• Classic idiopathic form affecting extremeties • Endemic form (African) • Immunosuppression-associated form • Acquired immunodeficiency syndrome (AIDS)-associated form
• All forms, especially AIDS-associated and immunosuppressionassociated forms, may be caused by or closely related to a herpesvirus (human herpesvirus 8 [HHV-8] or Kaposi’s sarcoma–associated herpesvirus [KSHV]).
Clinical Presentation • Classic form associated with slow but pernicious growth over
many years; oral lesions rarely seen • Endemic form more rapid; oral lesions rarely seen • AIDS-associated KS most commonly seen on keratinized
mucosa/mucoperiosteal tissues; strong predilection for hard palate, followed by gingiva, buccal mucosa, and tongue (prevalence decreasing with treatment for AIDS)
• Evolution from bluish macule to nodule(s) • Evolution to multiple lesions • May precede or follow cutaneous lesions • Usually asymptomatic
Diagnosis • Location and appearance • May occur in up to one-third of AIDS patients • Biopsy showing spindle cell proliferation with vascular slits,
extravascular red blood cells
Differential Diagnosis • Hematoma • Hemangioma • Ecchymosis • Malignant melanoma • Pyogenic granuloma • Amalgam tattoo
Treatment of AIDS-Associated Form • Radiation therapy: single fraction or equivalent fractionated
therapy of 800 cGy • Intralesional therapy: interferon-α, vincristine, vinblastine
(2 mg/cc), sclerosing agents (sodium morrhuate) • Systemic chemotherapy: interferon-α, vincristine, vinblastine,
bleomycin, daunorubicin • Most treatment is palliatively directed.
Prognosis • Variable, depending upon host’s immune status, but generally
poor in AIDS-associated form