Stanley JR, Amagai M. Pemphigus, bullous impetigo, and staphylococcal scalded-skin syndrome. NEJM 2006
Pathogenesis of desmoglein autoantibodies and differential diagnosis
Landmark trials
RITUX 3 trial
Joly P et al. First-line rituximab combined with short-term prednisone versus prednisone alone. Lancet. 2017. PMID 28342637
89% complete remission with rituximab plus prednisone at 24 months versus 34% with prednisone alone
Tedbirt B et al. Sustained remission without corticosteroids following RITUX 3 protocol. JAMA Dermatology. 2024
72% disease-free survival at 7 years without additional rituximab
PEMPHIX trial
Werth VP, Joly P, Mimouni D et al. Rituximab versus mycophenolate mofetil in pemphigus vulgaris. NEJM. 2021
Rituximab produced 6 flares versus 44 flares with mycophenolate over 52 weeks
Relapse prediction
Hebert V et al. Optimizing pemphigus management with rituximab and short-term relapse predictors. JAMA Dermatology. 2025
Baseline PDAI greater than or equal to 45, anti-Dsg1 greater than 20 IU/mL, or anti-Dsg3 greater than 130 IU/mL at month 3 predict relapse
Drug-induced pemphigus
Ghaedi F et al. Drug-induced pemphigus: systematic review of 170 patients. Int Immunopharmacol. 2021. PMID 33418246
25% resolve with drug cessation alone; 75% require immunosuppression
Baroukhian J et al. Comprehensive population-level evaluation of drug triggers of pemphigus. Front Immunol. 2024. PMID 39906745
Immunomodulatory capacity as common characteristic of pemphigus drug triggers
Trigger and predisposing factors
Moro F et al. Pemphigus: trigger and predisposing factors. Front Med. 2024. PMID 38213911
Environmental, infectious, and dietary triggers; HLA associations
Pathak GN et al. Pemphigus relapse: mechanisms, risk factors, and agents. J Dermatol. 2024. PMID 39460496
Relapse risk factors and mechanism of recurrence after rituximab
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