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Human herpes virus 8 (HHV-8), or Kaposi sarcoma–associated herpes virus, is the cause of all forms of Kaposi sarcoma. Kaposi sarcoma occurs in five forms. Classic Kaposi sarcoma occurs in older men, has a chronic clinical course, and is rarely fatal. Endemic Kaposi sarcoma occurs in an often aggressive form in young Black men of equatorial Africa. Iatrogenic Kaposi sarcoma occurs in patients receiving immunosuppressive therapy and improves upon decreasing immunosuppression. Epidemic Kaposi sarcoma is associated with HIV-associated immune deficiency. A fifth type is an indolent form of Kaposi sarcoma that occurs exclusively in HIV-negative men who have sex with men.

Red or purple plaques or nodules on cutaneous (eFigure 6–17)(eFigure 6–18) (eFigure 6–19) or mucosal surfaces are characteristic. Marked edema may occur with few or no skin lesions. Kaposi sarcoma commonly involves the GI tract and can be screened for by fecal occult blood testing. In asymptomatic patients, these lesions are not sought or treated. Pulmonary Kaposi sarcoma can present with shortness of breath, cough, hemoptysis, or chest pain; it may be asymptomatic, appearing only on chest radiograph. Bronchoscopy may be indicated. The incidence of AIDS-associated Kaposi sarcoma is diminishing. However, chronic Kaposi sarcoma can develop in patients with HIV infection, high CD4 counts, and low viral loads. In this setting, the Kaposi sarcoma usually resembles the endemic form, being indolent and localized. At times, however, it can be clinically aggressive. The presence of Kaposi sarcoma at the time of antiretroviral initiation is associated with Kaposi sarcoma–immune reconstitution inflammatory syndrome, which has an especially aggressive course in patients with visceral disease.

eFigure 6–17.

Kaposi sarcoma on the arm. (Used, with permission, from Lindy Fox, MD.)

eFigure 6–18.

A: Kaposi sarcoma presenting as purplish-red papules on the chest and arm of an HIV-positive man. His Kaposi sarcoma was disseminated and was found in his colon. B: Kaposi sarcoma on the foot of a 28-year-old HIV-positive man. Note the purplish-red color. C: Kaposi sarcoma presenting with purplish-red color on the palate of the same man in (A) above. D: Kaposi sarcoma causing gingival hypertrophy. Always look in the mouth when suspecting Kaposi sarcoma. (Reproduced with permission from Richard P. Usatine, MD, in Usatine RP, Smith MA, Mayeaux EJ Jr, Chumley HS. The Color Atlas and Synopsis of Family Medicine, 3rd ed. McGraw-Hill, 2019.)

eFigure 6–19.

Kaposi sarcoma. Characteristic dark purple macules and nodular plaques.


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