The skin lesions may be nodular, papular or blotchy and the colour may be red, purple, brown or black. These characteristics may also be seen under or on mucous membranes. The most common sites of the lesions are the throat, nose and mouth and unless they are swollen or inflamed, they are usually painless.

The lesions may equally involve internal organs like lungs (bringing about dyspnoea), the gastrointestinal tract (leading to fatal bleeding) and the lymphatics bringing about lymphedema.

In some cases, there is superimposed bacterial infection. In transplant recipients, the tumor spreads. On rare occasions, lesions occurring in the respiratory tract or the oesophagus may bring about obstruction [7].

• Generalized Lymphadenopathy

  CONCLUSIONS In conclusion, posttransplant KS may present as general lymphadenopathy without mucocutaneous manifestations, thus mimicking posttransplant lymphoproliferative disorder, which is often the first clinical suspicion. [ncbi.nlm.nih.gov]

  lymphadenopathy – particularly cervical region Highly aggressive – death within 2 years Epidemic form Males >30 years with HIV More common in men having sex with men Aggressive tumor with systemic involvement Transplant form Tends to develop within months [arupconsult.com]

  lymphadenopathy Generalized swelling of lymph nodes Swollen lymph nodes affecting all regions of the body [ more ] 0008940 Hemangioma Strawberry mark 0001028 Immunodeficiency Decreased immune function 0002721 Lymphoproliferative disorder 0005523 Papule [rarediseases.info.nih.gov]

  MCD: MCD is frequently characterized by fever, hepatosplenomegaly, and generalized lymphadenopathy. Other manifestations include splenomegaly, pleural effusion, autoimmune hemolytic anemia, rash, jaundice, and neurological symptoms. [infectiousdiseaseadvisor.com]

  It occurs more frequently in HIV-infected patients that present constitutional symptoms, generalized lymphadenopathy, hepatosplenomegaly, polyclonal hypergammaglobulinemia, anemia, and elevated serum levels of IL-6.(5) The histopathologic examination [scielo.br]

• Lymphedema

  Lymphedema could promote the development of certain tumors by altering immunocompetence. [ncbi.nlm.nih.gov]

  Quality-of-Life measure (LYMQOL) for Kaposi Sarcoma [ Time Frame: 3 years ] The Lymphedema Quality-of-Life measure (LYMQOL) has separate tools for assessing arm lymphedema and leg lymphedema. [clinicaltrials.gov]

  KS is also intimately associated with lymphedema. Chronic lymphedema may even precede KS lesions. [diagnosticpathology.biomedcentral.com]

• Lymphadenopathy

  CONCLUSIONS In conclusion, posttransplant KS may present as general lymphadenopathy without mucocutaneous manifestations, thus mimicking posttransplant lymphoproliferative disorder, which is often the first clinical suspicion. [ncbi.nlm.nih.gov]

  (may be present in up to 50%) 5 lymphadenopathy is typically of high attenuation 6 On liver CT: ill-defined multifocal peripheral portal nodules with variable attenuation (commonest hepatic neoplasm in AIDS patients) 1,4 Nuclear medicine Scintigraphy [radiopaedia.org]

  Paraaortic, parailiac and bilateral inguinal lymphadenopathies were detected by computer tomography imagination. [revistanefrologia.com]

• Cough

  The patient in our case presented with progressive dyspnoea and cough. Chest radiograph reveals complete opacification of the hemithorax. Complete lung consolidation was not seen on chest CT. [ncbi.nlm.nih.gov]

  Case Presentation A 40 year-old HIV positive homosexual male not on HAART Therapy presents with a 1month history of intractable cough, hemoptysis and shortness of breath. [austinpublishinggroup.com]

  When it spreads to the lungs, it often causes coughing, shortness of breath, and wheezing. Once in the lungs, the disease often quickly gets worse. It can cause respiratory failure, which can be fatal. [health.harvard.edu]

  […] reticulonodular pulmonary infiltrates, solitary lung nodules, or pleural effusions Tumor follows lymphatic channels Detection of HHV8 DNA is highly specific and sensitive for diagnosis of pulmonary Kaposi sarcoma Case reports 42 year old HIV+ man with chronic coughing [pathologyoutlines.com]

