Background

Immunosuppression is believed to be an integral factor in the pathogenesis of KS.

Recent data has also revealed that all forms of KS are closely associated with human herpes virus-8 (HHV-8), the production of inflammatory cytokines and the deregulation of new blood vessel formation (angiogenesis). (₁,₂)

KS most commonly affects the skin and oral mucosa. The initial presentation is usually in the form of pink, purple or red macules or papules, usually asymptomatic predominantly on the face and trunk. Often found on the tip of the nose, arms, neck or in the mouth, most commonly on the hard palate .As these lesions grow, they may interfere with eating and speaking. Spread to lymph nodes, the GI tract, lungs or other visceral organs is common. About 15% of patients visceral KS occurs without any cutaneous or oral lesions.

Case

39 years old HIV positive female patient with previous history of smear positive TB in 2001 when she completed a full course of anti TB drugs. Now referred to our ARV clinic for initiation of HAART with a CD4 < 200 cells/mm³.

On this visit the patient complains of a “nodule” on the nose. On physical exam we found a firm, ulcerated, purple to brown-black nodule, on the left nose flare.

At this point and based on our clinical findings the diagnosis of Kaposi's sarcoma was made and the patient was classified as Stage IV of the WHO clinical stage classification for HIV/AIDS. Commenced HAART, Regimen 1a (18/12/04). CD4 count of 13cells/mm³, BMI -16, Weight 43.8 Kg., Hb 11.1g/dl.

CD4 curve:

• CD4 count – 13 cells/mm³ (0.9%) on 13/07/04.

• CD4 count – 79 cells/mm³ (4.5%) on 12/02/05.

• CD4 count – 105 cells/mm³ (7.3%) on 07/06/05.

• CD4 count – 138 cells/mm³ (7.68%) on 16/01/06.

• CD4 count – 163 cells/mm³ (7.98%) on 03/08/06.

• CD4 count – 223 cells/mm³ (8.14%) on 29/03/07.

CD4 curve

Figure 1

Viral load testing was not accessible for our clients at the time when this patient was enrolled in the ARV program.

Viral load: <25 after HAART on 02/06/05, six months after initiation of HAART. Viral load: remained at the same level in subsequent measurements, on 16/01/06 and 03/08/06.

In subsequent visits we observed remarkable changes on the site of the lesion especially from month seven after HAART initiation with continuous improvement as shown in the pictures.

These changes and regression of the Kaposi's sarcoma was achieved with no use of any other therapeutic modalities.

The patient also experienced a sustained improvement of the immune function shown by an increase of the CD4 cell count from79 cell/m³ before commencement of HAART to223 cells/mm³ at 27 months.

Photo taken the day of initiation on HAART

Figure 2

Figure 1

Photo taken 1month after HAART initiation

Figure 3

Figure 2

Photo taken 2months after HAART

Figure 4

Figure 3

Photo taken 7months after HAART

Figure 5

Figure 4

Photo taken 11months after HAART

Figure 6

Figure 5

Photo taken 14months after HAART

Figure 7

Figure 6

Photo taken 15months after HAART

Figure 8

Figure 7

Photos taken 20months after HAART

Figure 9

Figure 8

Figure 10

Figure 9

Photos taken 22months after HAART

Figure 11

Figure 10

Discussion

Kaposi's sarcoma was first described in 1872 by the Hungarian dermatologist Moritz Kaposi. It was a rare condition before the AIDS epidemic. (₂)

Four different forms of Kaposi's sarcoma have been described: classic, endemic, acquired and epidemic.

Epidemic Kaposi's sarcoma is the form associated with HIV infection. It tends to follow a more variable but potentially more aggressive course than other forms of Kaposi's sarcoma.(₂)

While it is commonly found on the skin, KS can occur anywhere in the body.

KS is rare among women possibly due to hormonal factors, but it is still more common in HIV positive than HIV negative women. (₃)

The diagnosis of KS could be made by biopsy but doctors experienced on HIV may be able to diagnose KS without performing a biopsy. (₂)

In our patient the presence of a firm, ulcerated, purple lesion on the nose corresponded with findings of KS and the diagnosis was clinically based.

Treatment approach depends on the location of the lesions, course and extent of KS, and HIV disease stage. (₁)

In the age of HAART, KS generally responds well to therapy. (₄)

KS often significantly improves and blood levels of HHV-8 drop dramatically when HAART is commenced. (₂)

This clinical improvement is thought to be due to the drug's restoration of immune function, rather than any direct anti- KS effects. (₅)

In our patient we could observe an improvement of the immune function and at the same time a clinical remission of the KS.

A study of 53 people diagnosed with KS at a mean CD4 cell count of 174 cells/mm³ showed that the magnitude of the CD4 cell count increase after commencing HAART predicted the likelihood that KS would disappear.

In total 72 % of those who started HAART after a KS diagnosis had a complete or partial remission of their KS within 48 weeks. (₆) Optimal HAART, with maximal viral suppression and prevention of other opportunistic infections (OIs), is an essential part of the treatment often slows progression of KS and may result in complete remission, even without other therapy HAART is associated with prolonged survival. (₁)

Our patient did not receive any specific therapy for the KS.

KS of the skin is not in itself a life threatening condition and there is no evidence that the treatment of one or two small skin lesions makes any difference to life expectancy. Deciding to leave KS untreated also avoids the toxic effects of the chemotherapy which may also be immunosuppressive. (₂)

There are a range of treatments available for KS. These include local or general chemotherapy and pathogens based treatments.

Specific treatment for KS when indicated and available may be use in addition to HAART. Survival time is much greater in individuals who present with KS as a first sign of AIDS.

Conclusions

• Recognition of characteristic signs of KS allows for early clinical diagnosis.

• The above will help to make a correct staging of the patient with subsequent enrolment on the ARV program.

• In rural settings where access to specific treatment for KS is difficult, HAART provide an effective alternative.

• Improvement or remission of KS give patients a very evident proof of HAART effectiveness and helps to restore hope.