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Paediatric Behçet's disease presenting with recurrent papillitis and episcleritis: a case report
© Parentin et al; licensee BioMed Central Ltd. 2011
Received: 12 January 2010
Accepted: 25 February 2011
Published: 25 February 2011
Behçet's disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Ocular involvement is mainly represented by recurrent uveitis, especially posterior uveitis; however, iridocyclitis, retinal and choroidal vasculitis, optic neuritis and retinal vascular occlusion can also occur.
A 12-year-old Caucasian boy with a history of recurrent buccal aphthosis and nonspecific gastrointestinal symptoms was admitted to our hospital with blurred vision associated with acute episcleritis and papillitis. The patient's pathergy test was positive, suggesting a diagnosis of Behçet's disease. Corticosteroid and cyclosporine therapy was started, but further episodes were noted in both eyes. The patient was then switched to intravenous infliximab, with complete resolution of the inflammation after the second infusion.
Episcleritis and papillitis should be added to the list of uncommon manifestations of pediatric Behçet's disease. Infliximab is an effective, new therapeutic approach for Behçet's disease that is refractory to the conventional corticosteroid and immunosuppressive therapy.
Behçet's disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions . The hallmark of ocular involvement is recurrent uveitis, especially posterior uveitis; however, iridocyclitis, retinal and choroidal vasculitis, optic neuritis and retinal vascular occlusion can also occur . Other ocular manifestations, such as scleritis, are very unusual and are described only among adult patients [3, 4]. We report an exceedingly rare manifestation of childhood-onset Behçet's disease occurring together with recurrent and simultaneous episcleritis and papillitis.
Childhood-onset Behçet's disease is uncommon, accounting for 3% to 7% of all cases . Scleritis may be a harbinger of systemic autoimmune inflammatory diseases. Few publications have reported an association between Behçet's disease and scleral inflammation, and only among adult patients. Episcleritis is a benign inflammation confined to the episcleral tissue; patients with episcleritis usually complain of mild pain. In 2004, Dursun et al.  reported a case of systemic Behçet's disease with anterior necrotizing scleritis, lateral rectus myositis and recurrent retrobulbar optic neuritis. Nodular and diffuse scleritis is characterized by edema and injection in both the superficial and deep episcleral vessels, and pain is usually severe and deep-seated. Finally, scleromalacia perforans is a necrotizing scleritis, relatively asymptomatic and without inflammation. In 2005 Sakellariou et al.  reported the unique association between Behçet's disease and scleromalacia perforans.
Optic neuritis is a rare manifestation of neuro-Behçhet's disease. A search of MEDLINE retrieved only one case report of inflammatory optic nerve involvement in the pediatric age group . The diagnosis in children is difficult, as the disease is uncommon and clinically resembles other diseases, such as multiple sclerosis. Another important differential diagnosis is central venous sinus thrombosis, which is well described in Behçet's disease and is usually characterized by severe headache and deterioration in general condition.
Infliximab, a chimeric monoclonal antibody to TNF-α, was developed and used to treat systemic inflammatory disorders such as rheumatoid arthritis and Crohn's disease. Proinflammatory cytokines, including TNF-α, are known to be elevated in active Behçet's disease, suggesting that anti-TNF-α therapy might be effective. Clinically, significant improvement of various Behçet's disease manifestations with infliximab therapy has been reported in the literature [6, 7]. A recent study demonstrated that the effectiveness of infliximab on the ocular inflammation in Behçet's disease correlates with the infliximab serum concentrations .
First, our report underlines that episcleritis and papillitis without uveitis should be added to the list of uncommon manifestations of pediatric Behçet's disease. Second, infliximab seems to be an effective drug for the management of Behçet's disease that is refractory to the conventional corticosteroid and immunosuppressive therapy. The selection of optimal dose and frequency of infusion required standardization for our patient.
Written informed consent was obtained from the patient's legal guardian for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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