ABSTRACT
Background: Pemphigus is a rare, organ-specific autoimmune disease. The epidemiology and clinical course vary between reports from different countries. Objective: To evaluate clinical manifestations, investigation and clinical course of Thai patients with pemphigus. Methods: Demographic data, clinical presentations, laboratory investigations and treatment outcomes in 124 pemphigus patients who had attended the specialized autoimmune skin clinic at Siriraj Hospital during the period from January 1991 to December 2009 were retrospectively studied. Results: Of the 124 pemphigus patients, 79% were diagnosed with pemphigus vulgaris (PV) and 15.3% with pemphigus foliaceus (PF). The male to female ratio was approximately 1:2 in both groups. The mean age of onset was 45.4 years in PV patients and 57.4 years in PF patients. Oral mucosal involvement at the onset of disease was presented in 37.8% of PV patients. The sensitivity and specificity of DIF in the diagnosis of pemphigus was 97.8% and 98.3% while that of IIF was 94.7% and 98.4%. Disease control was achieved in 93.9% of PV patients and 94.7% of PF patients. Remission (off therapy) was achieved in 31.6% of patients in both groups. Conclusions: PV is the most common subtype of pemphigus in Thailand and usually affects females more than males. The disease usually occurs in the fifth decade of life and mucosal involvement is common. Immunofluorescence studies yields very high sensitivity and specificity. Corticosteroids are the mainstay of treatment. The majority of patients attain disease control and one-third of them achieve remission (off therapy).
ABSTRACT
Background: Bullous pemphigoid (BP) is a rare, subepidermal autoimmune blistering disease. Studies from different regions show discrepancies in clinical features and courses. Objectives: To reveal clinical characteristics, investigations and clinical outcomes of Thai patients with BP and to evaluate the association of BP with malignancy, diabetes mellitus and neurologic diseases. Methods: Patients diagnosed as BP who had visited the autoimmune skin clinic at Siriraj Hospital between 1991 and 2009 were retrospectively studied. Results: Fifty-eight patients were enrolled. Mean age of onset was 69.3 years. The female to male ratio was 2.7:1. Fifteen percent of the patients had mucosal involvement and 38.9% showed peripheral blood eosinophilia. The sensitivity of the direct and indirect immunofluorescence test in the diagnosis of BP was 95.7% and 73.5%, respectively. The frequency of diabetes mellitus in BP patients was significantly higher than that in the general population (p < 0.001). BP patients had a significantly higher chance of having neurologic diseases compared with other autoimmune vesiculobullous disease patients (adjusted odd ratios 4, 95% confidence interval 1.2-13.3). Disease control was achieved in 89.7% of the patients. One-year and three-year remission rate was 6.4% and 66.3%, respectively. Conclusions: BP usually occurred in the seventh and eighth decade of life and affected females more than males. BP is associated with diabetes mellitus and neurologic diseases. Corticosteroids are the mainstay of the treatment. Two-thirds of patients are likely to be in remission within three years.