Coexistence of pemphigus vulgaris and progressive localized scleroderma

In this article, we describe a patient with progressive localized scleroderma, who subsequently developed pemphigus vulgaris on the previously affected sclerodermatous skin, and on the oral mucosa, the nasal mucosa, the laryngeal mucosa, and the scalp. The coexistence of pemphigus vulgaris with localized scleroderma and the histopathologic documentation of pemphigus vulgaris arising on a sclerodermatous skin have not been previously reported. Whether this is a random association or an association based on common immunologic pathomechanisms in pemphigus and localized scleroderma may be important in understanding these diseases.