Enfermedad de Castleman. Reporte de caso / Castleman Disease. Case report and literature review

Resumen INTRODUCCIÓN: La enfermedad de Castleman, o hiperplasia angiofolicular de los ganglios linfáticos, es todo un reto diagnóstico y terapéutico para la mayoría de los médicos. Puede estar asociada con infecciones virales, como el herpes virus tipo 8 (HHV-8), o ser idiopática. A su vez, puede localizarse en una sola región (unicéntrica) o afectar varias (multicéntrica). Suele diagnosticarse en la cuarta década de la vida y ser un hallazgo cuando se trata de la variante unicéntrica. CASO CLÍNICO: Paciente de 19 años que acudió a consulta debido a la aparición de un nódulo en la mama derecha. En el ultrasonido mamario y axilar se encontraron fibroadenomas bilaterales y adenomegalias en el lado izquierdo, con alta vascularidad. Se catalogó como BIRADS 3. El reporte histopatológico de la biopsia, con aguja de corte, del ganglio axilar izquierdo fue de: proliferación linfoide atípica. La inmunohistoquímica reportó positividad para: CD20, CD3, CD21 en células dendríticas interfoliculares, Ki-67 y negatividad para HHV-8 en centros germinales residuales. CONCLUSIÓN: La extirpación quirúrgica de una masa unicéntrica de tipo hialino-vascular-plasmático es curativa. La evaluación de pacientes con sospecha de esta enfermedad debe incluir, además de la evaluación patológica con inmunotinción, estudios de laboratorio y de imágenes sistémicas con PET-TAC para determinar la extensión de la enfermedad (unicéntrica o multicéntrica) y para los marcadores de seguimiento.

Abstract BACKGROUND: Castleman's disease, or angiofollicular lymph node hyperplasia, is a diagnostic and therapeutic challenge for most physicians. It may be associated with viral infections, such as herpes virus type 8, or be idiopathic. In turn, it can be localized in a single region (unicentric) or affect several (multicentric). It is usually diagnosed in the fourth decade of life and is a finding when it is the unicentric variant. CLINICAL CASE: 19 year old patient who came to consult due to the appearance of a nodule in the right breast. Breast and axillary ultrasound showed bilateral fibroadenomas and adenomegaly on the left side, with high vascularity. It was classified as BIRADS 3. The histopathological report of the biopsy, with cutting needle, of the left axillary node was: atypical lymphoid proliferation. Immunohistochemistry reported positivity for: CD20, CD3, CD21 on interfollicular dendritic cells, Ki-67 and negativity for HHV-8 in residual germinal centers. CONCLUSION: Surgical removal of a unicentric hyaline-vascular-plasmic type mass is curative. Evaluation of patients with suspected disease should include, in addition to pathologic evaluation with immunostaining, laboratory and systemic imaging studies with PET-CT to determine the extent of disease (unicentric or multicentric) and for follow-up markers.

Case for diagnosis. Penile lesion in HIV-negative patient

Abstract We present the case of an HIV-negative man with asymptomatic penile erythematoviolaceous papules associated with similar slightly verrucous papules in the interdigital space of the right foot. A biopsy of the penile lesion confirmed Kaposi's sarcoma. No other causes of immunosuppression were observed. Penile lesions of KS are rare in HIV-negative individuals but it should also be considered in the differential diagnosis. Careful follow-up is recommended.

Two cases of rare HHV8-driven intravascular lymphoma with synchronous Kaposi sarcoma, both diagnosed at autopsy in renal transplant recipients

We present the first report of two rare yet remarkably similar autopsy cases of Kaposi sarcoma (KS) and intravascular human herpesvirus 8 (HHV8) positive lymphoproliferative disorder in renal transplant patients. It is well established that HHV8 infection causes Kaposi sarcoma (KS). More recently, it is recognized that HHV8 is also related to several lymphoproliferative conditions. These are poorly characterized and often difficult to diagnose. In both cases described herein, the diagnoses of multifocal hepatic KS and intravascular HHV8 positive (EBV negative) systemic diffuse large B-cell lymphoma, NOS were made at autopsy. Given the findings we describe in cases with fatal outcomes, we discuss the implications of HHV8 screening in solid allograft recipients.

