Vulvar Paget's Disease Presenting with Fever and Left Inguinal and Peritoneal Lymphadenopathies

BACKGROUND Asymptomatic vulvar Paget's disease is rare and commonly presents with vulvar eczema, erosions, or pruritus. The time from onset to diagnosis of vulvar Paget's disease tends to be rather long because of difficulty making a correct diagnosis owing to similar skin findings with eczema or patients' reluctance to undergo physical examination of their pubic area because of embarrassment. CASE REPORT A 55-year-old woman experienced recurrent episodes of fever for 10 months. Her primary care physician indicated inguinal lymphadenopathy 2 months prior to presentation at our hospital. Contrast-enhanced abdominal computed tomography revealed multiple intra-abdominal lymphadenopathies. With the failure of finding the primary lesion after biopsy, and with a diagnosis of metastatic carcinoma, she was referred to our hospital. On admission, she did not report having vulvar symptoms. As imaging studies revealed no primary lesions, we subsequently added immunostaining to the lymph node biopsy specimens, which suggested Paget's disease. We finally performed a vulvar physical examination and identified eczema. We performed a skin biopsy and histopathological examinations, which provided the final diagnosis of vulvar Paget's disease. CONCLUSIONS We experienced a case of vulvar Paget's disease presenting with inguinal and intraperitoneal lymphadenopathies, without a patient report of vulvar symptoms. When identifying lymphadenopathies, it is crucial to obtain a careful history and perform appropriate physical examinations, suspecting diseases of the vulva or perineum. In addition, immunostaining of lymph node biopsy specimens could be useful in making a correct diagnosis.