• Pleural Effusion

  A 50-year-old man who had received kidney transplantation from a living unrelated donor presented with a massive left-side pleural effusion, ascites, and a skin lesion. The pleural effusion and ascites were bloody. [ncbi.nlm.nih.gov]

  Definition / general Usually associated with AIDS, HHV8 infection; also immunocompromise, organ transplant Diffuse reticulonodular pulmonary infiltrates, solitary lung nodules, or pleural effusions Tumor follows lymphatic channels Detection of HHV8 DNA [pathologyoutlines.com]

  Chest examination was consistent with bilateral pleural effusion. Other physical examinations were unremarkable. [academic.oup.com]

  Arrows point to pleural effusions on both lungs. Despite completing a course of antibiotics for pneumonia, his symptoms did not improve. A follow-up CT scan of the chest demonstrated that the bilateral pleural effusions had worsened ( Figure 2 ). [f1000research.com]

• Hemoptysis

  It's a 46-year-old Chinese man presented with sore throat, hemoptysis, fever, dyspnea and multiple lung nodules. The lung lesions grew over a 5-month period so as the symptoms worsened. [ncbi.nlm.nih.gov]

  Case Report A 26-year-old African American man with AIDS presented with complaints of pleuritic chest pain, exertional shortness of breath, and hemoptysis of 3-week duration. [hindawi.com]

  Pulmonary Kaposi sarcoma can present with shortness of breath, cough, hemoptysis, or chest pain; it may be asymptomatic, appearing only on chest radiograph. Bronchoscopy may be indicated. [accessmedicine.mhmedical.com]

  Case Presentation A 40 year-old HIV positive homosexual male not on HAART Therapy presents with a 1month history of intractable cough, hemoptysis and shortness of breath. [austinpublishinggroup.com]

  Dyspnea, cough, hemoptysis, stridor, nodular infiltrates, pleural effusion, low diffusion capacity, and absence of arterial desaturations are more likely to be due to pulmonary KS than to opportunistic infections. 7 However, among persons at high risk [contagionlive.com]

• Dyspnea

  We report a 52-year-old Caucasian man with HIV/AIDS and Kaposi sarcoma who presented with dyspnea and pleural effusion. He reported numerous tender subcutaneous nodules developing over the past few months on his chest, back and abdomen. [ncbi.nlm.nih.gov]

  A 40 year old HIV positive homosexual male presented with fever, intractable cough and dyspnea. He was noted with non blanching purple skin lesions all over his body. CD4 count was 63 cells/mm3and viral load of 50,000. [austinpublishinggroup.com]

  Dyspnea, cough, hemoptysis, stridor, nodular infiltrates, pleural effusion, low diffusion capacity, and absence of arterial desaturations are more likely to be due to pulmonary KS than to opportunistic infections. 7 However, among persons at high risk [contagionlive.com]

  Herein we report the case of a 77 year-old- male who presented with a 6-week history of progressive dyspnea on exertion accompanied by productive cough of yellow sputum and intermittent hemoptysis. [f1000research.com]

• Nausea

  Imatinib may cause symptoms such as nausea, vomiting, joint pain, and skin rash. Bevacizumab may cause weakness, high blood pressure, bleeding, abdominal pain, nausea, and vomiting. [nyulangone.org]

  Gastric Kaposi’s sarcoma and vomitingin a 21-year-old female The medical history of a 21-year-old woman with complaints of nausea and vomiting is presented. [poj.peeters-leuven.be]

  Side effects include fever, chills, loss of appetite, headaches, nausea, fatigue and body aches. Preventive measures Preventive measures include practicing safe sex to reduce the risk of acquiring HIV. [news-medical.net]

  Do you experience nausea, vomiting, or abdominal pain, or have blood in your stool? This could mean that the cancer is affecting your gastrointestinal tract. [health.harvard.edu]

• Vomiting

  Imatinib may cause symptoms such as nausea, vomiting, joint pain, and skin rash. Bevacizumab may cause weakness, high blood pressure, bleeding, abdominal pain, nausea, and vomiting. [nyulangone.org]

  Gastric Kaposi’s sarcoma and vomitingin a 21-year-old female The medical history of a 21-year-old woman with complaints of nausea and vomiting is presented. [poj.peeters-leuven.be]

  Chemotherapy drugs for Kaposi's sarcoma include: Doxorubicin ( Adriamycin, Doxil ) Paclitaxel ( Taxol ) Vinblastine ( Velban ) Chemotherapy can have side effects, including hair loss, vomiting, and fatigue. [webmd.com]