Kaposi sarcoma of th e penis in anHIV-negative patient / Sarcoma de Kaposi de pênis em paciente HIV negativo

ABSTRACT Kaposi sarcoma is an angioproliferative disorder that ranges from a single indolent skin lesion to respiratory and gastrointestinal/visceral involvement. Kaposi sarcoma is rare in non-immunosuppressed patients. Nineteen cases of penile Kaposi sarcoma in HIV-negative patients were reported in 2012. We present the case report of a 48-year-old male patient with no previous medical history, who came to our urology clinic presenting a purple-color papule on the penis glans. Lab tests revealed negative serology for HIV, but tissue PCR was positive for human herpesvirus 8. Histopathology examination after lesion excision was compatible with Kaposi sarcoma. No other cutaneous or mucosal lesions were present. Primary Kaposi sarcoma of the penis is rare, but may occur in non-immunosuppressed patients.

RESUMO O sarcoma de Kaposi é uma doença angioproliferativa que varia de uma lesão cutânea indolente isolada ao envolvimento visceral respiratório e gastrintestinal. É raro em pacientes não imunossuprimidos. Dezenove casos de sarcoma de Kaposi de pênis em pacientes HIV negativos foram relatados em 2012. Descrevemos o caso de um paciente do sexo masculino, 48 anos, sem história pregressa, que se apresentou em nossa clínica urológica com pápula violeta na glande. Os testes de laboratório revelaram sorologia negativa para HIV, mas o PCR em tecido foi positivo para o herpesvírus humano 8. A histopatologia após a excisão da lesão foi compatível com sarcoma de Kaposi. Não existia outra lesão cutânea ou de mucosa. O sarcoma de Kaposi primário de pênis é raro, mas pode ocorrer em pacientes não imunossuprimidos.

Sarcoma de Kaposi em paciente portador de HIV: relato de caso / Kaposi sarcoma in a patient with HIV: case report

Sarcoma de Kaposi é um tumor maligno originado do endotélio vascular que acomete principalmente pele e mucosas. Geralmente, é associado à síndrome da imunodeficiência adquirida ­ aids, apresentando lesões vinhosas, arredondadas que, com o passar dos dias, tornam-se purpúricas, elevadas e com distribuição multifocal. Nesse estudo é relatado o caso de um paciente do sexo masculino, de 42 anos de idade, de fototipo IV, com emagrecimento, diarreia, pápulas e placas eritemato-violáceas nos membros inferiores. Durante a internação, encontrou-se sorologia positiva para HIV e ao realizar histopatológico das lesões cutâneas, confirmou-se o diagnóstico de sarcoma de Kaposi. O objetivo do presente estudo é ressaltar que quando presente infecção pelo citomegalovírus em paciente com aids há maior predisposição para o desenvolvimento de tal neoplasia. (AU)

Kaposi's sarcoma is a malignant tumor originating from the vascular endothelium, which mainly affects the skin and mucous membranes. Generally, it is associated with acquired immunodeficiency syndrome - AIDS, presenting rounded, wine-like lesions that become purpuric, elevated, and multifocal in the course of days. In this study the case of a 42-year-old male phototype IV with weight loss, diarrhea, papules and erythematous-purple plaques in the lower limbs was reported. During the hospitalization, positive serology for HIV was found and the diagnosis of Kaposi's sarcoma was confirmed in the histopathological examination of cutaneous lesions. The objective of the present study is to highlight that when present with cytomegalovirus in a patient with AIDS there is a greater predisposition for the development of such neoplasia. (AU)

Sarcoma de Kaposi clásico en paciente fototipo VI. Primer reporte en Colombia / Classic Kaposi's sarcoma in phototype VI patient. First report in Colombia

Resumen El sarcoma de Kaposi es un tumor vascular de bajo grado, que afecta piel y mucosas, pudiendo comprometer ganglios linfáticos y órganos internos. Generalmente está asociado a infección por virus herpes humano 8, se clasifica en cuatro subtipos clínico-epidemiológicos, entre ellos el tipo clásico, que se manifiesta habitualmente en ancianos blancos, inmunocompetentes, de origen judío o mediterráneo. Se presenta el caso de un varón de 69 años, con fototipo VI, que desarrolló inicialmente máculas violáceas, eritematosas, de borde definido en región externa del pie izquierdo, que progresivamente se tornaron a placas y nódulos violáceos, acompañado de edema. Se realizó biopsia que mostró lesión vascular, cuya inmunohistoquímica presentó reactividad para CD30, CD34 y virus herpes humano 8, confirmando un sarcoma de Kaposi en fase de nódulo. Este caso resulta excepcional y se debe tener presente en el diagnóstico diferencial; los reportes en Suramérica son escasos, siendo este el primero en Colombia. MÉD.UIS. 2017;30(3):129-33.

