocycline therapy,[7] and prostaglandin use for glaucoma. Our patient did not have a history of any drug usage.

Conjunctival freckles are flat patches of pigmentation limited to the area of conjunctiva near the limbus. Our patient had conjunctival pigment distributed all over the conjunctiva.

Conjunctival pigmentation is also associated with naevus of Ota characterized by pigmentation of episclera, uveal tract, angle of anterior chamber, and facial pigmentation. Pigmentation in the present case is located over the conjunctiva without the involvement of face and uveal tract. Gold et al. reported oculodermal melanocytosis associated with RP. Presentng features were oculo cutaneous pigmentation with bilateral retinal pigmentation.[1] Irregular Scleral pigmentation and atrophic optic nerve were also noted.

It may be a chance that the present case has two different pigmentary disorders. Embryologically, conjunctival and retinal pigment epithelium has different origin. However, retinal pigment epithelial and conjunctival melanocytes have origin from neural crest cells. Neural crest cells develop from neuroectoderm from which retinal pigment epithelium also develops. The association of these two disorders may be a coincidence.

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.

Acknowledgment

I would like to acknowledge Dr. Nitin Sapkal, Shri Aniruddha Pathology, Nagpur, Maharashtra, India, for histopathology report.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Gold DH, Henkind P, Sturner WQ, Baden M. Oculodermal melanocytosis and retinitis pigmentosa. Am J Ophthalmol 1967;63:271-9.  Back to cited text no. 1
    
2.
Shields CL, Demirci H, Karatza E, Shields JA. Clinical survey of 1643 melanocytic and nonmelanocytic conjunctival tumors. Ophthalmology 2004;111:1747-54.  Back to cited text no. 2
    
3.
Grossniklaus HE, Green WR, Luckenbach M, Chan CC. Conjunctival lesions in adults. A clinical and histopathologic review. Cornea 1987;6:78-116.  Back to cited text no. 3
    
4.
Sarnat-Kucharczyk M, Pojda-Wilczek D, Mrukwa-Kominek E. Diagnostic methods in ocular argyrosis: Case report. Doc Ophthalmol 2016;133:129-38.  Back to cited text no. 4
    
5.
Loh A, Hadziahmetovic M, Dunaief JL. Iron homeostasis and eye disease. Biochim Biophys Acta 2009;1790:637-49.  Back to cited text no. 5
    
6.
Fuentes-Páez G, Mendez MC, Montañez J, Herreras JM, Saornil MA, Calonge M. Conjunctival pigmentation in Stevens-Johnson syndrome. Compr Ther 2007;33:99-103.  Back to cited text no. 6
    
7.
Khan TT, Reddy UP. Conjunctival pigmentation following minocycline therapy. Ophthalmic Plast Reconstr Surg 2016;32:e129-30.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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