Rituximab, a new treatment for difficult-to-treat chronic XXXX ? Five cases.

@entity1 @entity32 @entity3761 @entity7271 @entity2816 @entity102 @entity2025 @entity230 @entity792 @entity9374
BACKGROUND: @entity9374 ( @entity9374 ) is an @entity32 affecting skin and mucosae, often triggered by @entity2025 . Some @entity1 have a @entity102 associated with antidesmoplakin autoantibodies, but the pathophysiology remains to be elucidated. First-line treatment is antiviral therapy. With treatment failure or in @entity1 without herpes-triggered disease, @entity2816 is effective but has neurological side-effects. Alternatives ( @entity3761 , immunosuppressant agents) are not codified. For many @entity1 , systemic @entity230 use is chronic. The immunosuppressant drug rituximab (RTX) may be effective. OBJECTIVES: We report five cases of severe chronic @entity9374 treated with rituximab (RTX). METHODS: Five @entity1 with severe chronic @entity9374 for 9-20  years received RTX after failure or side-effects of several treatments, especially antiviral therapy and @entity2816 . All had chronic use of @entity230 . Four patients had antidesmoplakin autoantibodies. RESULTS: Four @entity1 experienced complete or quasi-complete remission of @entity9374 with withdrawal of @entity230 and one @entity1 partial remission, for 3-11  months. Disease relapsed in all @entity1 , and three received a second cycle of RTX with shorter duration of efficacy. Two @entity1 received a third cycle, one without efficacy. CONCLUSION: The use of RTX for many @entity792 , especially @entity7271 , is increasing. Chronic @entity9374 , especially @entity9374 associated to antidesmoplakin autoantibodies, is an @entity32 in which the role of B cells is not well understood. However, we report a favourable benefit of RTX treatment for months in five @entity1 with severe disease. RTX could be a therapeutic option in severe, difficult-to-treat @entity9374 .
