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 Table of Contents    
CASE REPORT
Year : 2015  |  Volume : 8  |  Issue : 1  |  Page : 54-55  

Nodular scleritis as the eye manifestation in Behcet's syndrome


1 Department of Uvea and Intraocular Inflammations, Sankara Nethralaya, Chennai, India
2 Director of Uveitis and Ocular Pathology Departments,Sankara Nethralaya, Chennai, India

Date of Web Publication23-Jan-2015

Correspondence Address:
Karpagam Damodaran
Senior resident ,Department of Uvea and Intraocular Inflammations Sankara Nethralaya No. 41, College Road, Nungambakkam, Chennai
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-620X.149869

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   Abstract 

We report a case of a 45-year-old female, who was diagnosed as Behcet's disease and presented to us with nodular scleritis in her right eye.

Keywords: Behcets, human leukocyte antigen B51, nodular scleritis


How to cite this article:
Damodaran K, Majumder PD, Biswas J. Nodular scleritis as the eye manifestation in Behcet's syndrome. Oman J Ophthalmol 2015;8:54-5

How to cite this URL:
Damodaran K, Majumder PD, Biswas J. Nodular scleritis as the eye manifestation in Behcet's syndrome. Oman J Ophthalmol [serial online] 2015 [cited 2019 Nov 21];8:54-5. Available from: http://www.ojoonline.org/text.asp?2015/8/1/54/149869


   Introduction Top


Scleritis is known to be associated with systemic connective tissue diseases or vasculitic diseases such as rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and systemic lupus,other less frequently associated diseases include polyarteritis nodosa, reactive arthritis, psoriatic arthritis, giant cell arteritis and Cogan syndrome.The scleritis occurred in patients with Behcets disease has been rarely reported in the literature. We describe a female patient who developed the scleritis in the course of Behcets disease.


   Case Report Top


A 45-year-old female presented to us with chief complaints of pain and redness in her right eye for the past 1 month. She was diagnosed as having Behcet's syndrome by a rheumatologist elsewhere 4 years ago when she had been suffering from recurrent oral and genital ulcers. During the course of her disease she also developed acne like pustular skin eruptions, erythema nodosum like lesions and arthritis predominately involving her knees and a positive pathergy test. She also gave a history of intermittent painful redness alternating in both her eyes, initially in her left eye and later in the right eye. She was being treated with colchicine (1.5 mg/day), deflazacort and dapsone systemically for the past 4 years. However, when she presented to us she was totally off all medications.

Ophthalmic examination revealed a best-corrected visual acuity of 20/20 in both her eyes. Intraocular pressures by Goldman applanation tonometry were 12 and 14 mm of Hg in her right and left eye respectively. Slit lamp examination of the right eye revealed a tender scleral nodule temporally along with marked dilatation of the deeper episcleral vessels [Figure 1]. The rest of the anterior segment evaluation was normal in both the eyes. Fundus examinations of both her eyes were normal. Fundus fluorescein angiography did not reveal any vascular leakage or abnormality [Figure 2].
Figure 1: Slit lamp examination of the right eye showing scleral nodule with deep episcleral congestion

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Figure 2: Fundus fluorescein angiography showing no abnormality

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Physical examination was however not contributory at the time of her presentation. Laboratory investigations showed raised erythrocyte sedimentation rate, anaemia and leukocytosis. She was found to be negative for human leukocyte antigen-B51 and 5. All other investigations, including rheumatoid factor, antinuclear antibody, anti-neutrophil cytoplasmic antibodies (c and P anti-neutrophil cytoplasmic antibodies) were negative. She was diagnosed nodular scleritis in her right eye and after consultation with a rheumatologist, she was started on oral prednisolone 30 mg/day, to be tapered gradually along with oral azathioprine 50 mg twice a day.


   Discussion Top


Behcet's disease is a chronic, relapsing, multisystem, idiopathic inflammatory disorder characterized by a triad of oral ulcers, genital ulcers, and ocular lesions. However, the spectrum of disease is even wider with the involvement of articular, intestinal, vascular, urogenital, and neurologic systems, besides the mucocutaneous and ocular findings. [1],[2] The disease has a higher prevalence particularly in Turkey, Iraq, Iran, Korea, and Japan and accounts for up to 20% of cases of endogenous uveitis in some of these countries. [3],[4] The diagnosis of Behcet's disease is based on the identification of its typical clinical features, according to the diagnostic criteria laid down by the International Study Group and the Japanese criteria for the diagnosis of Behcet's disease. [5],[6]

Previously published reports on Behcet's disease among Indian patients highlight the fact that Behcet's disease, as well as ocular involvement, is significantly less common in Indian population. [7],[8],[9],[10] In a study of 53 patients at a tertiary care centre in India by Sachdev et al., [11] eight patients (16.3%) had isolated anterior uveitis, while 13 patients (26.5%) had predominantly posterior uveitis and the remaining 28 patients (57.1%) had panuveitis. Those with anterior segment involvement predominantly consisted of hypopyon uveitis and two cases of conjunctival ulceration. This was in contrast to a study by Rohatgi and Singal [7] who reported that the most common ocular manifestation in their patients was conjunctival ulceration (26.3%), while uveitis was a rare finding (10.5%).

