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 several 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. Although antiretroviral therapy has reduced the prevalence of HIV-related Kaposi sarcoma, Kaposi sarcoma continues to occur in both well controlled HIV infection or AIDS.
Red or purple plaques or nodules on cutaneous (eFigure 6–92) or mucosal surfaces are characteristic (eFigure 6–93) (eFigure 6–94). Marked edema may occur with few or no skin lesions. Kaposi sarcoma commonly involves the gastrointestinal 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.
Kaposi sarcoma on the arm. (Used, with permission, from Lindy Fox, MD.)
Kaposi sarcoma. (Reproduced, with permission, from Bondi EE, Jegasothy BV, Lazarus GS [editors]. Dermatology: Diagnosis & Treatment. Originally published by Appleton & Lange. Copyright © 1991 by The McGraw-Hill Companies, Inc.)
Kaposi sarcoma. Characteristic dark purple macules and nodular plaques.
For Kaposi sarcoma in elders, palliative local therapy with intralesional chemotherapy or radiation is usually all that is required. In the setting of iatrogenic immunosuppression, the treatment of Kaposi sarcoma is primarily reduction of doses of immunosuppressive medications. In AIDS-associated Kaposi sarcoma, the patient should first be given ART. Other therapeutic options include cryotherapy or intralesional vinblastine (0.1–0.5 mg/mL) for cosmetically objectionable lesions; radiation therapy for accessible and space-occupying lesions; and laser surgery for certain intraoral and pharyngeal lesions. Systemic therapy is indicated in patients with skin disease that is symptomatic or asymptomatic but cosmetically unacceptable or those with advanced cutaneous, oral visceral, or nodal disease. ART plus chemotherapy appears to be more effective than ART ...