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Kaposi’s Sarcoma
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Kaposi’s sarcoma (KS) is a vascular neoplasm of the skin which can also involve any organ. There are several subtypes of KS and have been found to be associated with a virus known as Human Herpesvirus-8 (HHV-8). While everyone with KS also has HHV-8, not everyone with HHV-8 has or develops KS.

Classic KS - This is seen most often in middle aged to elderly men of Southern Mediterranean or Eastern European heritage. It is seen predominantly on the legs but may also occur in the intestinal viscera. Early lesions appear like reddish, bluish or violaceous bumps on the feet and or toes. If left untreated these lesions may join together to form nodules or plaques and at this stage may cause swelling of the affected limb. This can be progressive with gradual spread of the lesions to other parts of the body.

African Cutaneous KS - This form occurs is endemic to tropical Africa and occurs primarily in men between 20 and 50 years of age. Patients develop vascular masses that are nodular and infiltrating. While it can have aggressive skin involvement the internal involvement is usually mild.

African Lymphadenopathic KS - This form may or may not have skin involvement but instead attacks the lymph nodes and lymph system. It generally occurs in children of tropical Africa under the age of 10 years. It has an aggressive nature and its victims usually die within 2 years.

AIDS-Associated Epidemic KS - In those with advanced HIV disease this can develop as red to purple macules that can rapidly spread to papules, nodules and plaques. It usually appears on the head and neck area first and can spread rapidly if the underlying Immunosuppression is not addressed. While this form has been common in HIV infected individuals, with the onset of aggressive HIV treatment it has shown a reduction in its prevalence. It is almost exclusively seen in homosexual or bisexual men.

Nonepidemic Gay related KS - There is a type of KS that develops in homosexual men who have no signs or symptoms of HIV infection. This type of Kaposi's sarcoma progresses slowly, with new lesions appearing every few years. The lesions are most common on the arms, legs, and genitals, but can develop anywhere on the skin.

Immunosuppression or Iatrogenic-Associated KS - This type is related to immunosuppression created artificially by medications such as those taken to prevent transplanted organ rejection. This type resembles the Classic KS form but its presentation can be much more variable.

When the viscera are involved it is most common in the intestines with the small intestine being the most frequently affected. However other organs can also be affected such as the lungs, heart, liver, lining of the mouth, conjunctiva, adrenal glands and of course the lymph vessels and nodes. In advanced disease there is also bone involvement. Kaposi’s sarcoma is also associated with an increase risk of other malignancies such as lymphomas, leukemias, and myeloma. The risk of lymphoreticular malignancies is about 20 times greater in the KS population as compared to those with out the disease.

Treatment is based to the general type of KS diagnosed. Therapies for localized lesions include cryotherapy (freezing), radiotherapy (radiation), laser surgery (burning), electrosurgery (burning) and even excisional surgery (cutting the lesion off). For generalized disease chemotherapy and biologic therapy is used.

 

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The medical information provided in this site is for educational purposes only and is the property of the American Osteopathic College of Dermatology. It is not intended nor implied to be a substitute for professional medical advice and shall not create a physician - patient relationship. If you have a specific question or concern about a skin lesion or disease, please consult a dermatologist. Any use, re-creation, dissemination, forwarding or copying of this information is strictly prohibited unless expressed written permission is given by the American Osteopathic College of Dermatology. 

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