Abstract Kaposi's sarcoma is a low-grade vascular tumor that affects the skin and mucous membranes and may compromise lymph nodes and internal organs. It is usually associated with human herpes virus infection 8, classified into four clinical-epidemiological subtypes, including the classical type, which is usually manifested in elderly, immunocompetent white Jews or of Mediterranean origin. We present the case of a 69-year-old male, with phototype VI, who initially developed violaceous macules, erythematous, with defined border in the external region of the left foot, which progressively turned to violet plaques and nodules, accompanied by edema. A biopsy was performed showing vascular lesion, whose immunohistochemistry presented reactivity for CD30, CD34 and human herpesvirus 8, confirming a Kaposi's sarcoma in the nodule phase. This case is exceptional and must be kept in mind in the differential diagnosis; the reports in South America are scarce, being the first in Colombia. MÉD.UIS. 2017;30(3):129-33.

Sarcoma de Kaposi variante queloidiana associada à síndrome da imunodeficiência adquirida (SIDA) / Keloidal Kaposi's sarcoma associated with acquired immunodeficiency syndrome (AIDS)

O sarcoma de Kaposi é neoplasia multicêntrica rara originária de células endoteliais com manifestação cutânea e extracutânea. Descreve-se o caso de variante clínica queloidiana de SK, incomum, em paciente do sexo masculino, de 32 anos, portador da síndrome da imunodeficiência adquirida (Aids), com regressão ao tratamento combinado de terapia antirretroviral e radioterapia.

Kaposi's sarcoma is a rare multicentric neoplasm originating from endothelial cells, with cutaneous and extracutaneous manifestation. The present paper describes a case of an uncommon clinic variant of a Kaposi's sarcoma in a 32 year-old male patient bearer of acquired immunodeficiency syndrome (AIDS), with regression after undergoing combined treatment with antiretroviral therapy and radiotherapy.

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

Atypical Kaposi's sarcoma in young inmunocompetent patient

Abstract: Kaposi´s sarcoma is a rare tumor associated with human herpes virus 8 (HHV-8) infection. Four main clinical subtypes have been described. This study reports on a form of KS in an HIV negative and immunocompetent middle-aged man. The only remarkable factor is that he has sex with other men. This form of Kaposi´s sarcoma is rare. It occurs more in younger patients than in the classic form, is limited to the skin, and is associated with a good prognosis. The means of transmission of the virus is through saliva in oroanal or orogenital sexual practices. Mechanisms of tumor development are still not well known. Given the possible increased number of this variant, it would be interesting to extend this study.

Development of Kaposi sarcoma and hemophagocytic lymphohistiocytosis associated with human herpesvirus 8 in a renal transplant recipient

No abstract available.

Angiosarcoma in HIV-negative patients is not associated with HHV-8

Abstract: BACKGROUND: Angiosarcoma is an aggressive, malignant neoplasm of vascular or lymphatic origin. Herpes virus 8 (HHV-8) is a member of the herpes family with a tropism for endothelial cells and it has been proven to induce vascular neoplasms, such as Kaposi's sarcoma. The role of HHV-8 in the pathogenesis of angiosarcoma has not been well defined. OBJECTIVE: To investigate the relationship between the presence of HHV-8 and angiosarcoma. METHODS: In this study, the team investigated the relationship between the presence of HHV-8, as determined by polymerase chain reaction, and angiosarcoma, using samples from patients with epidemic Kaposi's sarcoma as controls. RESULTS: While all control cases with epidemic Kaposi's sarcoma were positive for HHV-8, none of the angiosarcoma cases was. CONCLUSION: These findings support most previous studies that found no association between HHV-8 and angiosarcoma.

