Clinical features and course of pemphigus in Thai patients.

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 12 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).