  Do you experience nausea, vomiting, or abdominal pain, or have blood in your stool? This could mean that the cancer is affecting your gastrointestinal tract. [health.harvard.edu]

• Abdominal Pain

  We report the case of a 45-year-old young woman with abdominal pain, episodic diarrhea and a mild weight loss. [ncbi.nlm.nih.gov]

  Do you experience nausea, vomiting, or abdominal pain, or have blood in your stool? This could mean that the cancer is affecting your gastrointestinal tract. [health.harvard.edu]

  pain.4 In some cases, it may lead to hemorrhage, abdominal pain, gastric outlet obstruction or intussusception.1 GI-KS is endoscopically evident by appearance which ranges from a red maculopapular lesion to a darker, nodular or polypoid lesion.5 In more [elsevier.es]

  pain Treatment Chemotherapy and radiation preferred over surgery Clinical images Images hosted on other servers: Kaposi sarcoma Cherry red polyps on endoscopy Gross description Reddish brown to purple macules or polyps, typically 0.5 - 1.5 cm Microscopic [pathologyoutlines.com]

  Lesions that develop in the stomach and intestines can cause abdominal pain and diarrhea. Sometimes KS lesions bleed. If the lesions are in the lung, it can cause you to cough up blood and lead to shortness of breath. [cancer.org]

• Melena

  Walter, MD Department of Internal Medicine 1, Ulm University Hospital Patient history and clinical findings A 38-year-old man in a clearly impaired general state of health presented for clarification of Hb-relevant gastrointestinal bleeding with melena [endoscopy-campus.com]

  […] and fluorine-18-fluorodeoxyglucose (F-FDG) positron emission tomography (PET)/CT findings in an adult patient with KH associated with lymphangiomatosis involving mesentery and ileum.A 22-year-old female complained of a 9-month history of intermittent melena [cancerindex.org]

  Lesions are often asymptomatic and clinically indolent, but signs and symptoms can include the following: Odynophagia, dysphagia Nausea, vomiting, abdominal pain Hematemesis, hematochezia, melena Bowel obstruction Pulmonary lesions may be an asymptomatic [emedicine.com]

• Dysphagia

  Full Text A 57-year-old African woman was admitted to our clinic with a 1-week history of dysphagia and hematemesis. Her medical history was relevant for recently diagnosed human immunodeficiency virus (HIV). [elsevier.es]

  A 57-year-old African woman was admitted to our clinic with a 1-week history of dysphagia and hematemesis. Her medical history was relevant for recently diagnosed human immunodeficiency virus (HIV). On admission she was hypotensive and tachycardic. [scielo.mec.pt]

  Lesions are often asymptomatic and clinically indolent, but signs and symptoms can include the following: Odynophagia, dysphagia Nausea, vomiting, abdominal pain Hematemesis, hematochezia, melena Bowel obstruction Pulmonary lesions may be an asymptomatic [emedicine.com]

• Chest Pain

  Slightly elevated purple, pink, brown, or red blotches or bumps anywhere on the skin or in the mouth and/or throat Lymphedema, which is swelling caused by a blockage of the lymphatic system Unexplained cough or chest pain Unexplained stomach or intestinal [cancer.net]

  Pulmonary Kaposi sarcoma can present with shortness of breath, cough, hemoptysis, or chest pain; it may be asymptomatic, appearing only on chest radiograph. Bronchoscopy may be indicated. [accessmedicine.mhmedical.com]

  This can cause: uncomfortable swelling in the arms or legs (lymphoedema) breathlessness, coughing up blood, and chest pain nausea, vomiting, stomach pain, and diarrhoea How quickly the symptoms progress depends on the type of Kaposi's sarcoma. [nhs.uk]

• Vascular Disease

  […] failure, cardiac arrhythmia requiring medication, clinically significant peripheral artery disease, grade II or greater peripheral vascular disease, myocardial infarction. [clinicaltrials.gov]

  Cardiovascular late side effects have been termed radiation-induced heart disease (RIHD) and radiation-induced vascular disease (RIVD).[27] Symptoms are dose dependent and include cardiomyopathy, myocardial fibrosis, valvular heart disease, coronary artery [en.wikipedia.org]

