Pemphigus - Clinical analysis of 129 cases

Pemphigus is a chronic autoimmune bullous disease. It could be life-threatening if not treated appropriately. We retrospectively enrolled 129 cases diagnosed and treated at CGMH form 1998 to 2005. The mean age at onset was 52.6 years with a female to male ratio of 1.15:1. Female patients were significantly younger than male patients (p = 0.012). The frequency of pemphigus was 72% (94) in pemphigus vulgaris, 20% (26) in pemphigus foliaceus, 4% (5) in paraneoplastic pemphigus, 2% (2) in pemphigus vegetants, 1% (1) in pemphigus erythematosus and 1% (1) in IgA pemphigus repectively. In pemphigus vulgaris, mucosal involvement alone was observed in 11%, cutaneous involvement alone in 40% and both in 49%. Indirect immunofluorescence antibody titer of mucocutaneous type pemphigus vulgaris was significantly higher than that of either mucosal or cutaneous type pemphigus vulgaris (p = 0.002, p = 0.019). Complication rate was 57%. These included infections (dermatophytosis, candidiasis, bacterial infection, HSV/HZV, warts, UTI, pulmonary tuberculosis and pulmonary nocardiosis), acne/folliculitis, hypoalbuminemia, hypokalemia, hyperglycemia, GI problem (GI upset/PU/UGI bleeding), Cushing's syndrome, osteroporosis/ osteopenia, steroid-induced hepatitis, steroid-induced myopathy, cataract, psychosis, avascular necrosis of femorus head and elevated liver enzymes in chronic viral hepatitis. Of 129 patients, there were 23 patients hospitalized for further treatment. Analyzing the response of admitted patients, we suggested that high dose steroid has the same weight as pulse therapy in the treatment of pemphigus. And we present our recent treatment protocol of pemphigus.