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AIDS - associated Kaposi's sarcoma in a heterosexual male

  • Author(s): Chandan, Kiran
  • Madnani, Nina
  • Desai, Devendra
  • Deshpande, Ramesh
  • et al.
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AIDS - associated Kaposi's sarcoma in a heterosexual male - A case report
Kiran Chandan1, Nina Madnani2, Devendra Desai1, Ramesh Deshpande3
Dermatology Online Journal 8(2): 19

From the Departments of 1. Gastroenterology, 2. Dermatology, and 3. Pathology, P.D. Hinduja National Hospital and MRC, Mumbai 400016.

Abstract

A 39 year old married heterosexual male presented with extensive purpuric macules, papules, and lichenoid plaques scattered over his skin and mucocutaneous surfaces for one year. The lesions were larger in both his groins, and were associated with lymphedema of his penis and scrotum. His serology for the HIV-1 virus was positive. Biopsies from multiple lesions confirmed the diagnosis of Kaposi's sarcoma. Histochemistry was positive for the HHV-8 virus. He subsequently developed pulmonary and peritoneal effusions and died 3 months later of cardiopulmonary arrest.



Introduction

Kaposi's sarcoma (KS) was first described in 1872 by Moritz Kaposi as a disease seen in elderly men of Mediterranean or Jewish descent. In 1981, initial reports described it in homosexual men with AIDS, but recent publications have also reported its incidence in heterosexual males.[1] Patients with AIDS-related KS usually present with cutaneous lesions, mucous membrane lesions, or lymph node involvement. Visceral involvement occurs in 50% of the patients, especially of the lungs and gastrointestinal tract. Lung involvement occurs in 20% of the patients and is the most life-threatening form of the disease.[2] Recent findings of a herpetic-like viral DNA in Kaposi's sarcoma tissue have suggested an infectious co-factor for the disease.[3] We present a case of KS in an HIV-1 positive heterosexual male with extensive cutaneous and systemic involvement.


Case Report

A 39 year old married male was admitted to the gastroenterology department with distension of the abdomen and pedal edema for the last 15 to 20 days. He had been married for 4 years and had 2 children, aged 2 and 3 years. He chewed tobacco, consumed 1 oz of whisky daily, and had multiple sexual exposures over the last 20 yrs. He denied any homosexual exposure, and did not smoke or use intravenous drugs. He gave a vague history of a penile ulceration for which he had been treated with some homeopathic medication. A year ago, he was tested and found to be HIV positive. At that time he developed a non-pruritic rash over the legs which had been unsuccessfully treated with topical steroids, and had recently spread to involve his whole body. He did not have any other systemic complaints.


Figure 1Figure 2
Fig. 1 Multiple purpuric macules, lichenoid papules and plaques, on both forearms.
Fig 2. Bilateral inguinal lymphadenopathy with edema of the scrotum and penis.

Clinical examination revealed mild pallor with enlarged axillary and inguinal lymph nodes. He had extensive cutaneous lesions over his trunk and extremities which appeared as purpuric macules, lichenoid papules and plaques, and hyperkeratotic violaceous nodules. His scrotum and penis were grossly edematous. His oral cavity had a large dusky plaque over the soft palate. Chest auscultation indicated bilateral reduced air entry. He had ascites but no organomegaly.

A skin biopsy showed a hyperplastic epidermis with abnormal vascular channels arising from venules which dissected irregularly into the surrounding dermal collagen of the superficial and mid-dermis. These vascular channels formed irregular spindle cell bundles. There was extravasation of RBCs forming linear streaks amidst the collagen bundles with a moderate superficial and deep perivascular lymphocytic infiltrate. A Grams stain and a Z-N stain were negative for organisms. A diagnosis of Kaposi's sarcoma was made. A formalin section further tested by the anti-LANA method was positive for HHV 8.


Figure 3
Fig3. Marked proliferation of abnormal vascular channels forming irregular spindle-cell bundles.

Lab investigations demonstrated a normochromic normocytic anemia with a Hb of 7.8gm%, WBC count of 11,000/cmm (N61 E02 L30), low albumin of 1.9g/dl (N 3.2 - 5.5 g/dl), raised total bilirubin of 1.5mg/dl and an alkaline phosphate of 219 U/L (N 40-120IU/L). Sputum was negative for fungus and any other infectious organism. His TPHA was positive and an HIV-1 (Eliza and western blot) was positive. His absolute CD4 count was 247 cmm ( 337.00-1513.00 )and CD8 3283 cmm (174.00 - 1240.00 ). Viral markers for hepatitis B and C were negative. A chest X ray showed bilateral pleural effusion. A pleural tap was hemorrhagic, without any malignant cells or bacilli. A pulmonary function test was suggestive of severe mixed restrictive and obstruction defect with no post-bronchodilator reversibility. A chest computed axial tomography revealed multiple enlarged non-necrotic, non-calcified, bilateral axillary, prevascular, aorto-pulmonary and sub-carinal lymph nodes. An abdominal ultrasonography demonstrated mild to moderate ascites with enlarged lymph nodes at the splenic hilum. The patient was advised to start Highly active anti-retroviral therapy (HAART) and chemotherapy which included doxrubicin, bleomycin and vincristine. Due to financial constraints, the patient refused treatment and was discharged. A week later he was readmitted with dyspnoea, and subsequently died of cardiorespiratory arrest.


