A 71-year-old man was referred to us because of erythema with erosions and nodules on the genital area. It had gradually enlarged over several years. Physical examination revealed an asymptomatic erythematous plaque with erosions and nodules, 200×100mm in size, on the genital area. A biopsy specimen showed not only large round cells with ample pale-stained cytoplasm in the epidermis and mononuclear inflammatory cell infiltration in the upper dermis, but also elastorrhexis in the middle to lower dermis. With Elastic van Gieson stain and von Kossa stain, we confirmed marked degeneration of elastic fibers with calcium deposition. Physical reexamination revealed asymptomatic pale-yellowish papules, 1 to 3mm in diameter, on the lateral neck, groin, and proximal femur. We diagnosed the erythematous plaque on the groin with extramammary Paget's disease as pseudoxanthoma elasticum. Our case reemphasizes the importance of complete clinical and histopathologic assessment as extramammary Paget's disease and other skin disorders including pseudoxanthoma elasticum may occasionally be identified in elderly patients.Skin Research, 13: 421-425, 2014
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