• Skin Lesion

  CONCLUSIONS: The CXCL12/CXCR4-CXCR7 trio is upregulated in KS and correlates with KS pathophysiological markers and the severity of skin lesions. [ncbi.nlm.nih.gov]

  Coping with skin lesions Coping with skin lesions can be difficult, especially if they are very visible. It may be possible to use skin camouflage to cover small, flat skin lesions and make them less noticeable. [macmillan.org.uk]

  It is one of the most visible signs of AIDS, because it usually shows up as spots on the skin (lesions) that look red or purple on white skin, and bluish, brownish or black on dark skin. Lesions often occur on the face, arms and legs. [aidsinfonet.org]

• Skin Plaque

  There are four recognized variants 1: classic (chronic): multiple distal lower extremity predominant purple skin plaques and rarely involve visceral organs endemic (lymphadenopathic): common in equatorial Africa iatrogenic (organ transplant-related) AIDS [radiopaedia.org]

  […] of the liver Abnormal liver Liver abnormality [ more ] 0001392 Diarrhea Watery stool 0002014 Fatigue Tired Tiredness [ more ] 0012378 Fever 0001945 Lymphedema Swelling caused by excess lymph fluid under skin 0001004 Skin plaque 0200035 Skin rash 0000988 [rarediseases.info.nih.gov]

  A 26-year-old HIV-positive male student from North India was referred to us for the evaluation of widespread violaceous plaques and nodules on the skin. [ijdvl.com]

• Skin Papule

  Multiple small papules. Some are skin-colored; the larger papules are dark ("hydrocystome noire"). [doi.org]

• Eczema

  Because Paget’s disease often originates from breast duct cancer, the eczema-like cancer usually appears around the nipple. Paget’s disease also is considered a rare form of breast cancer. [massey.vcu.edu]

  Eczemas were the leading disease group (43.7%), with unclassified eczemas (33.9%) predominating. Papulosquamous disorders (15.4%) were second with psoriasis (50%) being the leading disease. [cancerindex.org]

• Skin Discoloration

  The use of intralesional interferon (IFN)-alfa (3-5 MU three times per week for 3-4 weeks) achieves similar results.(21) As with other local therapies, skin discoloration is the rule posttherapy. [hivinsite.ucsf.edu]

Even though KS can be detected from the appearance of lesions and the risk factor of the patient, definitive diagnosis can only be made by biopsy and microscopic examination. When KSHV protein LANA is detected in the tumor cells, the diagnosis is confirmed [8].

Again patients with HIV infection will need to undergo CD4 lymphocyte counts and plasma HIV viral-load studies.

• Chest X-Ray Abnormal

  However, the isolation of this agent is difficult to obtain, which justifies the sub-notification of the entity and the empirical treatment of patients with cough and chest X ray abnormalities.16 Data on viral load and CD4+ from the patients with the [scielo.br]

• Liver Biopsy

  We describe a case of rapid obstructive jaundice after initiation of antiretroviral therapy, in which the liver biopsy confirmed hepatic Kaposi sarcoma, and the clinical course was altered by the addition of montelukast. [ncbi.nlm.nih.gov]

• Pleural Effusion

  A 50-year-old man who had received kidney transplantation from a living unrelated donor presented with a massive left-side pleural effusion, ascites, and a skin lesion. The pleural effusion and ascites were bloody. [ncbi.nlm.nih.gov]

  Definition / general Usually associated with AIDS, HHV8 infection; also immunocompromise, organ transplant Diffuse reticulonodular pulmonary infiltrates, solitary lung nodules, or pleural effusions Tumor follows lymphatic channels Detection of HHV8 DNA [pathologyoutlines.com]

  Chest examination was consistent with bilateral pleural effusion. Other physical examinations were unremarkable. [academic.oup.com]

  Arrows point to pleural effusions on both lungs. Despite completing a course of antibiotics for pneumonia, his symptoms did not improve. A follow-up CT scan of the chest demonstrated that the bilateral pleural effusions had worsened ( Figure 2 ). [f1000research.com]

Kaposi Sarcoma is generally incurable and can only be controlled using palliative treatments. All underlying causes will however be treated where possible such as immunosuppression and immunodeficiency. For AIDS related KS or epidemic KS, beginning HAART will often lead to a reduction of lesions in more than 40% of cases [9]. However, the KS will continue to grow in some instances even with the use of antiretrovirals.

