A 25-year-old man originally from Sudan immigrated to Omaha, Nebraska, after residing in Egypt for the past 4 years. Promptly after arriving in Omaha, he presented to the emergency department seeking care for multiple, painful nodules on his legs. Eight months before admission, the patient had been diagnosed as human immunodeficiency virus (HIV) positive during the immigration screening process, but had not begun antiretroviral therapy nor was he aware of his CD4 count. Four months previously, he had noticed the progressive growth of multiple lower-extremity nodules. These had become much more painful, with a keratotic, pedunculated appearance associated with lower extremity edema (Fig. 1). Inguinal lymph nodes were enlarged but soft, mobile, and nontender. A plain film of the right foot showed periarticular osteopenia and soft tissue swelling.
What is your diagnosis?
Differential diagnoses: Cutaneous tuberculosis or atypical mycobacterium, leprosy, Kaposi sarcoma, lymphoma, syphilis, cutaneous endemic fungal infection.
Diagnosis: Kaposi sarcoma, likely endemic and acquired immunodeficiency syndrome (AIDS) associated.
Punch biopsy revealed nodular dermal tumor with slitlike and dilated vascular structures and hemorrhage (Figs. 2A, B). The tumor was composed of plump spindle cells with rare mitotic figures (Fig. 2C). DNA detection assay from the skin biopsy was positive for human herpes virus 8, and an acid-fast smear was negative. The CD4 count was 31/mL, and HIV RNA quantification revealed 718,000 copies/mL. The patient was referred to the HIV clinic for initiation of antiretroviral therapy.
Four clinical variants of Kaposi sarcoma have been described: classic, endemic, immunosuppressive or transplant associated, and epidemic or AIDS associated. Endemic Kaposi sarcoma was first described in the 1950s in equatorial Africa and has been described as nodular, florid, exophytic, or lymphadenopathic.1,2 Although the endemic form was initially not associated with an underlying immune deficiency, the eruption of HIV/AIDS in Africa has resulted in a more virulent blend of endemic and epidemic Kaposi sarcoma.3 Kaposi sarcoma is now the most frequently occurring tumor in central Africa.1,3
Primary treatment for Kaposi sarcoma is aggressive antiretroviral therapy. Chemotherapy is recommended for disseminated disease or visceral involvement. First-line chemotherapy consists of either pegylated liposomal doxorubicin or liposomal daunorubicin.1 Liposomal anthracyclines have been shown to have similar or improved response rates and more tolerable adverse effects compared with traditional multidrug regimens.1,4,5 Localized treatment with radiation, intralesional injections (typically vinblastine), alitretinoin gel, laser surgery, or cryotherapy can be used for cosmetic or palliative benefit.1
The authors thank Dr. Christine Hans for the pathology photographs.
1. Antman K, Chang Y. Kaposi's sarcoma. N Engl J Med
2. Taylor JF, Templeton AC, Vogel CL, et al. Kaposi's sarcoma in Uganda: a clinico-pathological study. Int J Cancer
3. Mwanda OW, Fu P, Collea R, et al. Kaposi's sarcoma in patients with and without human immunodeficiency virus infection, in a tertiary referral centre in Kenya. Ann Trop Med Parasitol
4. Northfelt DW, Dezube BJ, Thommes JA, et al. Pegylated-liposomal doxorubicin versus doxorubicin, bleomycin, and vincristine in the treatment of AIDS-related Kaposi's sarcoma: results of a randomized phase III clinical trial. J Clin Oncol
5. Stewart S, Jablonowski H, Goebel FD, et al. Randomized comparative trial of pegylated liposomal doxorubicin versus bleomycin and vincristine in the treatment of AIDS-related Kaposi's sarcoma. International Pegylated Liposomal Doxorubicin Study Group. J Clin Oncol