A systematic review on antibiotic therapy of cutaneous bacillary angiomatosis not related to major immunocompromising conditions: from pathogenesis to treatment.

BACKGROUND: Cutaneous bacillary angiomatosis (cBA) is a vascular proliferative disorder due to Bartonella spp. that mostly affects people living with HIV (PLWH), transplanted patients and those taking immunosuppressive drugs. Since cBA is mostly related to these major immunocompromising conditions (i.e., T-cell count impairment), it is considered rare in relatively immunocompetent patients and could be underdiagnosed in them. Moreover, antimicrobial treatment in this population has not been previously investigated. METHODS: We searched the databases PubMed, Google Scholar, Scopus, OpenAIRE and ScienceDirect by screening articles whose title included the keywords "bacillary" AND "angiomatosis" and included case reports about patients not suffering from major immunocompromising conditions to provide insights about antibiotic treatments and their duration. RESULTS: Twenty-two cases of cBA not related to major immunocompromising conditions were retrieved. Antibiotic treatment duration was shorter in patients with single cBA lesion than in patients with multiple lesions, including in most cases macrolides and tetracyclines. CONCLUSIONS: cBA is an emerging manifestation of Bartonella spp. infection in people not suffering from major immunocompromising conditions. Until evidence-based guidelines are available, molecular tests together with severity and extension of the disease can be useful to personalize the type of treatment and its duration.

Angiomatosis bacilar y sarcoma de Kaposi en lesión cutánea de paciente VIH positivo / Bacillary angiomatosis and Kaposi's sarcoma in skin lesion of HIV positive patient

La angiomatosis bacilar (AB) es una enfermedad infec-ciosa poco frecuente, causada por bacterias del género Bartonella spp. transmitidas por vectores como pulgas, piojos y mosquitos. En el ser humano provoca diferentes síndromes clínicos. En pacientes con infección por el virus de inmunodeficiencia humana (VIH) con recuento de LT CD4 + <100 cél/µL se asocia a lesiones angiomatosas con neovascularización que comprometen la piel y, en menor medida, mucosas, hígado, bazo y huesos.El sarcoma de Kaposi (SK) es una neoplasia caracteriza-da por hiperplasia vascular multifocal de origen endotelial relacionada con el herpes virus humano 8. También puede afectar piel, mucosas y vísceras, siendo la variante epidé-mica una enfermedad marcadora de la infección avanzada por VIH. El principal diagnóstico diferencial clínico para las lesiones cutáneas y mucosas del SK es la AB.Presentamos un paciente con enfermedad VIH/sida que desarrolló AB y SK en forma concomitante en la misma lesión cutánea

Bacillary angiomatosis (BA) is a rare infectious disease, caused by bacteria of the genus Bartonella spp, transmitted by vectors such as fleas, lice and mosquitoes. It causes different clinical syndromes in humans. In patients with human immunodeficiency virus (HIV) infection with an LT CD4 + <100 cell/µL count, it is associated with the development of angiomatous lesions with neovascularization involving the skin and, with less frequency, mucous membranes, liver, spleen and bones. Kaposi's sarcoma (KS) is a neoplasm characterized by multifocal vascular hyperplasia of endothelial origin related to human herpes virus 8. It can also compromiso the skin, mucous membranes and viscera, with the epidemic variant being a marker disease of advanced HIV infection. The main clinical differential diagnosis for KS skin and mucosal lesions is the BA.Herein we present a patient with HIV/AIDS disease that developed BA and KS concomitantly in the same skin lesion

Nódulo en la mama tras radioterapia / A Nodule on the Breast after Radiation Therapy

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Bacillary angiomatosis with bone invasion

Abstract Bacillary angiomatosis is an infection determined by Bartonella henselae and B. quintana, rare and prevalent in patients with acquired immunodeficiency syndrome. We describe a case of a patient with AIDS and TCD4+ cells equal to 9/mm3, showing reddish-violet papular and nodular lesions, disseminated over the skin, most on the back of the right hand and third finger, with osteolysis of the distal phalanx observed by radiography. The findings of vascular proliferation with presence of bacilli, on the histopathological examination of the skin and bone lesions, led to the diagnosis of bacillary angiomatosis. Corroborating the literature, in the present case the infection affected a young man (29 years old) with advanced immunosuppression and clinical and histological lesions compatible with the diagnosis.

