Pemphigus in Korea: clinical manifestations and treatment protocol.
Pemphigus, a rare, chronic blistering disease of the skin and mucous membranes with severe morbidity and occasional mortality, is the most common autoimmune bullous disease in Korea. The purpose of this study was to evaluate the clinical features and propose a treatment strategy for patients with pemphigus. A retrospective analysis was conducted of 51 pemphigus patients seen between 1993 and 2001. Pemphigus vulgaris (PV) was the most common type with 32 cases, followed by 19 cases of pemphigus foliaceus (PF). The male to female ratio was 1:1.3, with females predominating, particularly among PV patients (PV, 1:1.5; PF, 1:1.1). The average ages at onset of PV and PF were 44.3 and 51.0 years old, respectively. Mucosal involvement was noted in 27 cases (84.4%) of PV but in only 3 cases (15.8%) of PF. Most patients initially received relatively low to intermediate doses (0.3-1.0 mg/kg/day) of prednisolone, and 23 (71.9%) PV patients and 10 (52.6%) PF patients also received immunosuppressive agents. Oral prednisolone and azathioprine (100 mg/day) formed the mainstay of treatment for our patients (47.1%). At the time of writing, 25.5% (13/51) of patients are in complete remission, and 72.5% (37/51) are undergoing maintenance therapy. One patient died due to sepsis during the treatment. For the treatment of pemphigus, a course of the lowest possible corticosteroid dosage in combination with immunosuppressive agents appears to be effective and less toxic than a high corticosteroid dosage.
Seo
Pan Gyo
PG
Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea.
Choi
Won Woo
WW
Chung
Jin Ho
JH
D000284:Administration, Oral; D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001379:Azathioprine / Q000008:administration & dosage; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007723:Korea / Q000453:epidemiology; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D010392:Pemphigus / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology; D011239:Prednisolone / Q000008:administration & dosage; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors
D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine