title: Early invasive vulvar squamous cell carcinoma arising in a woman with vulvar pemphigus vulgaris and systemic lupus erythematosus.
abstract: BACKGROUND
  Pemphigus vulgaris (PV) is an autoimmune blistering disease of the skin and mucous membranes. Genital involvement occurs when most other common sites are concurrently affected or are in remission. 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 and SLE may be associated, albeit rarely. Here, we report the first case of a woman affected with SLE presenting with early invasive squamous cell carcinoma (SCC) arising from Pemphigus Vulgaris of the vulva.
  
  
  METHODS
  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 diagnosis
  
  
  CONCLUSIONS
  Our 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.
authors.affiliation: Department of Gynecology and Obstetrics, and Pathophysiology of Human Reproduction, University of Naples "Federico II", Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Bifulco
authors.forename: Vincenzo D
authors.initials: VD
authors.lastname: Mandato
authors.forename: Roberto
authors.initials: R
authors.lastname: Piccoli
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Giampaolino
authors.forename: Chiara
authors.initials: C
authors.lastname: Mignogna
authors.forename: Michele D
authors.initials: MD
authors.lastname: Mignogna
authors.forename: Luigi
authors.initials: L
authors.lastname: Costagliola
authors.forename: Carmine
authors.initials: C
authors.lastname: Nappi
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D002278:Carcinoma in Situ / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D002294:Carcinoma, Squamous Cell / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D009361:Neoplasm Invasiveness; D009367:Neoplasm Staging; D010392:Pemphigus / Q000150:complications* / Q000473:pathology; D013997:Time Factors; D014845:Vulvar Diseases / Q000150:complications* / Q000473:pathology; D014846:Vulvar Neoplasms / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology