CASE #2: Kaposi's sarcoma
This patient had Kaposi's sarcoma (KS), a cancerlike condition caused by human herpesvirus 8. There are four forms of KS. Classic KS was first described more than a century ago. This form usually affects middle-aged and older American men of Mediterranean and Eastern European (Ashkenazi) Jewish lineage. It produces painful and often disfiguring leg tumors, but these are rarely life-threatening. Endemic KS primarily occurs in boys and men from east Africa and the Congo. Iatrogenic KS is typically found in transplant patients who take immunosuppressive drugs to prevent rejection. This form can be reversed once the medication is stopped. The final form of KS is HIV-associated, which is what our patient had.
One of the most visible signs of the disease, HIV-associated KS usually manifests as violaceous patches, papules, plaques, or nodules on the face and trunk. These may be preceded by chronic lymphedema. Early lesions often mimic insect bites. The nodules tend to enlarge into dome-shaped tumors. In advanced stages, lesions may be disseminated on the face, head, and trunk, often forming large plaques.
In some cases (10%-15%), lesions first appear on the oral mucosa, most frequently the palate. This can cause difficulties with swallowing or eating. Involvement of the pharynx can lead to respiratory distress.
Although rare, tumors can also occur on the GI tract, lungs, and lymph nodes. GI involvement is found in up to 80% of AIDS patients with KS, especially those with extensive cutaneous lesions. Symptoms can include nausea, ulceration, blockage, and bleeding. Pulmonary involvement may cause severe coughing, shortness of breath, and respiratory distress. Lymph node involvement may result in swelling of the face or extremities. Prognosis is poor with extracutaneous KS, and morbidity and mortality is often the result of bleeding in the affected organ systems.
The differential diagnosis for HIV-associated KS includes eruptive dermatofibromas, pyogenic granulomas, bacillary angiomatosis, lymphoma, leukemia, vasculitis, and arteriovenous malformations (pseudo-Kaposi's sarcoma). A diagnosis is often made on visual inspection. Lesions of KS are usually painless, with no pruritus or drainage. When pressed, they do not lose their color.
If KS is suspected, a definitive diagnosis can be made by biopsy. A proliferation of slitlike blood vessels with atypical endothelial cells is characteristic; increased numbers of plasma cells and hemosiderin deposits are also seen. If internal involvement is suspected, bronchoscopy, endoscopy, stool guaiac testing, and chest x-ray or abdominal CT may be required.
Highly active antiretroviral therapy (HAART) has been shown to be the most effective treatment for HIV-associated KS. In many patients, HAART can stop the growth of lesions or even clear them. It can also extend survival substantially. If lesions are few or in their early stages, they can be treated with cryotherapy or surgically excised. For more extensive disease,a variety of chemotherapeutic agents and radiation treatments may be therapeutic. Optimal treatment is to raise the patient's CD4 count, at which point the KS often resolves on its own.
Prior to our initial meeting, this patient's lesions had been treated with cryotherapy two times over two months. After each treatment, the lesions would clear. They eventually resolved completely.
Dr. Scheinfeld is assistant attending and assistant clinical professor at St. Luke's-Roosevelt Hospital, Department of Dermatology, in New York City.