Early invasive vulvar squamous cell carcinoma arising in a woman with vulvar pemphigus vulgaris and systemic lupus erythematosus

A 27-year-old Caucasian woman was admitted to our Gynaecology Unit for bleeding vegetant lesions of the vulva. Her history was characterized by systemic lupus erythematosus and PV. Biopsy showed concomitant PV and vulvar intraepithelial neoplasia (VIN) grade 3. One month later a new biopsy revealed progression from VIN 3 to early SCC. Despite chemotherapy, no remission of disease was observed. She died six months after diagnosisOur case underlines PV as another chronic inflammatory disease of the lower genital tract predisposing to VIN-SCC. It suggests the need for careful follow-up of patients with chronic inflammatory disease, especially when concomitant autoimmune disorders are present. Moreover, a biopsy should be always performed if there are PV lesions because of the possibility of neoplastic disease.Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes characterized by the presence of autoantibodies targeting desmoglein 3, a surface antigen of keratinocytes involved in maintaining cell-cell junctions [1]. Its incidence is 0.1-3.2 cases per 100,000 individuals per year. PV typically runs a chronic course, with blisters, painful erosions and ulcers on the mucosa and skin [2,3]. The sites most commonly involved are the oral mucosa, pharynx, larynx, oesophagus, conjunctiva and anal mucosa [1-4]. Involvement of the genital tract in women with PV has rarely been reported [5]. Usually, genital involvement occurs when most other common sites are concurrently affected or are in remission [1,6].Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many parts of the body and the skin with occasional bullous lesions. Pemphigus vulgaris may exceptionally be associated with other blistering diseases such as SLE [7].Here, we report a rare case of a woman affected with SLE presenting with early invasive squamous cell carcinoma (SCC) arising from Pemphigus Vulgaris of the vulva.A 27-year-old Caucasian woman was admi