Disseminated Kaposi's Sarcoma in an HIV-Positive Patient: A Rare Entity in an Indian Patient.

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.

What was known?

AIDS-associated Kaposi's sarcoma (KS) is rare in Indian patient

The prevalence of human herpes virus-8 infection is low in Indian population

AIDS-associated KS has more rapid and progressive clinical course.

Introduction

Kaposi sarcoma (KS) is characterized by multicentric neoplastic proliferation of vascular endothelial cells.[1] AIDS-related KS has a rapidly progressive course with early mucosal and systemic involvement.[1] 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.[2] We are reporting the case for its rarity in India (probably the 18th case) and to highlight the importance of the early institution of highly active antiretroviral therapy (HAART).

Case Report

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. He gave history of unprotected sexual exposures with multiple female partners. 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/mm3. 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, endothelial cells were positive for CD31 [Figure 6] and CD34. A final diagnosis of AIDS-associated disseminated KS was made but unfortunately the patient died before starting HAART because of recurrent massive pleural effusion.

Figure 1

Multiple violaceous macules, papules, nodules, and plaques over the lower abdomen and bilateral thigh, some of which are coalescing to give “cobblestone-like appearance”

Figure 2

Two well-circumscribed violaceous patches present over the hard palate

Figure 3

Cord-like proliferation of spindle-shaped endothelial cells with areas of hemorrhage and variable lymphoplasmacytic infiltration in the dermis (H and E, ×100)

Figure 4

Spindle-shaped endothelial cells arranged in fascicles in the dermis (H and E, ×400)

Figure 5

“Promontory sign” - new blood vessels protruding into the lumen of existing blood vessel (arrow) (H and E, ×400)

Figure 6

CD31 positive proliferating endothelial cells (IHC, ×100)

Discussion

KS is an angioproliferative neoplasm that affects the skin and other organs.[1] 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.[1] 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.[2] 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.[3] 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.[4] 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.[5] 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.[2] 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.[6] 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.[7] 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.[89] 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, paclitaxel is preferred as a second-line agent. Other systemic treatment modalities include interferon-α and combination chemotherapies with adriamycin, bleomycin plus vincristine, or vinblastine.[8] In conclusion, our case highlights the rapidly progressive nature of AIDS-associated disseminated KS and importance of the early institution of HAART.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

What is new?

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. positive cases and rapid progressive nature of KS.