Scleritis is associated with systemic connective tissue diseases and/or vasculitic problems, such as rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, relapsing polychondritis and systemic lupus erythematosus in approximately 50% of cases. [12] To our knowledge only four cases of scleritis associated with Behçet's disease have been reported in the literature in the past.

Chang and Cho reported a 46-year-old woman presenting with nodular scleritis [13] and Sainz de la Maza et al. reported one out of 172 patients. [14] Sakellariou et al. reported a case of scleromalacia perforans in a patient with Behcets disease. [15] Akova et al. have also reported an atypical case of myositis with scleritis in association with Behcet's disease. [16]

Cases of diffuse or nodular scleritis that show resistance to conventional therapy or recur very frequently should be investigated carefully. The association of scleritis in Behçet's disease may suggest the aggressive use of immunosuppressive agents earlier in the disease process, two or three immunsupressive agents may be considered. Aggressive systemic therapy is necessary to save not only the eye but also the patient's life.

 
   References Top

1.
Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999;341:1284-91.  Back to cited text no. 1
    
2.
Michelson JB, Chisari FV. Behçet's disease. Surv Ophthalmol 1982;26:190-203.  Back to cited text no. 2
    
3.
Azizlerli G, Köse AA, Sarica R, Gül A, Tutkun IT, Kulaç M, et al. Prevalence of Behçet's disease in Istanbul, Turkey. Int J Dermatol 2003;42:803-6.  Back to cited text no. 3
    
4.
Mishima S, Masuda K, Izawa Y, Mochizuki M, Namba K. The eighth Frederick H. Verhoeff Lecture. presented by saiichi mishima, MD Behçet's disease in Japan: Ophthalmologic aspects. Trans Am Ophthalmol Soc 1979;77:225-79.  Back to cited text no. 4
    
5.
Behcet's Disease Research Committee of Japan. Behcet's disease guide to the diagnosis of Behcet's disease (1972) Japanese Journal of Ophthalmology 1974;18:291-4.  Back to cited text no. 5
    
6.
Criteria for diagnosis of Behçet's disease. International study group for Behçet's disease. Lancet 1990;335:1078-80.  Back to cited text no. 6
    
7.
Rohatgi J, Singal A. Ocular manifestations of Behcet's disease in Indian patients. Indian J Ophthalmol 2003;51:309-13.  Back to cited text no. 7
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8.
Kunjuraman G, Phillip J, Joseph PP. Behcet's syndrome (a rare and poorly understood entity) (a report of three cases with review of literature). J Assoc Physicians India 1980;28:95-8.  Back to cited text no. 8
    
9.
Singh RR, Malaviya AN. Behcet's syndrome in north India. J Assoc Physicians India 1988;36:238.  Back to cited text no. 9
    
10.
Pande I, Uppal SS, Kailash S, Kumar A, Malaviya AN. Behçet's disease in India: A clinical, immunological, immunogenetic and outcome study. Br J Rheumatol 1995;34:825-30.  Back to cited text no. 10
    
11.
Sachdev N, Kapali N, Singh R, Gupta V, Gupta A. Spectrum of Behçet's disease in the Indian population. Int Ophthalmol 2009;29:495-501.  Back to cited text no. 11
    
12.
Akova YA, Jabbur NS, Foster CS. Ocular presentation of polyarteritis nodosa. Clinical course and management with steroid and cytotoxic therapy. Ophthalmology 1993;100:1775-81.  Back to cited text no. 12
    
13.
Chang HK, Cho EH. A case of nodular scleritis in association with Behcet's disease. Korean J Intern Med 2001;16:47-9.  Back to cited text no. 13
    
14.
Sainz de la Maza M, Jabbur NS, Foster CS. Severity of scleritis and episcleritis. Ophthalmology 1994;101:389-96.  Back to cited text no. 14
    
15.
Sakellariou G, Berberidis C, Vounotrypidis P. A case of Behcet's disease with scleromalacia perforans. Rheumatology (Oxford) 2005;44:258-60.  Back to cited text no. 15
    
16.
Dursun D, Akova Y, Yücel E. Myositis and scleritis associated with Behcet's disease: An atypical presentation. Ocul Immunol Inflamm 2004;12:329-32.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2]



 

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