https://doi.org/10.1177/1120672118803534
European Journal of Ophthalmology
1–4
© The Author(s) 2018
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DOI: 10.1177/1120672118803534
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Introduction
Behçet’s disease (BD) is a chronic systemic inflamma-
tory vasculitis of unknown etiology. Non necrotizing
inflammatory damage involves many organ systems and
patient may present with oral ulcers, genital ulcers, joint
disease, uveitis, recurrent venous thrombosis, intracra-
nial sinus thrombosis, gastrointestinal complications, and
erythema nodosum.
1
Mucocutaneous ulcers have been
found to be most common presenting illness followed by
genital ulcers and ocular inflammation.
1
Eye involve-
ment in BD is common and usually presents with panu-
veitis with mainly posterior segment inflammation.
2
Severe kerato-conjunctivitis without other features of eye
involvement has been reported infrequently.
3
Our patient
presented with severe bilateral keratitis followed by
development of seizures, which was subsequently diag-
nosed as neuro-BD.
Case summary
A 19-year-old Nepalese male presented to the emergency
services of our tertiary-care center with complaints of ocu-
lar discomfort, photophobia, watering, and redness in both
the eyes (OU) (left > right eye) for the past 1 week. The
patient had been diagnosed as viral conjunctivitis else-
where and was started on topical moxifloxacin 0.5% and
fluorometholone 0.1%. Since there was no improvement
in the symptoms, the patient was referred for a second
opinion. On examination, his visual acuity was 6/18 and
6/60 in right eye (OD) and left eye (OS), respectively.
Slit-lamp examination revealed bilateral diffuse conjuncti-
val congestion with palpebral conjunctival follicular reac-
tion. There was presence of stromal infiltrates with
overlying punctate epitheliopathy and was located densely
at the termination of limbal vessels at peripheral cornea
OU. The stromal infiltrates were more dense, diffuse and
were progressing centripetally OS (Figure 1). Corneal sen-
sations were normal OU. These corneal findings were
Immune keratitis: An unusual primary
presentation of neuro-Behçet’s disease
Jitender Jinagal
1
, Aniruddha Agarwal
1
, Avinash Negi
1
,
Gaurav Gupta
1
, Vikas Sharma
2
, Parul Chawla Gupta
1
and Jagat Ram
1
Abstract
A 19-year-old Nepalese male presented with complaints of bilateral ocular discomfort, photophobia, watering, and
redness for 1 week. Visual acuity was 6/12 and 6/60 in the right and left eye, respectively. On biomicroscopic examination,
presence of peripheral stromal infiltrates with conjunctival follicles was noted; infiltrates progressed to involve central
cornea with further decrease in vision over next few days. After ruling out infectious keratitis, detailed systemic
examination and laboratory investigations were diagnostic of neuro-Behçet’s disease. Patient responded to systemic
steroidal and immunosuppressive therapy characterized by corneal healing and visual acuity improvement to 6/6 and 6/9.
Although rare, but neuro-Behçet’s disease can primarily present as bilateral immune keratitis and every case of bilateral
keratitis needs early systemic evaluation after ruling out infective etiologies.
Keywords
Behçet disease, bilateral keratitis, immune keratitis, peripheral keratitis, neuro-Behçet
Date received: 8 July 2018; accepted: 4 September 2018
1
Advanced Eye Centre, Department of Ophthalmology, Postgraduate
Institute of Medical Education & Research (PGIMER), Chandigarh, India
2
Department of Rheumatology, Postgraduate Institute of Medical
Education & Research (PGIMER), Chandigarh, India
Corresponding author:
Jagat Ram, Advanced Eye Centre, Department of Ophthalmology,
Postgraduate Institute of Medical Education & Research (PGIMER),
Sector 12, Chandigarh 160012, India.
Email: drjagatram@gmail.com
803534EJO 0 0 10.1177/1120672118803534European Journal of OphthalmologyJinagal et al.
case-report 2018
Case Report