Pemphigus
Conditions
Interventions
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1. Patients who meet the diagnostic criteria for pemphigus, especially those with long-term oral administration of corticosteriods or immunosuppressive agents; Clinical manifestations: (1) multiple looseness on the skin, easy to rupture bullae; (2) refractory erosion surface secondary to blisters, scales or scars; (3) non-infectious blisters or erosions in the mucosal area (including the oral mucosa); (4) Nikolsky sign positive: 1) Histopathology: acanthosis; blister formation between epidermal cells; 2) Immunodiagnostic indicators: normal skin direct immunofluorescence (DIF) around the lesion area or lesions indicates IgG and/or complement deposition between epidermal cells; indirect immunofluorescence (IIF) detects anti-cell interphase in serum Anti-Dsg antibodies were detected in the serum by antibodies or ELISA. 2. Aged 18 to 80 years old; 3. Informed consent has been signed; 4. Women of childbearing age who have no birth plan.
Exclusion criteria
Exclusion criteria: (1) Pregnant or lactating women; (2) Women of childbearing age who have a birth plan; (3) Those who are allergic to the drug; (4) Patients with active hepatitis virus replication; (5) HIV-positive patients; patients with potential tuberculosis infection; (6) Combined with other diseases such as tumors, obvious systemic infections, arrhythmia, angina pectoris, cardiac insufficiency, etc.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Partial remission rate;Complete remission rate; | — |
Secondary
| Measure | Time frame |
|---|---|
| Desmosin antibody 1;Desmosin antibody 3;B cell count;Pemphigus Disease Area Index; | — |
Countries
China
Contacts
West China hospital, Sichuan University