In the four patients described in this report, conjunctival ulceration developed in concordance with the exacerbation of systemic symptoms of Behçet's syndrome such as oral and genital ulcerations and erythema nodosum. This indicates that conjunctival ulceration may develop as a clinical manifestation of Behçet's syndrome. In addition to conjunctival ulceration, corneal infiltration with ulceration was noted as a kissing ulcer corresponding to the lesion in the upper palpebral conjunctiva in case 3. Conjunctival ulcerations in all patients showed a positive response to topical corticosteroids or topical corticosteroids plus oral colchicine.
It should be noted that conjunctival ulceration in all four patients developed in the absence of intraocular inflammation. This suggests that conjunctival ulcers may present as an isolated finding in Behçet's syndrome without other retinal or intraocular inflammation. The present diagnostic criteria for Behçet's syndrome,4
as used in this study, require the presence of at least three of four signs such as oral and genital ulcerations or skin and eye lesions, with an emphasis on oral ulcerations. The eye lesions specified in the criteria do not include conjunctival ulceration; however, to better diagnose Behçet's syndrome, the dissociation of conjunctival ulceration with intraocular inflammation in the present four patients suggests that conjunctival ulceration may be recognised as one sign of eye lesions.
It should not be surprising that conjunctival ulcers develop in Behçet's syndrome as the conjunctiva is of course a mucous membrane, as are the oral and genital mucosa. Therefore, conjunctival ulceration would be expected to parallel the findings in the other mucosa. Such mucosal involvement would probably be distinct from intraocular inflammation in Behçet's syndrome. Conjunctival ulceration in the present series of patients was, indeed, observed in parallel with exacerbation of oral ulceration, but not in association with intraocular inflammation. All four patients were given topical corticosteroids for conjunctival ulceration, combined with oral colchicine in three of them, mainly to control the systemic manifestations such as oral ulceration. Topical corticosteroids did alleviate ocular symptoms in all the patients, but it remains unknown whether conjunctival ulceration might be rather self limiting. Oral colchicine, at least in case 1, did reduce the number of episodes of conjunctival ulceration in both eyes.
Until now, there have been three case reports describing patients with Behçet's syndrome who developed conjunctival ulceration: a 49 year old white man in the United States,1
a 45 year old woman in Italy,2
and a 45 year old woman in Tunisia.3
The clinical features of conjunctival ulceration in the present series of four patients are similar to those three patients reported previously. The incidence of conjunctival ulceration in this series is 2.6% (four of 152 patients). In comparison, the incidence of conjunctival ulceration in Turkey as reported and cited in Olivieri et al2
is 0.9% (five of 540 patients), similar to the present series.
In case 3 of the present series, corneal infiltration causing ulceration could have derived from the palpebral conjunctiva since the conjunctival and corneal lesions formed a configuration of a kissing ulcer. In a series of patients with Behçet's syndrome in England, corneal ulceration was also reported as an eye lesion, the incidence of which was as high as 16% (four of 25 patients).5
The incidence of corneal ulceration reported in the literature5
seems rather high in our experience, but the reason remains unknown.
A biopsy of the conjunctival ulceration in case 1 revealed infiltration of the ulcer bed, predominantly with neutrophils, small lymphocytes, and plasma cells as well as a small number of eosinophils. In addition, intraepithelial infiltration with neutrophils and their adhesion to the intravascular wall were observed. These findings were consistent with pathological findings of oral ulceration in Behçet's syndrome.6
Immunohistochemical staining of the specimen identified small lymphocytes as both T cells and B cells, supporting the role of T cells in Behçet's syndrome. Indeed, T cells were shown immunohistochemically to be involved in biopsy induced conjunctival inflammation in patients with Behçet's syndrome.7
In conclusion, conjunctival ulceration should be recognised as an uncommon but possible manifestation of Behçet's syndrome. Therefore, routine examination of the conjunctiva is recommended in patients with Behçet's syndrome, and Behçet's syndrome should be included in a list of differential diagnoses for patients with conjunctival ulcerations. In this context, ophthalmologists should look for systemic signs of Behçet's syndrome in patients presenting with conjunctival ulcerations. Once diagnosed, conjunctival ulcerations in Behçet's syndrome show good response to topical corticosteroids. In addition, oral colchicine can be used to reduce episodes of conjunctival as well as oral ulcerations.