Abstract
BACKGROUND: Kaposi sarcoma, as an epidemiological factor, is associated with acquired immunodeficiency syndrome (AIDS) and it is related to human herpes virus (HHV-8), as well as a higher prevalence in males and non-genital involvement. Vulvar localization is quite infrequent; therefore it may be considered in the differential diagnosis of genital lesions, especially in HIV patients. CASE PRESENTATION: We describe the atypical presentation of a female HIV patient with multiple comorbidities, with the clinical manifestation of Kaposi sarcoma (KS) in a vulvar region that was initially diagnosed as a syphilitic gumma. The patient underwent a biopsy of the lesion, and histopathology revealed a Kaposi sarcoma. DISCUSSION: This case reinforces that the pathogenesis of Kaposi sarcoma is still unclear and that probably multiple factors, regarding both the virus and the patient characteristics may lead to carcinogenesis. CONCLUSION: It is imperative to seek more excellent knowledge about this disease, to facilitate the diagnosis, to warrant the appropriate treatment and to improve the prognosis of the patient, especially the genital lesions.