Pemphigus vulgaris has never before been associated with silicosis, although there are many reports of silicosis accompanied by several autoimmune diseases such as progressive systemic sclerosis, systemic lupus erythematosus, dermatomyositis or rheumatoid arthritis. We observed a patient with pemphigus vulgaris accompanied with silicosis. The patient was a 75-year-old man with a 2-month history of repeated oral erosions and blisters on the back, thighs and axillas. Histological examination showed suprabasal cleavage with acantholysis. Immunoblotting analysis demonstrated binding of the patient’s serum to the 130-kD pemphigus vulgaris antigen (desmoglein 3) and the 160-kD pemphigus foliaceus antigen (desmoglein 1). The patient has radiographically been diagnosed as having silicosis. An elevated serum IgG, antinuclear antibody, anti-ssDNA, antimicrosomal antibodies and a biologically false-positive reaction to the Wassermann test were also detected. Although the clinical symptoms improved after treatment with systemic steroids, the patient died due to pneumonia. This is the first reported case in which the characteristics of both pemphigus vulgaris and silicosis could be detected.

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