Extramammary Paget's disease of the oral mucosa and perioral skin

Abstract

Extramammary Paget's disease (EMPD) is an unusual intraepidermal adenocarcinoma. Only 2 cases of EMPD in the oral mucosa have been reported previously. Here, we present the first report of a rare case of Paget's disease of the oral mucosa and perioral skin with extensive contiguous erythematous and erosive manifestations. Ultimately, the patient was treated with oral thalidomide for 8 weeks. We noticed an improvement with resolution of symptoms. A 3-year follow-up showed no relapse. Thus, this case may provide new insights to clinicians about treatment of EMPD. More importantly, we advise that dentists be vigilant about the possibility of some clinically significant conditions in patients with extensive nonspecific clinical lesions mimicking inflammatory or infectious diseases in the oral and maxillofacial regions, especially lesions that are refractory to long-term treatment. Requesting consultations with relevant clinical departments promptly and obtaining histopathologic confirmation at an early stage are crucial for early therapy and a better prognosis.