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Long-term prognosis of pemphigus in Korea: retrospective analysis of 199 patients

Title
 Long-term prognosis of pemphigus in Korea: retrospective analysis of 199 patients 
Authors
 Kim M.R. ; Kim H.C. ; Kim S.-C. 
Issue Date
2011
Journal Title
 Dermatology 
ISSN
 1018-8665 
Citation
 Dermatology, Vol.223(2) : 182~188, 2011 
Abstract
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.
URI
http://ir.ymlib.yonsei.ac.kr/handle/22282913/94742
DOI
10.1159/000332848
Appears in Collections:
1. 연구논문 > 1. College of Medicine > Dept. of Dermatology
1. 연구논문 > 1. College of Medicine > Dept. of Preventive Medicine
Yonsei Authors
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Link
 http://www.karger.com/Article/FullText/332848
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