Human herpesvirus 8 (HHV-8) detected by nested polymerase chain reaction (PCR) in HIV patients with or without Kaposi's sarcoma. An analytic cross-sectional study / Herpesvírus humano 8 (HHV-8) detectado por reação da polimerase em cadeia do tipo nested PCR em pacientes HIV-positivos com ou sem sarcoma de Kaposi. Um estudo transversal analítico

CONTEXT AND OBJECTIVE: Kaposi's sarcoma (KS) is a common neoplastic disease in AIDS patients. The aim of this study was to evaluate the frequency of human herpesvirus 8 (HHV-8) infection in human immunodeficiency virus (HIV)-infected patients, with or without KS manifestations and correlate HHV-8 detection with KS staging. DESIGN AND SETTING: Analytic cross-sectional study conducted in a public tertiary-level university hospital in Ribeirão Preto, São Paulo, Brazil. METHODS: Antibodies against HHV-8 lytic-phase antigens were detected by means of the immunofluorescence assay. HHV-8 DNA was detected in the patient samples through a nested polymerase chain reaction (nested PCR) that amplified a region of open reading frame (ORF)-26 of HHV-8. RESULTS: Anti-HHV-8 antibodies were detected in 30% of non-KS patients and 100% of patients with KS. Furthermore, the HHV-8 DNA detection rates observed in HIV-positive patients with KS were 42.8% in serum, 95.4% in blood samples and 100% in skin biopsies; and in patients without KS, the detection rate was 4% in serum. Out of the 16 serum samples from patients with KS-AIDS who were classified as stage II, two were positive (12.5%); and out of the 33 samples from patients in stage IV, 19 (57.6%) were positive. CONCLUSION: We observed an association between HHV-8 detection and disease staging, which was higher in the serum of patients in stage IV. This suggests that detection of HHV-8 DNA in serum could be very useful for clinical assessment of patients with KS and for monitoring disease progression.

CONTEXTO E OBJETIVO: Sarcoma de Kaposi (SK) é uma doença neoplásica comum em pacientes com aids. O objetivo deste estudo foi avaliar a frequência da infecção por herpesvírus humano 8 (HHV-8) em pacientes infectados por HIV, com ou sem SK e associar a detecção do HHV-8 com o estadiamento do SK. TIPO DE ESTUDO E LOCAL: Estudo transversal analítico realizado em hospital universitário público terciário de Ribeirão Preto, São Paulo, Brasil. MÉTODOS: Anticorpos contra antígenos de fase lítica do HHV-8 foram detectados por imunofluorescência. O DNA viral de HHV-8 foi detectado em amostras de pacientes pela reação em cadeia da polimerase do tipo nested (nested PCR), que amplificou uma região do fragmento de leitura aberta (ORF)-26 do HHV-8. RESULTADOS: Anticorpos anti-HHV-8 foram detectados em 30% dos pacientes sem SK e 100% dos com SK. Além disso, a detecção de HHV-8 DNA observada em pacientes HIV-positivos com SK foi de 42,8% no soro, 95,4% em amostras de sangue e 100% em biópsias de pele, e em pacientes sem SK foi de 4% no soro. Das 16 amostras de soro de pacientes com SK-AIDS classificados como estádio II, duas foram positivas (12,5%) e, das 33 amostras de pacientes no estádio IV, 19 (57,6%) foram positivas. CONCLUSÃO: Observamos associação entre a detecção do HHV-8 e o estadiamento da doença, que foi maior no soro de pacientes no estágio IV. Isso sugere que a detecção do HHV-8 no soro poderia ser muito útil para a avaliação clínica de pacientes com SK e para o monitoramento da progressão da doença.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

Linfoma primário de efusão em mulher com síndrome da imunodeficiênciaadquirida (SIDA), derrame pleural e ascite: diagnóstico citológico e imunocitoquímico / Primary effusion lymphoma in women with acquired immunodeficiency syndrome (AIDS), pleural effusion, and ascites: cytological diagnosis and immunocytochemistry

O linfoma primário de efusão é tipo raro de forma não Hodgkin que se manifesta com derrame cavitário, em geral associado à imunodepressão. Associa-se à infecção pelo vírus herpes humano 8 (HHV-8), fato necessário para que seja confirmado seu diagnóstico.Este relato descreve o linfoma primário de efusão, diagnosticado por meio de citologia e imunocitoquímica, em paciente com manifestação não usual de comprometimento de duas cavidades serosas. Seu prognóstico é reservado e seu tratamento é limitado.

The primary effusion lymphoma is a rare type of non-Hodgkin form that manifests with cavity stroke, usually associated with immunosuppression. It is associated with infection with the human herpes virus 8 (HHV-8), a necessary fact to confirm the diagnosis. This report describes the primary effusion lymphoma, diagnosed by cytology and immunocytochemistry,in patients with the unusual manifestation of involvement of two serouscavities. Its prognosis is poor, and treatment is limited.

