HIV-Related Complications: AIDS-Related Kaposi Sarcoma

Progressive cough and dyspnea of 2 months' duration prompted a 23-year-old man to seek medical attention for the fourth time. On previous emergency department visits, he had received antibiotics, which failed to relieve his symptoms. The patient reported fatigue, cough that produced whitish sputum, dyspnea on exertion and at rest, orthopnea, palpitations, and night sweats. He also had a purple patch on the tip of his nose, which had appeared about the same time as the cough. The patch had enlarged, and maculopapular satellite lesions had developed on his chin and cheeks (A).

The patient--a Mexican immigrant and machine factory worker--had no significant medical conditions or allergies. He denied recent travel, weight change, and use of alcohol or illicit drugs. He smoked about a pack of cigarettes per week and admitted to having unprotected sex with many women whose history he did not know.

Temperature was 38.2°C (100.7°F); heart rate, 125 beats per minute; blood pressure, 125/61 mm Hg; respiration rate, 48 breaths per minute; and oxygen saturation, 75% on room air (99% on nonrebreather mask). The patient had thrush. Further examination of the oral cavity revealed an infection of the upper right second molar.

Coarse crackles were heard throughout the upper and lower lung fields bilaterally, and breath sounds were decreased. The abdomen was soft and nontender without hepatosplenomegaly. Compared with previous imaging studies, lateral (B) and posteroanterior (C) chest radiographs showed worsening pulmonary edema and bibasilar pulmonary opacities with air bronchograms. A CT scan confirmed the presence of severe bilateral pneumonia.

Laboratory values included a white blood cell count of 6700/µL, with 83% neutrophils; hemoglobin level, 12 g/dL; hematocrit, 37.2%; and platelet count, 393,000/µL. The basic metabolic profile was normal.

A punch biopsy of a satellite lesion revealed an atypical vascular proliferation consistent with Kaposi sarcoma (KS). Results of immunostaining for human herpesvirus 8 (HHV-8) were positive.

KS is associated with HHV-8 infection and is the most common tumor in HIV-infected persons. This patient had epidemic, or AIDS-related, KS, which is considered an AIDS-defining illness by the CDC. The initial presentation can vary from an incidental skin lesion to extensive GI and respiratory metastases. Skin lesions appear most often on the lower extremities, face (especially the nose), oral mucosa, and genitalia. The lesions range from a few millimeters to several centimeters; they may have an elliptical shape and may be symmetrically or haphazardly distributed. The degree of vascularity determines whether their color is pink, red, purple, or brown. They are often papular and can be accompanied by significant lymphedema. Because the lesions are usually asymptomatic, many patients ignore them.

The use of HAART is associated with a reduction in the size of existing lesions. Intralesional chemotherapy, radiation therapy, laser therapy, and cryotherapy may also reduce the size and spread of lesions. Systemic chemotherapy is reserved for patients with widespread skin involvement (more than 25 lesions) or cutaneous lesions that are unresponsive to local treatment, and for symptomatic patients with visceral organ involvement.

This patient was given oxygen therapy, high-dose intravenous trimethoprim-sulfamethoxazole, and systemic corticosteroids for pneumonia. Within 2 weeks, oxygen therapy was discontinued. An HIV test showed an absolute CD4⁺ cell count of 3/µL. The patient was informed of his HIV status, its implications, and the need for vigilant adherence to HAART. His illness will be monitored on an outpatient basis.

(Case and photographs courtesy of Aundie Bishop and Bruce Latham, MD.)