03. Kaposi’s Sarcoma.ppt dermatological condition

Ks

1. Kaposi’s Sarcoma
Dr. O Ngalamika
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2. First described by Moritz Kaposi in 1872
Most common cutaneous malignancy in HIV
disease
Various visceral organs can also be affected
It has several subtypes which have different
presentations, epidemiology, and prognoses
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3. Etiopathogenesis
Formed by abnormal proliferation of vascular
endothelial cells
Infection with HHV-8 has been associated
with the development of KS

HHV-8 is found in KS lesional tissue
irrespective of clinical type
Transmission of HHV-8: mainly through
saliva. Organ transplantation, blood
transfusion
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4. HHV-8 is known to encode products that lead
to growth dysregulation or evasion of immune
surveillance
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5. Clinical Features
Characterized by brown, pink, red or
violaceous macules/patches,
papules/plaques, nodules
Lesions may vary depending on the clinical
variant
Mucous membrane, cutaneous and visceral
involvement is common (lymph nodes, GIT,
and lungs)
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8. KS Subtypes
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9. 1. Classic KS
Indolent disease
Seen chiefly in middle-aged men of southern
and eastern European origin
Early lesions appear most commonly on the
toes or soles as reddish, violaceous, or
bluish-black macules and patches that
spread and coalesce to form nodules or
plaques
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10. Brawny edema of the affected limb may be
present
Macules or nodules may appear, usually
much later, on the arms and hands, and
rarely may extend to the face, ears, trunk,
genitalia, or oral mucosa
Classic KS has a slowly progressive course
In the early stages of disease, lesions may
undergo spontaneous resolution
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11. 2. African cutaneous KS
Endemic in tropical Africa
Mostly seen in men between the ages of 20
and 50
It has a locally aggressive but systemically
indolent course

Characterized by nodular, infiltrating,
vascular masses on the extremities
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12. 3. African lymphadenopathic KS
An aggressive disease of young patients,
mainly children under age 10
Lymph node involvement with or without skin
lesions

Has an aggressive course, often terminating
fatally within 2 years of onset
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13. 4. AIDS-associated KS
KS in patients immunosuppressed by AIDS
Cutaneous lesions begin as one or several
red to purple-red macules, rapidly
progressing to papules, nodules, and plaques

There is a predilection for the head, neck,
trunk, and mucous membranes
A fulminant, progressive course with nodal
and systemic involvement is expected
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14. It may be the presenting manifestation of HIV
infection (HIV stage 4)
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15. 5. Immunosuppression-associated KS
Occurs in patients on immunosuppressive
therapy
Clinical features may be similar to that of
classic KS; however, site of presentation is
more variable
Removal of the immunosuppression may
result in regression of the KS
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18. Internal involvement
Classic KS: GIT is most frequently involved.
Lungs, heart, liver, conjunctiva, adrenal
glands, abdominal lymph nodes, and bones
may also be affected.
African cutaneous KS: frequently
accompanied by massive leg edema and
frequent bone involvement
African lymphadenopathapic KS :
Reported among Bantu children who develop
massive lymphnode involvement, preceding
appearance of skin lesions
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19. -The children also develop lesions on the
eyelids and conjunctiva
-Eye involvement is often associated with
swelling of the lacrimal, parotid, and
submandibular glands
AIDS-associated KS: Lungs (37%),
gastrointestinal tract (50%), and lymph nodes
(50%)
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20. Epidemiology
KS is worldwide in distribution

In africa, it occurs largely in the south of the sahara

Prevalence of AIDS-related KS has decreased due
to widespread availability of HAART
KS associated with other forms of
immunosuppression include those with iatrogenic
suppression from oral prednisone or other chronic
immunosuppressive therapies, as may be given to
transplant patients
It affects more men than women. Ratio ~ 2:1
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21. Differential diagnosis
Bacillary angiomatosis
Pyogenic granuloma
Leukemia cutis
Cellulitis
Severe stasis dermatitis
Small-vessel vasculitis
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22. Work-up
Baseline investigations
Skin biopsy
Immunohistochemistry
HIV test
HHV-8 PCR
CXR/CT-Scan
Endoscopy
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23. Histopathology
Prominent spindle cells
Prominent slitlike vascular spaces
Extravasated red blood cells
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24. Treatment
Topical retinoid
Surgical excision
Radiation
Laser
ART for epidemic KS
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25. Chemotherapy:
-liposomal doxorubicin, liposomal
daunorubicin
-Vincristine, Bleomycin, Doxorubicin: 1
st
line at
UTH/CDH
-Paclitaxel monotherapy: 2
nd
line agent
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26. Course
Classic KS: Progresses slowly, rare lymph node or
visceral involvement. Death occurs years later from
unrelated causes
African cutaneous KS: aggressive, early nodal
involvement, death from KS expected within 1-2
years
AIDS-related KS: although widespread, most
patients die of intercurrent infection
Immunosuppression related KS: removal of
immunosuppression may result in resolution of the
KS without therapy
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27. End
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