Angiomiolipoma epitelioide hepático: correlación clínico-patológica en una serie de 4 casos / Epithelioid angiomyolipoma of the liver: clinicopathological correlation in a series of 4 cases

El angiomiolipoma hepático es una neoplasia rara que puede llegar a ser difícil de diagnosticar en casos de biopsia ecoguiada. Hemos estudiado 4 casos de pacientes de sexo femenino, con una media de edad de 51 años. Ninguno de los pacientes se ha presentado con síntomas abdominales específicos, ni otras masas tumorales detectadas incidentalmente. Uno de ellos tenía antecedentes personales relevantes: carcinoma de células renales. Este mismo paciente fue diagnosticado de un angiomiolipoma renal contralateral. Ninguno de los pacientes ha mostrado evidencia de padecer esclerosis tuberosa. Tres de los tumores han sido diagnosticados por biopsia y en sólo uno de los pacientes se ha decidido la extirpación quirúrgica completa del tumor. En ninguno de los pacientes se ha observado evidencia de recidiva de la enfermedad o aumento del tamaño tumoral durante un periodo medio de 45 meses. Histológicamente los tumores se han subclasificado en angiomiolipomas epitelioides. En el 50% de los casos se ha observado hematopoyesis extramedular. La primera impresión diagnóstica mediante métodos de imagen incluía: hiperplasia nodular focal, adenoma hepático, carcinoma hepatocelular y metástasis. Mediante técnicas de inmunohistoquímica se ha demostrado que todos los tumores expresaban marcadores melanocíticos (HMB45 y Melan A) y marcadores de células musculares lisas (actina de músculo liso). El diagnóstico de estos tumores está aumentando debido a los programas de detección precoz de carcinoma hepatocelular en pacientes con cirrosis. Hay que tener en cuenta su amplio espectro de diversidad morfológica para evitar el diagnóstico incorrecto de una neoplasia maligna (AU)

Hepatic angiomyolipoma is a rare neoplasm that can be difficult to diagnose in cases of ecoguide biopsy. We studied 4 cases of female patients with a mean age of 51 years. None of the patients presented specific abdominal symptoms, or other tumour masses detected by chance. One of them had relevant personal history: Renal cell carcinoma. This same patient was diagnosed with a contralateral renal angiomyolipoma. None of the patients showed evidence of tuberous sclerosis. Three tumours have been diagnosed by biopsy and only in one patient was decided to completely remove the tumour surgically. None of the patients had evidence of recurrence of disease or an increase in tumour size over an average period of 45 months. Histologically, the tumours have been sub classified into angiomyolipomas epithelioid. In 50% of the cases, extramedullary haematopoiesis was observed. The first diagnostic impression using imaging methods included: Focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma and metastasis. By immunohistochemistry, it has been demonstrated that all tumours expressing melanocytic markers (HMB45 and Melan A) and markers of smooth muscle cells (smooth muscle actin). The diagnosis of these tumours is increasing due to programmes for early detection of hepatocellular carcinoma in patients with liver cirrhosis. It must be taken into account their broad spectrum of morphological diversity to avoid incorrect diagnosis of a malignant neoplasm (AU)

Manifestaciones dermatológicas asociadas a la infección por VIH/SIDA / Dermatologic manifestations associated with HIV/AIDS

The ongoing human immunodeficiency virus (HIV) infection epidemic coupled with more efficacious and available treatments has led to a larger number of patients living with HIV or AIDS. As a result, skin manifestations related to HIV/AIDS or its therapy have become increasingly more common and are reported to occur in as many as 95% of patients. Herein, we review the most common HIV/AIDS related cutaneous pathologies and classify them into inflammatory, HAART-associated, neoplastic, and infectious manifestations. Cutaneous manifestations should be promptly recognized and treated by physicians and health care personnel in order to provide optimal care.

La epidemia del virus de la inmunodeficiencia humana (VIH) sumado al mayor acceso a terapias antiretrovirales (TARV) han llevado a un aumento del número y la sobrevida de pacientes que viven con esta infección crónica. Se estima que hasta 95% de los pacientes con infección por VIH/SIDA presentarán alguna manifestación cutánea, por lo cual, debemos conocerlas. En la presente revisión se estudiarán las manifestaciones cutáneas de la infección por el VIH/SIDA clasificadas como: manifestaciones inflamatorias, manifestaciones asociadas a la TARV, manifestaciones neoplásicas y manifestaciones infecciosas asociadas a infección por VIH/SIDA (bacterianas, virales, fúngicas y parasitarias). Estas manifestaciones deben ser reconocidas por los médicos y el personal de salud a cargo del tratamiento y control de los pacientes con esta patología crónica.

Angiomiolipoma epitelioide renal / Renal epithelioid angiomyolipoma

Los angiomiolipomas epitelioides de origen renal son una variante muy infrecuente de los angiomiolipomas clásicos pero con la peculiaridad de poder presentar potencial maligno. Su diagnóstico suele ser postoperatorio, puesto que este tumor simula tanto las características clínicas como radiológicas del carcinoma de células renales. Presentamos el caso clínico de una paciente de 14 años que fue diagnosticada de esta patología como hallazgo incidental, y que tras ser sometida a nefrectomía parcial hace tres años actualmente se encuentra asintomática y sin signos de recidiva de la enfermedad (AU)

Epithelioid angiomyolipoma of renal origin are a very rare variant of the classic angiomyolipomas but with the peculiarity to present malignant potential. Its diagnosis is usually after surgery because the tumor simulates both clinical and radiological features of renal cell carcinoma. We report a case of a 14 year old patient who was diagnosed with this disease as an incidental finding, and that after she underwent partial nephrectomy for three years is currently asymptomatic with no signs of disease recurrence (AU)

