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.