Anogenital Lesions: Kaposi's Sarcoma and Its Mimicks

Abstract

Kaposi's sarcoma (KS) is a low-grade vascular neoplasm associated with human herpes virus-8 (HHV-8) infection, and, in the epidemic form, with the human immunodeficiency virus (HIV). Although HHV-8 is present in all body fluids and is sexually transmitted, there are surprisingly few reports of anogenital KS. Clinically, especially in HIV/KS endemic areas, dark stained skin patches or nodules are prone to misdiagnosis, especially in dark-skinned individuals. Therefore, a biopsy is recommended. The histologic appearance spans a broad spectrum of KS and non-KS lesions; therefore, the final diagnosis should be confirmed by HHV-8 immunohistochemistry. We report a series of 36 anogenital biopsies from a group of 16 documented HIV-positive patients; in 20 the HIV serostatus was unknown. There were ten KS (five in HIV-positive patients), and 26 non-KS (11 in HIV-positive subjects) lesions. In the era of HIV/AIDS, anogenital lesions may be the first manifestation of KS in immunocompromised individuals and should be biopsied. The histological diagnosis should be confirmed by HHV-8 immunohistochemistry.

Genital Kaposi sarcoma in a HIV and syphilis co-infected patient: case presentation

“…The etiopathogenesis of KS is not entirely elucidated but is known to include exposure to an infectious agent in addition to HIV [5]. The development of KS is related to HIV infection and, especially, to opportunistic infections that may lead to the inflammatory response to such diseases [6]. Among the infectious agents that contribute to the development of KS, HHV- 8, which is found in more than 90% of KS lesions in individuals with AIDS [2, 4–7], stands out.…”

“…The patient had HIV and HHV-8 and had several concomitant opportunistic infections such as syphilis, which may present aggressive or atypical clinical manifestation, as well as, pulmonary tuberculosis, oral candidiasis and Molluscum contagiosum. Thus, immunosuppression caused by HIV allowed the development of these multiple infections, which in turn triggered inflammatory responses with excessive production of lymphokines and cytokines, which eventually contributed to the development of KS [6]. The co-existence of syphilis and KS in the genital area of this HIV patient caused a difficult treatment challenge to treat both diseases adequately.…”

Primary Classic Kaposi's Sarcoma Confined to the Vulva in an HIV-Negative Patient

“…The most frequently affected sites are the lower limbs and feet; extracutaneous involvement is uncommon 1 . The lesions of KS are rarely localized exclusively in unusual mucocutaneous areas, thus leading to frequent diagnostic errors 2 . To our knowledge, there are no reports of KS confined to female external genitalia not associated with HIV infection.…”

“…Overall, KS restricted to the external genitalia is a very rare finding that is more frequent in men, particularly on the penis; approximately 50 cases have been reported in the English-language literature 3 . Meanwhile, it is very uncommon in women, with only 9 reported cases, all of which involved the vulva 2 , 4 . Most cases of KS of the external genitalia are related to HIV infection.…”

“…In fact, to our knowledge, only 15 well-documented cases of primary penile KS in HIV-negative patients have been reported 5 , while there are no instances of KS confined to female external genitalia not associated with HIV infection. All previous cases of vulvar KS were monolesional forms, clinically presenting as a non-specific mass, nodule, papilloma, or abscess; none were initially suspected to be KS 2 .…”