Abstract: Vulvar Paget disease is a rare malignant tumor of the skin that usually occurs in postmenopausal women. It causes symptoms such as itching, local pain or burning sensation of the vulva. It usually presents as erythema or eczema-like changes of the skin of the vulva. The diagnosis is confirmed by pathological biopsy. Vulvar Paget disease can be classified into primary and secondary vulvar Paget disease. Immunohistochemical stains can be used to make a relatively clear differential diagnosis of vulvar Paget disease. Due to its non-specific clinical manifestations and atypical pathological features, the rate of misdiagnosis and delayed diagnosis is high. Surgical treatment is the first choice for the treatment of vulva Paget disease. However, the formulation of surgical scope is a difficulty of current treatment. If the surgical scope is too large, it may cause serious tissue damage and dysfunction. Topical imiquimod cream and photodynamic therapy have provided new ideas for the conservative treatment in recent years. Regardless of surgical treatment or conservative treatment, the recurrence rate of vulvar Paget disease is high. Its prognosis is closely related to pathological type, lymph node metastasis and infiltration depth. It is necessary to conduct long-term follow up for patients with the disease. This article will review the progress in diagnosis and treatment of vulva Paget disease.

Key words: Vulvar Paget disease;, Postmenopause;, Paget disease, extramammary;, Skin;, Vulvar neoplasms;, Diagnosis, Therapy