한국인 천포창 환자의 임상연구

Abstract

Background: Pemphigus is a chronic blistering disease of the skin and mucous membranes, with severe morbidity and occasional mortality. It is necessary to investigate the clinical features, treatment and long-term outcome of Korean patients with pemphigus as it is the most common and severe autoimmune bullous disease in Korea.

Objective: The purpose of this study was to evaluate the clinical features, treatment and prognosis of Korean patients with pemphigus.

Method: A retrospective analysis was conducted on 124 pemphigus patients seen between 1987 and 2003 at Youngdong Severance Hospital.

Results: Out of 124 patients diagnosed with pemphigus during the study period, 62 patients had pemphigus vulgaris (PV), and 62 patients had pemphigus foliaceus (PF). For the 62 PV patients, the mean age of onset was 48.3 years, and the male to female ratio was 1:1.7. For the 62 PF patients, the mean age of onset was 44.7 years, and the male to female ratio was 1.4:1. Average initial dose of oral prednisolone was 30.2mg in PV patients and 19.9mg in PF. Fifty six (90%) patients with PV and 38 (61%) patients with PF received immunosuppressive agents. Partial remission was induced in 9.7%, 23.0%, 41.7%, and 75% of PV patients and in 12.9%, 19.0%, 13.6% and 50% of PF patients after 1,3,5, and 10 years of diagnosis, respectively. Five (8%) of each PV and PF patients died during the treatment period.

Conclusion: The clinical manifestations and results of immunofluorescent studies on 124 Korean pemphigus patients were similar to those of previous studies. Contrast to previous studies, the PV to PF ratio was 1:1, and a male preference was observed on PF. More studies are needed to improve the prognosis and reduce the complications in the treatment of pemphigus patients.