This is a research study among patients with Rosai-Dorfman disease.
Rosai-Dorfman disease is a rare disorder characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body (lymphadenopathy), most often those of the neck (cervical lymphadenopathy). In some cases, abnormal accumulation of histiocytes may occur in other areas of the body besides the lymph nodes (extranodal). These areas include the skin, central nervous system, kidney, and digestive tract. The symptoms and physical findings associated with Rosai-Dorfman disease vary depending upon the specific areas of the body that are affected. The disorder predominantly affects mainly adolescents or young adults. The exact cause of Rosa Dorfman disease is unknown. The purpose of this research study is to learn the prevalence , therapies and long term prognosis of Rosa Dorfman disease.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Enrollment
20
Mycophenolate mofetil oral 500mg twice a day combined with standard of care of oral prednisone 5mg-15mg/day from baseline to week 108
Tongji Hospital
Wuhan, Hubei, China
RECRUITINGTongji Hospital
Wuhan, Hubei, China
RECRUITINGAutoimmune bullous disease quality of life (ABQoL)
Total ABQoL scores range from 0 to 30 . The ABQoL score is calculated by summing the score of each question resulting in a maximum of 30 and a minimum of 0. The higher the score, the more quality of life is impaired.
Time frame: From baseline to 108 Weeks
Cumulative Oral Corticosteroid Dose
Calculate the Cumulative Oral Corticosteroid Dose
Time frame: From 12th, 24th, 36th ,52th and 108 Weeks
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