When there are only a few lesions, the treatments that can be considered include: Radiotherapy, cryothrapy or cryosurgery.

Surgery has the risk of KS occurring on the edges of the wound and is often only appropriate for small lesions on the surface. To prevent KS occurring on edges, electrodessication with curettage can equally be used.

For a more widespread KS or organ involvement, systemic therapy is required. Examples of such therapy include interferon alfa, liposomal anthracyclines and paclitaxel.

Prognosis is dependent on the variation of KS, how deeply the immune system is affected, presence of dissemination and whether or not the KS is recurrent [6]. Since the AIDS death rate declined in the 1990s however, there has been a reduction in the severity of KS conditions and the general occurrence.

As stated above, the KS disease is caused by infection with a virus known as the Kaposi sarcoma herpes virus, KSHV or the human herpesvirus 8 (HHV8). The KSHV virus is similar to the Epstein-Barr virus known to cause mononucleosis and other varieties of cancer.

With KS, the endothelial cells become infected with KSHV. The presence of the virus brings in genes that cause excessive cell divisions and make the cells live longer than normal. A combination of these changes, end up turning them into cancerous cells.

Infection with the KSHV virus is more common than the KS disease condition and many people who get infected with this virus do no go on to develop KS. In fact a greater percentage of people with KSHV do not show any symptoms [2]. For KS to occur KSHV must be present but in many cases the virus on its own does not bring about KS. People who develop KS have a weak immune system that may be caused by HIV infection, organ transplant, older age etc.

Kaposi Sarcoma was rare in the United States before the AIDS epidemic. In recent times, the figures have gone back to pre-epidemic figures.

In Africa however, the incidence of KS is quite high with 37.7 cases per 100,000 men and 20.5 cases per 100,000 women. The highest incidence of Kaposi sarcoma in Europe is seen in Sicily with 30 cases per million for men and 5.4 cases per million in women [3].

Regardless of the name, KS is not truly considered to be a sarcoma as sarcomas often arise from a mesenchymal tissue. On the other hand, KS is a cancer of the lymphatic endothelium forming vascular channels that are filled with blood cells. The filled vascular channels are what give the resultant KS tumor its bruise like appearance. In KS cancer cells, KSHV proteins are uniformly detected [4].

KS lesions have tumor cells with a characteristically abnormal elongated shape known as spindle cells. The most typical feature of Kaposi sarcoma is the presence of spindle cells that form slits containing red blood cells. Pleomorphism is often absent and Mitotic activity is only moderate. The tumor is very vascular and contains irregular blood vessels that are abnormally dense. This is what gives the tumor its dark colour. Inflammation around this tumor may produce swelling and pain.

HHV8 is found in all lesions of KS regardless of whether it is HIV related, classic, endemic or iatrogenic [5].

There are still doubts till date as to how Kaposi sarcoma herpes virus is acquired but it is commonly believed to be transmitted through the saliva of an infected person. Therefore the first step is to avoid body fluids from individuals diagnosed with the KSHV. However, keep in mind that the KS condition will only develop if there are other enabling factors [10].

Kaposi sarcoma (KS) is a cancer of the connective tissue caused by the human herpes virus 8. This virus is now known as the Kaposi sarcoma associated virus (KSHV) [1]. The condition is basically a malignant lesion that is characterised by neoplastic cells and abnormally growing blood vessels. Named by the Hungarian dermatologist Moritz Kaposi who discovered it in 1872, its viral gene sequence was only determined recently in 1995-1996. The lesions from KS may begin in more than one place at the same time making it different to other neoplasms.

The different types of KS include:

• Classic KS
• Endemic or African KS
• Transplant-related or acquired KS
• Epidemic KS
• Non-epidemic KS
• Recurrent KS

Kaposi sarcoma (KS) is a cancer of the skin that can also affect other parts of the body. The condition is caused by a virus and it is mostly seen in individuals that have an immune system that is not as strong as should be. The condition is relatively common with individuals living with AIDS and is seen around the world.

On its own, KS isn't life threatening but a lot depends on how deeply the immune system has been affected. There is no conclusive treatment for it but when there are a few lesions, radiotherapy may be used. One important part of management of this condition is regular support by health professionals. To prevent Kaposi sarcoma, patients with a disease that affects the immune system should stay away from body fluids from patients of the KS condition.

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