kaposi sarcoma ( ks ) is characterized by multicentric neoplastic proliferation of vascular endothelial cells .
aids - related ks has a rapidly progressive course with early mucosal and systemic involvement .
the incidence of aids - associated ks , especially the disseminated form , is very rare in india .
furthermore , the importance lies in early diagnosis and treatment which significantly affect the outcome .
we are reporting the case for its rarity in india ( probably the 18 case ) and to highlight the importance of the early institution of highly active antiretroviral therapy ( haart ) .
a 34-year - old male presented with multiple asymptomatic pigmented raised lesions over the trunk , bilateral upper and lower limbs for last two months .
he had history of fever and weight loss ( > 10% total body weight ) for the past six months .
history of swelling of bilateral lower limbs , scrotum , and penis was present since one month .
on examination , the patient had pallor , generalized lymphadenopathy , bilateral pitting pedal edema , and hepatosplenomegaly .
cutaneous examination revealed multiple discrete to grouped violaceous macules , papules , nodules and plaques measuring 0.2 cm  0.2 cm to 0.5 cm  3 cm over the trunk , bilateral upper and lower limbs [ figure 1 ] .
cutaneous lesions over the lower abdomen and left thigh were coalescing to give a  cobblestone appearance  [ figure 1 ] .
oral cavity showed two well - defined violaceous patches over the hard palate [ figure 2 ] . on investigation
, he had low hemoglobin ( 6.9 g / dl ) , deranged renal function parameters ( urea - 69 mg / dl and creatinine - 1.9 mg / dl ) and massive bilateral pleural effusion on chest x - ray .
serology for hiv-1 was positive by enzyme - linked immunosorbent assay and negative for hiv-2 , and his cd4 count was 573/mm .
histopathology of skin biopsy specimen revealed proliferation of endothelial cells with formation of slit - like vascular spaces in the upper and middle dermis .
spindle - shaped endothelial cells were arranged in solid cords and fascicles admixed with variable lymphoplasmacytic infiltrate , hemorrhage , and lymphatic - like dilated spaces [ figures 3 and 4 ] .
promontory sign , characterized by protruding of new vessels into the lumen of existing large vessel , was also demonstrated [ figure 5 ] . on immunocytochemistry ,
a final diagnosis of aids - associated disseminated ks was made but unfortunately the patient died before starting haart because of recurrent massive pleural effusion .
multiple violaceous macules , papules , nodules , and plaques over the lower abdomen and bilateral thigh , some of which are coalescing to give
 cobblestone - like appearance  two well - circumscribed violaceous patches present over the hard palate cord - like proliferation of spindle - shaped endothelial cells with areas of hemorrhage and variable lymphoplasmacytic infiltration in the dermis ( h and e , 100 ) spindle - shaped endothelial cells arranged in fascicles in the dermis ( h and e , 400 )  promontory sign  - new blood vessels protruding into the lumen of existing blood vessel ( arrow ) ( h and e , 400 ) cd31 positive proliferating endothelial cells ( ihc , 100 )
human herpes virus-8 ( hhv-8 ) is considered as the putative causative agent of ks .
co - infection of hhv-8 with hiv promotes the oncogenic capabilities of hhv-8 , leading to the development of ks .
the low incidence of ks in india is thought to be because of the low prevalence of the hhv-8 infection , with a prevalence of 4.7% reported from south india .
cutaneous lesions of classic ks start over the extremities as violaceous patches which gradually progress to form plaques and nodules .
progression is slow in the classic form , and mucosal and systemic involvement is rarely seen .
the incidence of aids - associated ks is higher in homosexual males in comparison to females .
clinically , aids - related ks differs from the classical form because of its rapid clinical course and widespread dissemination that affect the mouth , lymph node , lungs , gastrointestinal tract and genitalia .
oral mucosal involvement can occur in all type of ks but more common in aids - associated ks .
common sites affected are hard palate and soft palate followed by gingiva , and dorsal tongue .
the presence of oral lesion not only points towards undiagnosed hiv infection but also its association with the disseminated form of the disease which have potential diagnostic and therapeutic implications . in 50% patients of ks , oral mucosa
is affected and in 20 - 25% , cases oral mucosa is the initial site of involvement . in indian patients , an involvement of both cutaneous and oral mucosa occurred in 61.53% cases while 38.46% had only cutaneous involvement .
our patient presented with two violaceous patches over hard palate which probably correlates with the rapidly progressive nature of the disease .
in contrast to most of the malignancies , lymph node involvement in ks is not associated with worse prognosis and is of no special clinical significance .
lung involvement in ks occurs in 20% of the patients and is the most life - threatening form of the disease .
patients commonly present with progressive dyspnea , nonproductive cough , fever , chest pain and pleural effusion .
our patient had recurrent massive pleural effusion probably due to involvement of the lungs leading to the demise of the patient . from a dermatologist 's point of view
, ks should be kept in mind while dealing with an hiv - seropositive case with violaceous raised lesions , as the prognosis depends on the timely initiation of haart therapy .
the closest mimics of ks are bacillary angiomatosis , pseudo - ks , lichen planus , malignant melanoma , pyogenic granuloma , and hemangioma .
immunohistochemical staining with hhv-8 clinches the diagnosis of ks , but in our case , we could not perform the test because of financial constraint .
aids - related ks responds well to haart therapy , and systemic chemotherapeutic drugs are indicated in nonresponsive patients or in patients with systemic involvement .
liposomal anthracyclines ( doxorubicin and daunorubicin ) are the united states food and drug administration approved first - line agent for treatment of ks . due to its side - effect profile ,
other systemic treatment modalities include interferon- and combination chemotherapies with adriamycin , bleomycin plus vincristine , or vinblastine . in conclusion ,
our case highlights the rapidly progressive nature of aids - associated disseminated ks and importance of the early institution of haart .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosismucosal lesion can give a clue to the undiagnosed hiv .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosis mucosal lesion can give a clue to the undiagnosed hiv .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosismucosal lesion can give a clue to the undiagnosed hiv .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosis mucosal lesion can give a clue to the undiagnosed hiv .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosismucosal lesion can give a clue to the undiagnosed hiv .
early diagnosis and institution of antiretroviral therapy is crucial in aids - associated kaposi 's sarcoma ( ks ) to achieve favorable prognosis mucosal lesion can give a clue to the undiagnosed hiv .
 aids - associated disseminated kaposi sarcoma ( ks ) is a rare entity , especially in india due to the low prevalence of human herpes virus-8 infections in indian population . due to its rapid and progressive nature 
 , early diagnosis and institution of highly active antiretroviral therapy is crucial in aids - associated ks , with a view to achieving favorable prognosis . 
 we report a case of disseminated ks in an hiv-1 positive patient , who presented with two months history of multiple violaceous patches and plaques over the trunk , bilateral upper limbs , lower limbs , and hard palate . 
 the patient died of recurrent massive pleural effusion before starting antiretroviral therapy . 
 this case is being reported due to the paucity of ks in the indian literature , especially the disseminated type and to highlight its rapidly progressive course which can be fatal . 