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Rosai-Dorfman disease (RDD) is a rare non-neoplastic histiocytic proliferative disorder characterized by painless lymphadenopathy. Extranodal lesions frequently occur in the head and neck regions. We report the clinical and histological features of extranodal RDD in a 43-year-old man with a previously unreported combination of multiple gross anterior epibulbar nodules in the right eye, submucosal masses of nasal septum and trachea, and no lymphadenopathy during the 12-year follow-up. The patient underwent ophthalmological, otolaryngological and systemic evaluation; gallium 67 scintigraphy; bronchoscopy; ophthalmic ultrasound; head and neck CT scan; biopsies of epibulbar, nasal and tracheal tissues; and septoplasty. Histological specimens showed lymphocytophagocytosis and positive immunoperoxidase staining for S100 protein in foamy histiocytes; both features were typical for RDD. No response to topical or systemic steroids or to radiation therapy was recorded. Removal of nasal septum masses resolved nasal obstruction. The diagnosis of RDD requires histological and, in challenging cases, immunohistological specimens and is difficult--especially with pure extranodal localizations as in our case. RDD should be suspected in cases of subconjunctival mass and/or submucosal nasal and tracheal swellings not responding to systemic steroids.

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Behcet's disease (BD) is a chronic, relapsing, multisystem disease. In some patients, ocular involvement can lead to severe vision impairment despite immunosuppressive therapy. Since high levels of circulating TNF-alpha have been found both in peripheral blood and aqueous humor of patients with active BD, we evaluated the efficacy of anti-TNF-alpha therapy in seven patients with severe ocular involvement resistant to previous treatment. Seven patients with sight-threatening relapsing uveitis refractory to immunosuppressive regimens received intravenously infliximab, at a dose of 3-5 mg/kg, on week 0-2-4 and then every 6-8 weeks, in combination with low-dose prednisone and methotrexate or azathioprine. Efficacy was assessed in terms of number and severity of relapses of posterior uveitis, visual acuity, and reduction of corticosteroids and immunosuppressive drugs. After a mean follow-up period of 23 months, the total number of relapses dropped to 6, compared to the 21 observed in an equivalent period of time before treatment. The visual acuity improved in 4 eyes, while it remained stable in 9. Therapy with infliximab considerably reduced the required daily dose of both corticosteroids and immunosuppressive drugs. In our experience infliximab proved to be safe and effective in controlling both the number and intensity of cases of posterior uveitis and the extraocular manifestations of BD. It also allowed a reduction of corticosteroids and immunosuppressive drugs required to control the disease. However, ocular and systemic manifestations tended to recur after drug withdrawal or when the interval between infliximab courses was longer than 8 weeks. Moreover, infliximab administration is costly and requires hospital admission.