Conjunctival Ulcers in Behcet's Disease and Response to Colchicine.

Sir,

A 14-year-old girl presented with painful orogenital ulcers of 4 months duration, associated with intermittent high-grade fever. She developed pain and redness in left eye 2 weeks back and crops of painful nodular lesions over shins 4 days ago along with joint pains in ankle, wrist, and knee joints restricting her mobility significantly. Patient denied prior drug intake, sexual exposure, or personal or family history of tuberculosis.

Mucocutaneous examination revealed painful aphthous ulcers smeared with purulent discharge over buccal, labial, and gingival mucosa [Figure 1a]. Irregular, punched out, and extremely tender ulcers associated with foul-smelling blood-stained mucopurulent discharge were found involving bilateral labia majora, labia minora, and extending onto the vulva [Figure 1b]. There was no regional lymphadenopathy. Examination of left eye showed two adjoining large, circular conjunctival ulcers of size 3 x 3 mm, located in the inferior bulbar conjunctiva about 2 mm below the limbus and associated dilated conjunctival vessels [Figure 1c]. Visual acuity was 6/6 in both eyes. The ulcers stained positive with fluorescin dye with no evidence of uveitis or posterior segment involvement. Examination of right eye was normal. In addition, patient had lesions of erythema nodosum over shins with swelling of ankle joints [Figure 2a].

Figure 1

Multiple aphthous ulcers over buccal, labial, and gingival mucosa (a). Multiple irregular, punched out ulcers over bilateral labia majora, labia minora, and extending onto the vulva (b). Left eye showed two closely placed round ulcers with surrounding erythema and dilated conjunctival vessels (c)

Figure 2

Erythma nodosum over both legs (a). Positive Pathergy test (b)

Routine hematological and biochemical investigation were normal. VDRL (The venereal disease research laboratory), serology for HIV, hepatitis B and HSV (Herpes simplex virus) 1 and 2 were negative. Pus culture from genital ulcers grew methicillin resistant Staphylococcus aureus. Pathergy test was positive after 48 h [Figure 2b].

A diagnosis of Behçet's disease (BD) with ocular involvement was assigned to the patient. She was started on tablet colchicine 0.5 mg thrice daily in addition to appropriate systemic and topical antibiotics to address secondary infection for a week. For eye lesions, loteprednol 0.5% eye drops 4 times a day was prescribed for 3 weeks that was tapered over a period of 4 weeks before stopping completely. Ocular as well as orogenital ulcers showed remarkable improvement in 1 month and healed completely in next 2 months [Figure 3]. Colchicine was continued for additional 6-months with no evidence of relapse of either conjunctival or genital ulcer during 1-year follow-up.

Figure 3

Remarkable improvement in genital and ocular involvement on treatment with colchicine

BD is a chronic, relapsing inflammatory disorder characterized by a classic triad of oral, genital, and cutaneous lesions. Being a multisystem disease, it affects ocular, articular, vascular, and neurological system.[1] International Diagnostic criteria recommends presence of recurrent oral aphthae plus presence of two of the following: genital ulcers, ocular lesion, cutaneous lesion, and positive pathergy test.[2] Ocular manifestations in BD are seen in 36.8–79%.[345] The duration between the disease onset and ocular involvement is usually 2–3 years. Both eyes may be affected simultaneously, but usually one eye is affected before the other. Though 38% patients develop bilateral ocular lesions within a month.[36]

Classically, ocular BD presents as recurrent anterior uveitis with hypopyon so patient complains of photophobia, blurred vision, orbital pain, hyperemia, and excessive lacrimation. Less common manifestations include uveoreitinits, scleritis, episcleritis, and retinal vascultis. If left untreated, recurrence is quite common and may rarely result in optic atrophy and permanent blindness.[34] Conjunctival ulcers in association with BD is rare; reported in 5/540 (0.9%) cases from Turkey and 4/152 (2.6%) cases from Japan.[78] However, in a case series of 29 BD patients by the authors, most common ocular manifestation was conjunctival ulcers in 17.2%.[1] Treatment options for ocular BD include colchicine, cyclosporine along with topical or oral corticosteroids. The ulcers usually heal without any residual scarring with low rate of recurrence. In another study by the authors, only one out of five patients with BD and conjunctival ulcers showed recurrence after complete treatment.[3]

We feel that scarce literature about the occurrence of conjunctival ulcers in BD may be due to under reporting. We, therefore, advocate detailed ophthalmological examination in all BD patients for early diagnosis and treatment. Colchicine is an effective, safe, and economical drug to treat mild to moderate mucocutaneous, arthritic, and ocular manifestations of BD.

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Conflicts of interest

There are no conflicts of interest.