Paget's disease of the vulva in a patient with scleroderma and underlying adenocarcinoma: case report.

Systemic sclerosis (SSc) is a multisystem connective tissue disease characterized by fibrosis of the skin and internal organs. Several studies have demonstrated an increased frequency of cancer in patients with SSc. We report a case of a 71-year-old woman with SSc, who presented with an eczematous lesion of the vulva. The diagnosis of Paget's disease of the vulva (PVD) was established. The patient underwent radical vulvectomy, but 18 months later died due to adenocarcinoma of unknown primary origin. SSc and PVD are associated with various types of malignancies and patients suffering from these diseases should be under surveillance in order for any suspicious symptoms of malignancy to be early detected and investigated.