Can Behçet's Syndrome Present with Ocular and Oral Involvement Alone?
Yes, Behçet's syndrome can absolutely present with ocular inflammation and oral ulcerations without nasal or genital involvement. The diagnostic criteria do not require all manifestations to be present simultaneously, and the disease follows a relapsing-remitting course where different organ systems may be affected at different times.
Diagnostic Framework
The diagnosis of Behçet's disease requires recurrent oral aphthous ulcers plus at least two of four additional criteria: recurrent genital ulceration, uveitis/retinal vasculitis, specific skin lesions, or positive pathergy test 1, 2. This means a patient with oral ulcers and ocular involvement already meets diagnostic criteria without genital or nasal manifestations.
Key Clinical Points:
Almost all patients with Behçet's syndrome have recurrent oral aphthae as the most common manifestation, but the presence of other features varies considerably 3.
Ocular involvement can be an initial presenting symptom in 10-20% of cases, sometimes appearing before genital ulcers or other manifestations develop 4.
Nasal involvement is not part of the standard diagnostic criteria and is not required for diagnosis 1, 2.
Genital ulcers, while common, do not occur in all patients, and their absence does not exclude the diagnosis when other criteria are met 5, 3.
Clinical Implications for This Presentation
Immediate Management Priorities:
Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment must immediately start azathioprine (2.5 mg/kg/day) combined with systemic corticosteroids 1, 6. Corticosteroids should never be used alone for posterior segment involvement 6.
Risk Stratification:
Ocular involvement is associated with poor prognosis and can cause serious damage including blindness if untreated 1, 7, 8.
Young males with early disease onset follow a more severe disease course, warranting more aggressive treatment and closer monitoring 1, 6, 7.
The typical ocular pattern is relapsing-remitting panuveitis and retinal vasculitis, with initial attacks potentially improving spontaneously but recurring if untreated 8.
Treatment Escalation Criteria:
For severe eye disease (>2 lines drop in visual acuity on a 10/10 scale and/or retinal vasculitis or macular involvement), escalate immediately to either infliximab or cyclosporine A in combination with azathioprine and corticosteroids 1, 6. Interferon-alpha represents an alternative second-line option 1, 6.
Common Pitfalls to Avoid
Do not delay immunosuppression waiting for genital ulcers or other manifestations to appear - the presence of sight-threatening ocular disease demands immediate aggressive treatment regardless of other features 1, 7.
Avoid cyclosporine A if any CNS involvement is present or suspected due to neurotoxicity risk 1, 7.
Do not undertreate based on incomplete manifestations - organ-threatening disease requires full immunosuppression even when mucocutaneous features are limited 1, 7.
Remember that disease manifestations may appear sequentially over time rather than simultaneously, so absence of genital or nasal involvement at presentation does not rule out future development 1, 7.