Paediatric Behçet's disease presenting with recurrent papillitis and episcleritis: a case report

Fulvio Parentin,Ingrid Rabach,Loredana Lepore,Stefano Pensiero

doi:10.1186/1752-1947-5-81

Fulvio Parentin, Ingrid Rabach + Show 2 more

Open Access

https://doi.org/10.1186/1752-1947-5-81

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Abstract

IntroductionBehçet'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.Case presentationA 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.ConclusionEpiscleritis 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.

Highlights

Behçet’s disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions
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

Summary

Conclusion

Our report underlines that episcleritis and papillitis without uveitis should be added to the list of uncommon manifestations of pediatric Behçet’s disease. Infliximab seems to be an effective drug for the management of Behçet’s disease that is refractory to the conventional corticosteroid and immunosuppressive therapy. Author details 1Department of Surgery, Ophthalmology Unit, Institute for Maternal and Child Health Burlo Garofolo, Via dell’Istria, 65/1, Trieste I-34100, Italy. LL and IR analyzed the patient data regarding the systemic manifestation of the disease. They provided support in administering the systemic immunosuppressive and biological therapy. All authors read and approved the final manuscript.

Introduction

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International Study Group for Behçet’s Disease