Rituximab in the treatment of Rosai-Dorfman syndrome with IgG4 disease
A 47-year-old Afro-Caribbean lady presented with a 6-month history of multiple asymptomatic indurated, erythematous papules, nodules and plaques on thighs bilaterally. A deep incisional skin biopsy showed features in keeping with Rosai-Dorfman syndrome (RDD). Screening CT scan and MRI scans revealed retroperitoneal and sacral fibrosis associated with retroperitoneal and para-aortic lymphadenopathy. A retroperitoneal lymph node biopsy excluded an underlying lymphoma. Despite treatment with super potent topical steroids, protopic ointment, dapsone, azathioprine and mycophenolate mofetil, the patient ’s cutaneous lesions and retroperitoneal fibrosis continued to progress.
Source: Journal of the American Academy of Dermatology - Category: Dermatology Source Type: research
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