Variable clinical presentations of Classic Kaposi Sarcoma in Turkish patients

March 2012

Ilknur Altunay, Asli Kucukunal,^* Gulsen Tukenmez Demirci, and Bilge Ates

Abstract

Key Words: HHV-8, HIV, Kaposi sarcoma, skin cancer

Background

Kaposi sarcoma (KS) is a vascular neoplasm with multicentric cutanenous and extracutaneous involvements, which was first described by Moriz Kaposi in 1872. Since then, different epidemiological clinical and histopathological variants of this neoplasm have been identified. Classic Kaposi sarcoma (CKS) is one of four main clinico-epidemiologiologic variants. characteristics of the disease.

Materials and Methods

Four Turkish inpatients with CKS were evaluated in the study. All medical history and clinical data were noted. A screening immunodeficiency workup were performed for all patients. HHV-8 immunofluorescence testing on the specimens and ELISA test for human immunodeficiency virus (HIV 1 and 2) were performed. Pulmonary X ray graphies and computurized tomography (CT) scan were applied. Stage of the tumor was determined, in each case, according to the classification system proposed by Brambilla et al in 2003.

Results

All patients are positive for HHV-8. They were all immunocompetent and negative for HIV1 and HIV2. The first patient was unusual for morphological presentation of several verrucoid lesions that was evaluated as verrucoid KS. He was considered stage IB CKS. The patient 2 was a young man and the course of KS seemed unexpectedly aggressive for CKS. His clinical appearence seemed us to be a patient with AIDSassociated KS. The patient was evaluated as stage IVB CKS. Our third patient had also prominent lymphedema associated with bluish discoloration on the toes and fingers, suggesting a diagnosis of peripheral vascular disorder. He was diagnosed as stage IIIB CKS. The fourth case was interesting for very extensive lesions involving big sized plaques and also the existence of mucosal lesion. The patient was diagnosed as stage IVB CKS.

Conclusions

It seems that the reports of exceptional cases of KS are accumulating. Data from various cases should be collected and perhaps, novel clinical classifications should be considered. 

Full Text Article

NIH - Journal of Dermatological Case Reports

Eosinophilic panniculitis presenting with Kaposi's sarcoma-like plaques in a patient who is human immunodeficiency virus positive: a case report.

Nov 2012

Ustuner P, Dilek N, Saral Y, Dilek AR, Bedir R.

Abstract

INTRODUCTION: Eosinophilic panniculitis is an unusual type of panniculitis characterized by a prominent infiltration of subcutaneous fat with eosinophils without an exact etiopathogenesis. To the best of our knowledge, up to now eosinophilic panniculitis has been described in only one previous case with human immunodeficiency virus disease in the literature.

CASE PRESENTATION:

Here we report the case of a 44-year-old Caucasian man, who is human immunodeficiency virus positive, diagnosed with eosinophilic panniculitis. A dermatological examination revealed multiple, confluent Kaposi's sarcoma-like purple colored, deep plaques and nodules on his right gluteal area and right thigh. The presence of the mixed inflammatory infiltrate of lymphocytes, macrophages, and numerous eosinophils involving both septa and lobules of the subcutis were noted on the histopathological examination. On the basis of all these clinical and histopathological findings the patient was diagnosed with eosinophilic panniculitis. He was given intravenous 60mg/day methylprednisolone for 3 consecutive days a week for 6 months. The lesions resolved almost completely after 6 months.

CONCLUSION:

The predominance of T helper-2 subset of T helper cells and the consequential increase in interleukin-5 cytokines accompanying peripheral eosinophilia and high serum immunoglobulin E levels may all be blamed for the development of eosinophilic panniculitis in our case study. As a result, we aim to emphasize that eosinophilic panniculitis should be kept in mind in the differential diagnosis of subcutaneous nodular lesions in patients who are human immunodeficiency virus positive. We also focus on the requirement of histopathological examination for the definitive diagnosis because the clinical features of eosinophilic panniculitis may easily be confused with Kaposi's sarcoma.

NIH/BioMed

Clinical Manifestations of Kaposi Sarcoma Herpesvirus Lytic Activation: Multicentric CastlemanDisease (KSHV-MCD) KSHV Inflammatory Cytokine Syndrome

Clinical Manifestations of Kaposi Sarcoma Herpesvirus Lytic Activation: Multicentric CastlemanDisease (KSHV-MCD) and the KSHV Inflammatory Cytokine Syndrome.

2012

Polizzotto MN, Uldrick TS, Hu D, Yarchoan R.

Source

HIV/AIDS Malignancy Branch, Center for Cancer Research, National Cancer Institute Bethesda, MD, USA.

Abstract

Soon after the discovery of Kaposi sarcoma (KS)-associated herpesvirus (KSHV), it was appreciated that this virus was associated with most cases of multicentric Castleman disease (MCD) arising in patients infected with human immunodeficiency virus. It has subsequently been recognized that KSHV-MCD is a distinct entity from other forms of MCD. Like MCD that is unrelated to KSHV, the clinical presentation of KSHV-MCD is dominated by systemic inflammatory symptoms including fevers, cachexia, and laboratory abnormalities including cytopenias, hypoalbuminemia, hyponatremia, and elevated C-reactive protein. Pathologically KSHV-MCD is characterized by polyclonal, IgM-lambda restricted plasmacytoid cells in the intrafollicular areas of affected lymph nodes. A portion of these cells are infected with KSHV and a sizable subset of these cells express KSHV lytic genes including a viral homolog of interleukin-6 (vIL-6). Patients with KSHV-MCD generally have elevated KSHV viral loads in their peripheral blood. Production of vIL-6 and induction of human (h) IL-6 both contribute to symptoms, perhaps in combination with overproduction of IL-10 and other cytokines. Until recently, the prognosis of patients with KSHV-MCD was poor. Recent therapeutic advances targeting KSHV-infected B cells with the anti-CD20 monoclonal antibody rituximab and utilizing KSHV enzymes to target KSHV-infected cells have substantially improved patient outcomes. Recently another KSHV-associated condition, the KSHV inflammatory cytokine syndrome (KICS) has been described. Its clinical manifestations resemble those of KSHV-MCD but lymphadenopathy is not prominent and the pathologic nodal changes of KSHV-MCD are absent. Patients with KICS exhibit elevated KSHV viral loads and elevation of vIL-6, homolog of human interleukin-6 and IL-10 comparable to those seen in KSHV-MCD; the cellular origin of these is a matter of investigation. KICS may contribute to the inflammatory symptoms seen in some patients with severe KS or primary effusion lymphoma. Additional research is needed to better define the clinical spectrum of KICS and its relationship to KSHV-MCD. In additional, research is needed to better understand the pathogenesis and epidemiology of both KICS and KSHV-MCD, as well as the optimal therapy for both of these disorders.

Frontiers