作者: Kanokvalai Kulthanan , Wararat Sirikudta , Leena Chularojanamontri , Samruay Pinkaew , Papapit Tuchinda
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摘要: Background: Pemphigus is a rare, organ-specific autoimmune disease. The epidemiology and clinical course vary between reports from different countries. Objective: To evaluate manifestations, investigation of Thai patients with pemphigus. Methods: Demographic data, presentations, laboratory investigations treatment outcomes in 124 pemphigus who had attended the specialized skin clinic at Siriraj Hospital during period January 1991 to December 2009 were retrospectively studied. Results: Of patients, 79% diagnosed vulgaris (PV) 15.3% foliaceus (PF). male female ratio was approximately 1:2 both groups. mean age onset 45.4 years PV 57.4 PF patients. Oral mucosal involvement disease presented 37.8% sensitivity specificity DIF diagnosis 97.8% 98.3% while that IIF 94.7% 98.4%. Disease control achieved 93.9% Remission (off therapy) 31.6% Conclusions: PV most common subtype Thailand usually affects females more than males. occurs fifth decade life common. Immunofluorescence studies yields very high specificity. Corticosteroids are mainstay treatment. majority attain one-third them achieve remission therapy).