Expressions of Kaposi′ s sarcoma-associated herpesvirus type 8-associated microRNAs k12-1 and k12-12 in Kaposi′s sarcoma and their significance / 中华皮肤科杂志

Objective To measure the expressions of Kaposi′s sarcoma-associated herpesvirus type 8 associated-microRNAs k12-1 (kshv-miR-k12-1)and k12-12 (kshv-miR-k12-12)in Kaposi′s sarcoma tissue, and to assess their relationship with pathological stage and lesion area of Kaposi′s sarcoma, HIV infection, and human herpesvirus type 8 (HPV-8)infection. Methods Totally, 18 paired tissue specimens stored in liquid nitrogen from Kaposi′ s sarcoma lesions and paralesional skin were collected. Total RNAs were extracted from these specimens by using Trizol reagent, and reversely transcribed into cDNA. SYBR Green real-time fluorescence-based quantitative PCR was performed to measure the expressions of kshv-miR-k12-1 and kshv-miR-k12-12 in these specimens. The relationship of kshv-miR-k12-1 and kshv-miR-k12-12 expressions with the pathological stage and lesion area of Kaposi′s sarcoma, HIV and HPV-8 infections was analyzed. Results Compared with paralesional normal skin, Kaposi′s sarcoma lesions showed significantly increased expressions of kshv-miR-k12-1 (2-ΔΔCt: 1.016 ± 1.645 vs. 0.029 ± 0.019, t = 2.542, P = 0.016)and kshv-miR-k12-12 (2-ΔΔCt: 2.104 ± 1.973 vs. 0.102 ± 0.093, t = 4.301, P = 0.000). There were no significant differences in the expressions of kshv-miR-k12-1 or kshv-miR-k12-12 between patients with HIV or HPV-8 infection and those without, among patients with different pathological stages of Kaposi′s sarcoma, or among patients with different lesion areas (all P > 0.05). Conclusion Both kshv-miR-k12-1 and kshv-miR-k12-12 are highly expressed in Kaposi′s sarcoma, but neither of their expressions is related to HIV or HPV-8 infection, pathological stage or lesion area of Kaposi′s sarcoma.

Acroangiodermatitis (pseudo-Kaposi's sarcoma) in an HIV sero-positive patient with syphilis and hepatitis C virus coinfection: clinical and dermatopathological features

Acroangiodermatitis is an angioproliferative disease usually related to chronic venous insufficiency, and it is considered a clinical and histological simulator of Kaposi's sarcoma (KS). Immunohistochemistry is the suitable method to differentiate between these two entities. It reveals the following immunostaining profile: immunopositivity with anti-CD34 antibody is restricted to the vascular endothelium in acroangiodermatitis, and diffuse in the KS (endothelial cells and perivascular spindle cells); immunopositivity with anti-HHV-8 only in KS cases. We report the case of an HIV seropositive patient without apparent vascular disease, who presented violaceous and brownish erythematous lesions on the feet, and whose histopathology and immunohistochemistry indicated the diagnosis of acroangiodermatitis.

Human immunodeficiency virus-1 negative factor protein promotes human herpesvirus-8 viral interleukin-6-induced angiogenesis: role of glycogen synthase kinase-3β/β-catenin signaling pathway / 中华传染病杂志