Discussion

In the early 1980s, an epidemic form of KS was detected in homosexual men in urban areas of New York and California. This was recognized to be associated with a new retrovirus HIV-1. Upto 30% of those infected men developed KS.[4] In patients with other modes of HIV transmission, the prevalence of KS was less than 5%.[5] Epidemiological studies suggest that HIV did not spread to India until the year, 1985. By 1990, several studies in the Bombay(now Mumbai) area reported a high prevalence of HIV infection.[6,7]

KS is reported as the initial manifestation of the AIDS syndrome in approximately 30% of cases. Overall mortality is approximately 41% with over 60% of patients alive at 1 year and 50% at 22 months. Overall survival is 18 months with some exceptions.[8] The HIV- associated form usually gets disseminated to the skin, visceral organs and lymph nodes. The possibility that the Human Herpes Virus 8 (HHV 8) is the inciting factor in KS pathogenesis was considered because of the presence of virus in pathological lesions of KS.[9] Whether this KSHV/HHV 8 is an etiological agent or a cofactor in the development of this vascular neoplasm is uncertain and remains to be proven.[10] The KS - associated herpes virus (KSHV) is the 8th HHV to be identified.[3] This virus has also been identified in classic, transplant, endemic, and AIDS - associated KS. KSHV can be transmitted sexually and by other means, more often by homosexual than heterosexual activities. Receptive anal intercourse has been identified as a risk factor. Martin et al studied the antibodies to HHV 8 latency-associated nuclear antigen (anti-LANA) in HIV infected and uninfected males, in order to detect the seroprevalence of HHV-8 in the population.[11] Their results showed a 37.6% positivity in men who gave a history of homosexual exposure in the past 5 yrs and no positivity in exclusively heterosexual males. Also the 10 year probability of developing KS among the men infected by both HIV and HHV 8 was 49.6%.[11] Armenian et al in their multicentre AIDS Cohort Study (MACS) found a dose-response relationship between KS and the number of sexual partners with an odds ratio of 2 between the most and least sexually active subgroups, which increased to 4.18 in the presence of a history of 5 infections. This supported their hypothesis that an agent in addition to HIV-1 was involved in the etiology of KS in AIDS patients.[12] The study by Lifson et al did not support these findings.[13]

Pulmonary KS is an unusual pre-mortem diagnosis and its clinical presentation is indistinguishable from opportunistic pneumonia with respect to the symptoms, physical examination results, and laboratory findings. Garay studied 318 patients with AIDS-associated KS and found 19 to have pulmonary involvement; all 19 were homosexual males. [14]

The features useful in the histopathological diagnosis of KS are dissection of collagen, lymphatic vessel-like spaces, angiomatoid lesions and spindle cell proliferation.

This case has been reported to demonstrate a case of extensive AIDS-associated KS, which presented with unusual purpuric macules, lichenoid papules, and keratotic violaceous plaques and nodules, in an Indian heterosexual male. The initial differential diagnoses entertained were vasculitis, drug eruption, lichen planus, and prurigo nodularis. The histopathology showed spindle cell proliferation and lymphatic-vessel like changes extending throughout the superficial and deep dermis. His condition deteriorated rapidly, and he died 15 months after the onset of the KS, and 3 months after diagnosis. Very few cases of AIDS-associated KS have been reported from India. A medline search revealed only two.[6,7] Since the patient had never left India and was heterosexual, we hypothesize that he must have acquired the infection locally from the commercial sex workers in Mumbai who he had repeatedly visited, and who could be reservoirs of HHV-8. Further studies need to be carried out to study the seroprevalence of HHV-8 in the commercial sex workers of Mumbai with and without HIV positivity after informed consent and taking into consideration the ethical issues of the study.

We gratefully acknowledge the help of Naresh Kikkeri MD, Department of Pathology, Tata Memorial Hospital, Mumbai 400012 for performing the histochemistry for HHV - 8.

References

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12. Armenian HK, Hoover DR, Rubb S, Metz S, Kaslow R, Visscher B, Chmiel J, Kingsley L, Saah A. Composite risk score for Kaposi's sarcoma based on a case-control and longitudinal study in the Multicentre AIDS Cohort Study (MACS) population. Am J Epidemiol 1993 Aug 15;138(4):256-65.

13. Lifson AR, Darrow WW, Hessel NA, O'Malley PM, Barnhart JL, Jaffe HW, Rutherford GW. Kaposi's sarcoma in a cohort of homosexual and bisexual men. Epidemiology and analysis of cofactors. AIDS Office , Department of Public health, San Fransisco, CA94102.

14. Garay SM, Belenko M, Fazzinni E, Schinella R. Pulmonary manifestations of Kaposi's sarcoma. Chest 1987 Jan; 91(1):39-43.

15. Francis ND, Parkin JM, Weber J, Boylston AW. Kaposi's sarcoma in acquired immune deficiency syndrome (AIDS). J Clin Pathol 1986 May;39(5):469-74.

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