A 36-year-old Thai woman presented with a painful, burning sensation in the gingiva and desquamative gingivitis. Findings from histopathologic and direct immunofluorescence examination of the maxillary gingiva confirmed a diagnosis of pemphigus. Two months later, she developed a round erythematous lesion on her face, and findings from a skin biopsy were consistent with lupus erythematosus. Four years after initial presentation, the patient developed amyotrophic dermatomyositis. Seven years after initial presentation, she developed joint pain and tenositis of the neck, shoulders, knees and ankles, which was diagnosed as asymmetric polyarthritis and multiple enthesopathy. There are no previous reports of pemphigus, discoid lupus erythematosus, and dermatomyositis in a Thai patient.Fluocinolone acetonide 0.1% in Orabase and systemic steroid were effective in treating oral PV in this patient. The gingiva showed complete remission after 10 months of treatment and remission with recession at 8.5 years of follow-up. (J Oral Sci 55, 255-258, 2013)