Healthcare workers should maintain a high index of suspicion for KS, given how common it is in people with HIV. The earlier the condition is recognised and treated, the better the chances of a good outcome. But unfortunately, many patients only present with very advanced cases.
Again, KS can occur while CD4 cell counts are high, and before there any other symptomatic disease, but is more aggressive at lower CD4 cell counts, and may occur alongside other serious illness (that will need concurrent diagnosis and treatment for the best outcomes).
Early KS lesions may be very subtle, suggestive of a bruise or a pigmented callous (Wilson) usually starting in the head or neck area, upper torso or lower extremities (Sissolak and Mayaud). As they grow, they form flat patches, or firm, raised, round or oval nodules. If pressed, these lesions do not become pale (as a bruise would).
The lesions may occur anywhere in the body, notably the face, hands, anorectal or genital regions, mucous membranes (around the eye, inside the mouth), lymph nodes, lungs and gastrointestinal tract, but they are especially common on the limbs and feet. Often, there is swelling (oedema) appearing at the edges of lesions, at which point the lesions may start to become painful.
When many nodules are present, they may follow skin-fold lines. In some cases, the nodules can ulcerate, bleed and become infected with secondary infections. They can also coalesce and cover large areas (Sissolak and Mayaud).
Blocked fluid drainage in associated lymph nodes can cause serious swelling, especially in the feet, lower legs or genitals. Swelling may also occur around the eyes and face (von Paleske).
Lesions and swelling can be disfiguring and people with KS end up being stigmatised and socially excluded.
When KS occurs on the thighs and soles of the feet, the lesions may become large plaques that are often swollen and extremely painful, making walking difficult, limiting mobility and causing functional disability. With invasive deep tumours, there may even be bone involvement (Master). Secondary infections can form seeping, malodorous wounds, which also cause the patient much distress.
KS can also involve the membranes in and around the eye. KS of the conjunctiva appears as flat or nodular reddish lesions, surrounded by tortuous vessels, and may also involve the eyelids and other ocular structures (Kagu, Shuler, Kurumety).
KS in the oral cavity is particularly common, and the initial site of involvement at presentation in about 20% of the cases (Flaitz). KS usually affects the palate (roof of the mouth) the gingiva (gums) or base of the tongue. Initially lesions may go unnoticed, but as they progress, secondary infections with thrush, pain and bleeding are common. Oral KS can cause difficulties talking or eating, and could ultimately lead to malnutrition and wasting. It is also commonly associated with other visceral involvement in the lungs, gastrointestinal tract or other internal organs (Kagu, Ficarra).
Many people with KS have gastrointestinal involvement or lesions in the spleen, liver and other internal organs, but these rarely appear to cause symptoms and go undetected. However, sometimes KS causes a blockage in the intestines, resulting in nausea, vomiting, abdominal pain, weight loss and occasionally bleeding that may be mistaken for ulcerative colitis.
Patients with KS who present with difficulty breathing, progressive shortness of breath and cough may also have KS in the lungs (pulmonary KS). This is the most serious form of KS, and can rapidly lead to sometimes bloody pleural effusions (fluid in the thoracic cavity), pulmonary failure and death within months if untreated (Aboulafia, Gill).
Multicentric Castleman's Disease: KS, however, is sometimes closely associated with a condition called multicentric Castleman's Disease (MDC), which is a pseudo-lymphoma or a non-cancerous proliferation of B cells in the lymph nodes that is either caused or exacerbated by HHV8 (Soulier, Hengge). It causes lymphadenopathy, hypoalbuminuria and often proteinuria as well as “B symptoms” including high fevers, anaemia, weight loss, fatigue, weight loss, loss of appetite, and low white blood cell counts. It is consistently associated with a poor prognosis for the person with KS.