Objective To explore the role of glycogen synthase kinase (GSK)-3β/β-catenin signaling pathway on human immunodeficiency virus 1 (HIV-1) negative factor (Nef) protein promoting of human herpesvirus-8 (HHV-8) viral interleukin-6 (vIL-6)-induced angiogenesis.Methods GSK-3β mutant plasmid GSK-3β-S9A,dominant negative (DN) form GSK-3β-DN and the control vector pcDNA3.1+ were transfected into endothelial cells which stably expressed HHV-8 vIL-6 or HIV-1 Nef,or co-expressed vIL-6 and Nef protein.Microtubule formation assay was performed to explore microtubule formation ability.A chick embryo chorioallantoic membrane (CAM) model was used to detect angiogenesis.The expression of GSK-3β/β-catenin signaling pathway-related kinases in transfected cells and CAM tissue were further detected by Western blot.The measurement data were compared by t test.Results The activity of GSK-3β was decreased and the ability of HIV-1 Nef protein was enhanced by transfection with GSK-3β-DN in promoting vIL-6 induced microtubule formation (3.42 vs 2.51,t =3.67,P＜0.01) and angiogenesis (6.25 vs 3.97,t=4.06,P＜0.01).In contrast,the activity of GSK-3β was significantly increased and these functions of HIV-1 Nef protein mentioned above were inhibited by transfection with GSK-3β-S9A (0.62 vs2.51,t=8.48,P＜0.01; 0.39 vs 3.97,t=8.59,P＜0.01).The results of Western blot showed that with the elevated level of,β-catenin (in cells:3.53 vs 2.07,t=6.60,P＜0.05; in tissues:2.76 vs 1.74,t=17.40,P＜0.01) and vascular endothelial growth factor (VEGF,in cells:2.68 vs 1.87,t=4.28,P＜0.01; in tissues:2.20 vs 1.39,t=7.08,P＜0.01) were increased in the GSK-3β-DN transfected cells or tissues,while the opposite results were achieved in the GSK-3β-S9A-transfected cells (GSK-3β phosphorylation:0.50 vs 1.47,t=7.33,P＜0.01; β-catenin:1.05 vs 2.62,t=29.50,P＜0.01; VEGF:0.74 vs 2.16,t=20.95,P＜0.01) or tissues (GSK-3β phosphorylation:0.35 vs 1.97,t=10.72,P＜0.01; β-catenin:0.79 vs 1.77,t=5.72,P＜0.01; VEGF:0.43 vs 1.65,t=11.89,P＜ 0.01).Conclusion GSK-3β/β-catenin signaling pathway is involved in vIL-6-induced angiogenesis promoted by HIV-1 Nef protein,which would be valuable for the therapy of Kaposi's sarcoma,an acquired immunodeficiency syndrome,as a potential molecular target.

A Case of Etoposide Therapy in a Patient Newly Diagnosed with Classic Kaposi's Sarcoma and Rheumatoid Arthritis / 대한내과학회지

Kaposi's sarcoma typically occurs in immunocompromised patients, especially those with acquired immunodeficiency syndrome. Human herpesvirus 8 (HHV-8) and human immunodeficiency virus (HIV) may play important roles in the development of Kaposi's sarcoma. We report the case of a 75-year-old male who presented with progressive multiple purplish papules and nodules on the skin for 3 months. Social and past medical histories seemed incompatible with an immunesuppressed condition and tests for HIV antibodies and HHV-8 were negative. He was newly diagnosed with rheumatoid arthritis, according to the 2010 ACR-EULAR criteria. Typical findings of Kaposi's sarcoma were confirmed by biopsy. After treatment with metronomic oral etoposide 25 mg once daily for 9 months, the skin lesions had almost resolved with no serious complication. Thus, we report a rare case of Kaposi's sarcoma developing in a patient with newly diagnosed rheumatoid arthritis who showed a good response to oral etoposide.

Immunohistochemical detection of the latent nuclear antigen-1 of the human herpesvirus type 8 to differentiate cutaneous epidemic Kaposi sarcoma and its histological simulators / Detecção imuno-histoquímica do antígeno nuclear latente-1 do herpesvirus tipo 8 para diferenciar o sarcoma de Kaposi epidêmico cutâneo de seus simuladores histológicos

Kaposi's sarcoma is the most common neoplasia diagnosed in AIDS patients and the expression of the human herpesvirus-8 (HHV-8) latent nuclear antigen-1 has been useful for its histological diagnosis. The aim of this study is to confirm that immunohistochemistry is a valuable tool for differentiating KS from its simulators in skin biopsies of HIV patients. Immunohistochemical and histological analyses were performed in 49 Kaposi's sarcoma skin biopsies and 60 of its histological simulators. Positivity was present in the 49 Kaposi's sarcoma skin biopsies and no staining was observed in the 60 simulators analyzed, resulting in sensibility and specificity of 100%. HHV-8 immunohistochemical detection is an effective tool for diagnosing Kaposi's sarcoma, especially in early lesions in which neoplastic features are not evident. It also contributes to its histological differential diagnosis.

