Pemphigus vulgaris in association with silicosis

We report on a sixty-seven year old miner with pemphigus vulgaris character ised clinically by a three month history of relapsing oral lesions and blis ters/erosions on the trunk, axillae and extremities, histologically by supr abasal cleavage due to acantholysis, immunologically by the epidermal inter cellular net-like pattern due to deposits of IgG- and IgM-antibodies and co mplement C3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysi s. Silicosis has already been known for 6 years. In addition, antinuclear a ntibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were fou nd. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patie nt died of sepsis deriving from recalcitrant pneumonia. Although the associ ation of silicosis with various autoimmune diseases such as systemic sclero sis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge , the second case with features of the two diseases: pemphigus vulgaris and silicosis.