Long-term prognosis of pemphigus in Korea: retrospective analysis of 199 patients.
BACKGROUND
Pemphigus is the most common and severe autoimmune bullous disease in Korea.


OBJECTIVE
The aim of this clinical study was to evaluate the clinical features, treatment modalities and long-term prognosis of pemphigus in Korea.


METHODS
We conducted a retrospective analysis of 199 patients diagnosed with pemphigus vulgaris (PV) and pemphigus foliaceus (PF) between 1993 and 2008. Complete and partial remissions were evaluated according to the definitions proposed by the International Pemphigus Committee.


RESULTS
The mean age of onset was 46.1 years, and the female-to-male ratio was 1.1:1.0 (women: 102, men: 97). The ratio of PV to PF was 1.1:1.0 (PV: 104, PF: 95). Complete/partial remission rate for PV was 77% at 5 years and 94% at 10 years after initial diagnosis. The corresponding rate for PF was 87% at 5 years and 98% at 10 years after initial diagnosis. There was no difference in time to remission between mild cases (treated with prednisolone, Pd, alone) and severe cases (treated with Pd ± adjuvant therapy). Sixteen patients, who were recalcitrant to conventional therapy, received rituximab; all of these achieved overall remission without adverse effects. Eight (8%) PV patients and 5 (5%) PF patients died during the 16-year follow-up period.


CONCLUSIONS
Most patients reached remission within 10 years of treatment. Pd + adjuvant therapy did not yield a better prognosis than Pd alone. All patients who received rituximab experienced remarkable clinical improvement. Rituximab is an effective and safe drug for patients who are resistant to conventional therapy.
Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
Mi Ri
MR
Kim
Hyeon Chang
HC
Kim
Soo-Chan
SC
Kim
D000328:Adult; D000368:Aged; D000890:Anti-Infective Agents / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D010392:Pemphigus / Q000188:drug therapy* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use*; D011379:Prognosis; D056910:Republic of Korea; D012189:Retrospective Studies; D000069283:Rituximab; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
D000890:Anti-Infective Agents; D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D016572:Cyclosporine; D003520:Cyclophosphamide; D003622:Dapsone; D011239:Prednisolone; D009173:Mycophenolic Acid; D001379:Azathioprine; D008775:Methylprednisolone