O sarcoma de Kaposi é a neoplasia mais diagnosticada em pacientes com SIDA e a expressão do antígeno nuclear latente-1 do herpesvírus humano tipo-8 (HHV-8) tem se mostrado útil no seu diagnóstico histológico. O objetivo deste estudo é confirmar que o método imuno-histoquímico é uma ferramenta útil para diferenciar o sarcoma de Kaposi cutâneo de seus simuladores histológicos em pacientes HIV positivos. Análise histológica e imuno-histoquímica foram realizadas em 49 casos de sarcoma de Kaposi cutâneo e 60 casos de seus simuladores histológicos. Positividade à imuno-histoquímica para o antígeno nuclear latente 1 do HHV-8 foi observada nos 49 casos de sarcoma de Kaposi e nenhuma reação foi detectada nos 60 simuladores analisados, resultando em 100% de sensibilidade e especificidade. A detecção do HHV-8 por imuno-histoquímica é uma ferramenta útil para o diagnóstico de sarcoma de Kaposi, especialmente na lesão inicial cujo caráter neoplásico não é evidente, e contribui para seu diagnóstico diferencial histológico.

Kaposi's Sarcoma: clinical and pathological aspects in patients seen at the Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil / Sarcoma de Kaposi: achados clínico-patológicos nos pacientes atendidos no Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil

BACKGROUND: Kaposi's sarcoma is a neoplasm of endothelial origin that is divided into four distinct types according to the clinical characteristics and the affected population: Classic (in elder men of Jewish or Mediterranean origin); Epidemic (in patients affected by AIDS); Endemic (in black African men) and Iatrogenic (in patients under immunosuppressive regimens). Human herpesvirus 8 infection is essential but not sufficient for the sarcoma development. OBJECTIVE: To describe the epidemiological, clinical and histopathological aspects of patients with KS seen at the Dermatology Clinic -Cassiano Antônio Moraes University Hospital - Federal University of Espirito Santo, Vitória - ES. METHODS: A descriptive and retrospective study based on clinical charts of patients with KS seen at the Dermatology Clinic from 1986 to 2009. RESULTS: The majority of the 15 cases were male patients (93,3%) and white (60%). Epidemic Kaposi's sarcoma occurred in 80%, and the Classic form in 20%, with no cases in the Endemic or Iatrogenic groups. All the histopatho logical exams of the cutaneous lesions were reviewed and a proliferation of fusiform cells, extravasated erythrocytes and vascular rifts among the largest vessels, assuming the "vessels in vessels" typical aspect, were seen. CONCLUSION: The number of cases of Kaposi's Sarcoma was linear throughout the years of the study, especially of the epidemic form, although the incidence and prevalence of AIDS increased in the state of Espírito Santo. Therefore, if we consider the relation between KS and AIDS, a decreasing line of Kaposi's sarcoma could be seen, especially after the introduction of HAART.

FUNDAMENTOS: O Sarcoma de Kaposi é neoplasia de origem endotelial, dividida em quatro formas clínicas: clássica (homens idosos de origem judaica e mediterrânea), epidêmica (associada ao HIV), endêmica (negros africanos) e iatrogênica (relacionada à imunossupressão). A infecção pelo herpes vírus humano tipo 8 (HHV-8) é necessária, mas insuficiente para que todas as formas possam ocorrer. OBJETIVOS: Avaliar os aspectos epidemiológicos, clínicos e características histopatológicas das lesões dos pacientes com Sarcoma de Kaposi consultados no Serviço de Dermatologia do Hospital Universitário Cassiano Antônio Moraes - Universidade Federal do Espírito Santo, Vitória - ES. MÉTODOS: Estudo retrospectivo, descritivo, realizado pela análise dos prontuários dos pacientes diagnosticados com Sarcoma de Kaposi, durante janeiro de 1986 a dezembro de 2009, no Serviço de Dermatologia. RESULTADOS: Dos 15 pacientes estudados, houve maioria do sexo masculino (93,3%) e predomínio da raça branca (60%). A forma epidêmica foi a mais freqüente (80%), seguida pela clássica (20%). Não foram observadas as formas: endêmica e iatrogênica. A revisão das lâminas das biópsias cutâneas foi feita nos 15 casos, e demonstrou derme com proliferação de células fusiformes, extravasamento de hemácias e fendas vasculares em torno de vasos maiores, com aspecto clássico de "vasos em torno de vasos". CONCLUSÕES: O número de casos de Sarcoma de Kaposi foi linear ao longo do estudo, especialmente da forma epidêmica. Por outro lado, a incidência e a prevalência da AIDS no Espírito Santo foram crescentes. Portanto, considerando-se a relação entre o sarcoma de Kaposi e a AIDS houve decréscimo do primeiro, mais acentuado após a era HAART.