Kaposi syndrome
Kaposi's disease (or syndrome) includes a multifocal malignant neoplastic form, not very frequent, which mainly involves the skin, viscera and mucous membranes.
Kaposi's bite has vascular origin and presents with typical eruptions (papules and / or nodules) or reddish-purple macules, often with hemorrhagic character.
The disease takes its name from the dermatologist who, at the end of the 19th century, described it for the first time: Móric Kohn Kaposi.
Incidence
Until the 1980s it was believed that Kaposi's disease was a very rare neoplasm that mainly affected the elderly of African ethnicity and those undergoing organ transplantation.
Again, Kaposi's disease epidemic tends to cause damage in adults aged 30-40, especially males (ratio 3 males: 1 female); although it is believed that the syndrome occurs especially among males, it seems that in females the prognosis is more often poor.
When considering that shape endemic of Kaposi's disease constitutes 10% of cancer cases in African countries, the disease certainly cannot be defined as rare.
In conclusion, the statistics show that Africans and homosexual men with AIDS are the two categories most predisposed to Kaposi's disease.
Where and how it manifests itself
For further information: Symptoms of Kaposi's disease
The syndrome, ideally, could manifest itself in any part of the body, as it could affect the skin, the anal and oral-nasal mucous membranes, and the viscera (especially the stomach, intestines, spleen, kidney, genital and pulmonary systems). However, the less severe form appears to be limited to the skin. Generally, cutaneous Kaposi's disease begins in the lower limbs, but progressively it could cover the entire body surface: in similar situations, the skin is covered with macules that fade from blue, to purple and red, often associated with true and own skin rashes such as papules, plaques and nodules, often asymptomatic.The raised lesions have precise, regular outlines; they appear angiomatous and distributed on a geographical map. With the evolution of the disease, the lesions expand, in terms of number and size; they tend to thicken and can even erode and deform the fingers or toes, progressively involving also tendons and muscles.
Kaposi's syndrome can generate edema, up to creating a real elephantiasis of the limb.
The most severe forms of Kaposi's disease mainly affect the viscera of the gastrointestinal tract, in addition to the lymph nodes: this severe form generally affects immuno-depressed or AIDS patients. It is not uncommon for people with Kaposi's disease to also have Burkitt's lymphoma.
The disease has a chronic course and, when left untreated, is often fatal.
Causes
Most likely, the causative factor responsible for the pathogenesis of the disease is a virus known as HHV-8 (Herpes virus type 8) or KSHV (Anglo-Saxon acronym of Kaposi Sarcoma-associated Herpes Virus); although the direct correlation with this virus has not been fully demonstrated, the data speak for themselves: in 95% of patients with Kaposi's disease the presence of the Herpes virus type 8 was found, therefore considered a very important cofactor not only in the pathogenesis of the disease, but also and above all in the conservation and evolution of the syndrome itself.
In all likelihood, Kaposi's disease is caused by abnormal replication of endothelial cells, called endothelial cells fusate; it is a multifocal disease that could metastasize, therefore it is absolutely oncological competence.
Some risk factors for Kaposi's disease have been identified: chronic immune depression, release of angio-proliferative substances, viral infections.
Classification
The complexity of Kaposi's syndrome is also given by the "heterogeneity of the forms in which it occurs. The disease is distinguished in:
- Epidemic Kaposi's disease (related to AIDS): the disease is systemic and multifocal, typical of the neck and head areas. Viscera can also be involved.
- Iatrogenic Kaposi's disease: The drugs responsible are immunosuppressants, which weaken the immune system. Typical of patients who have been transplanted an organ, forced to take this type of pharmacological substance to prevent an immune attack against the new organ [taken from www.aimac.it/]
- African Kaposi disease (endemic): this is the lymphadenopathic form, typical of Africa. The disease tends to preferentially affect young Africans, particularly in the cervical lymph nodes or located in the groin and in the pulmonary hilum. Unfortunately, the prognosis is often poor.
- Relapsing Kaposi's disease: the syndrome recurs even after the first treatment; the disease does not necessarily involve the same areas as the primary neoplasm.
Care
For further information: Drugs for the treatment of Kaposi's sarcoma
Therapy is essential not only for the recovery from the tumor, but also and above all for the survival of the patient: it must be pointed out that Kaposi's disease, when not treated or treated too late, is lethal.
The patient will have to undergo a medical examination: the specialist, after a careful diagnostic examination, generally refers the affected patient to an oncologist. There are four different therapeutic treatments:
- chemotherapy: the patient is given pharmacological specialties aimed at eliminating the diseased cells. Often times, unfortunately, this treatment causes serious repercussions to the patient.
- surgical excision of the tumor, in which the abnormal mass is removed.
- immunotherapy (biological therapy): uses the same immune system to defeat Kaposi's disease: the goal is to exploit substances synthesized by the body, or to administer similar synthetic substances from the outside, in order to restore the immune system, stimulating the defense.
- radiotherapy: uses X-rays, useful for the destruction of malignant neoplastic cells of kaposi's disease.
It is the duty of the oncologist to direct the patient affected by Kaposi's disease towards the most appropriate therapeutic option, based on the severity of the condition, age and state of health.
Summary
Kaposi's disease: in brief
Illness
Kaposi's disease
Description
Not very frequent multifocal malignant neoplastic form, mainly involving the skin, viscera and mucous membranes
Incidence of kaposi disease
Disease until the 1980s:
- very rare neoplasm
- target: elderly people of African ethnicity and organ transplant recipients
After the Eighties: the boom of the disease
- Kaposi's disease occurs in 34% of AIDS patients
- Endemic Kaposi's disease: constitutes 10% of cancer cases in African countries → the disease cannot be defined as rare
- Epidemic Kaposi's disease: mainly affects adults aged 30-40, mainly males.
Currently: Africans and homosexual men with AIDS are the two categories most at risk
Clinical evidence of Kaposi's disease
The disease could ideally manifest itself in any part of the body: skin, mucous membranes (anal and oro-nasal), viscera (especially stomach, intestine, spleen, kidney, genital and pulmonary system)
- Less severe form: limited to skin with blue-purple macules associated with skin rashes;
- Evolution of the disease: the lesions expand, in terms of number and size; they tend to thicken, can erode and deform the fingers or toes, spread to the tendons and muscles, generate edema and even elephantiasis;
- More severe forms: Kaposi's disease affects the viscera of the gastrointestinal tract, as well as the lymph nodes.
Course of the disease
Chronic course
When left untreated: it is often fatal.
Etiological picture
- HHV-8 (Herpes virus type 8)
- Abnormal replication of endothelial cells
- Risk factors for Kaposi's disease: chronic immune depression, release of angio-proliferative substances, viral infections
Classification of Kaposi's disease
- Epidemic Kaposi's disease (related to AIDS)
- Iatrogenic Kaposi's disease: the drugs responsible are immunosuppressants
- African Kaposi disease (endemic): lymphadenopathic form
- Relapsing Kaposi's disease
Possible treatments for Kaposi's disease
- Chemotherapy
- Surgical excision
- Immunotherapy (biological therapy)
- Radiotherapy