Angiomatosis bacilar por Bartonella quintana como primera manifestación de infección por VIH: Report of one case / Bacillary angiomatosis

Background: Bacillary angiomatosis is an unusual infectious disease, with angioproliferative lesions, typical of immunocompromised patients. It is caused by Bartonella quintana and Bartonella henselae, two infectious agents of the genus Bartonella, which trigger variable clinical manifestations, including cutaneous vascular and purpuric lesions, and regional lymphadenopathy, and even a systemic disease with visceral involvement. We report a 38-year-old HIV positive male presenting with a history of six months of cutaneous growing purple angiomatous lesions, located also in nasal fossae, rhi-nopharynx and larynx. The skin biopsy was compatible with bacillary angiomatosis. Polymerase chain reaction of a tissue sample showed homology with B. quintana strain Toulouse. The patient was treated with azithromycin and ciprofloxacin with a favorable evolution.

Erythema Elevatum Diutinum in Human Immunodeficiency Virus Infected Patient / 감염과화학요법

Erythema elevatum diutinum (EED) is emerging as a specific HIV-associated dermatosis which can be easily misdiagnosed as Kaposi's sarcoma or bacillary angiomatosis. Until now, no case of HIV-associated EED had been reported in Korea. We report a case of EED in a 49-year-old man with HIV infection. The patient was diagnosed with HIV-infection and treated with a combination of anti-retroviral agents and dapsone. Two years after the start of treatment the lesion had regressed.

Bacillary angiomatosis in HIV-positive patient from Northeastern Brazil: a case report / Angiomatose bacilar em paciente HIV positivo no nordeste brasileiro: relato de caso

It is a report of disseminated bacillary angiomatosis (BA) in a 23-year-old female patient, who is HIV-positive and with fever, weight loss, hepatomegaly, ascites, and papular-nodular skin lesions. The clinical and diagnostic aspects involved in the case were discussed. Bacillary angiomatosis must always be considered in the diagnosis of febrile cutaneous manifestations in AIDS.

Relato de angiomatose bacilar (AB) disseminada em paciente do sexo feminino de 23 anos, HIV positiva, com febre, emagrecimento, hepatomegalia, ascite e lesões de pele pápulo-nodulares. Foram discutidos os aspectos clínicos e diagnósticos envolvidos no caso. Angiomatose bacilar deve sempre ser considerada no diagnóstico de doença febril com manifestações cutâneas na AIDS.

Nódulo angiomatoso epitelioide cutáneo. Un nuevo tumor vascular recientemente descrito / Cutaneous Epithelioid Angiomatous Nodule: A Recently Described Vascular Tumor

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Tratamiento de las infecciones por Bartonella spp / Treatment of human infections caused by Bartonella spp

Las infecciones por Bartonella spp. incluyen un amplio espectro de enfermedades infecciosas emergentes y reemergentes. En este tipo de infecciones no existe una pauta de tratamiento universalizado, por ello, se debe ajustar a cada situación clínica. El objetivo de esta revisión es actualizar los aspectos terapéuticos de las diferentes manifestaciones clínicas provocadas por las bartonellas(AU)

Infections by Bartonella spp. include a wide spectrum of emerging and re-emerging infectious diseases. There is not a universal therapy for this infection, therefore treatment should be chosen individually. The aim of this review is to update the therapeutics aspects of this kind of infections(AU)

La depuracion de endotoxina como tratamiento coadyuvante en la sepsis grave por microorganismos gramnegativos / Endotoxin adsortion as adjuvant therapy in gramnegative severe sepsis

La sepsis grave y el shock séptico siguen siendo situaciones clínicas con alta mortalidad. En los últimos años, un mejor conocimiento de su fisiopatología y la puesta en marcha de un conjunto de medidas encaminadas a una rápida detección y un inmediato tratamiento de los pacientes sépticos han demostrado que pueden disminuir la mortalidad. Así mismo, se sigue investigando en modular la respuesta inflamatoria y limitar la acción dañina de los productos bacterianos sobre el sistema inmune. Fruto de esta investigación se han desarrollado dispositivos adsorbentes de endotoxina para actuar sobre ella por ser ésta una de las principales dianas que inician la cascada inflamatoria en infecciones causadas por gérmenes gramnegativos. En este artículo se revisa la utilidad y modo de empleo que estos dispositivos de depuración de endotoxinas pueden tener como tratamiento coadyuvante del Síndrome séptico. Así mismo se sugiere, en base a los conocimientos actuales, el perfil de paciente que puede beneficiarse de dicha terapia(AU)

The mortality rate of severe sepsis and septic shock remains still high. Within the last years a better knowledge of its physiopathology and the implementation of a group of measures addressed to a fast identification and early treatment of the septic patients have proved to reduce mortality rate. Likewise, it continues being investigated in modulating the inflammatory response and limiting the harmful action of the bacterial products on the immune system. As a result of this research some endotoxin adsorber devices have been designed to control one of the most important targets that start the inflammatory cascade when gramnegative microorganisms are involved. The usefulness that these endotoxin removal devices might have as adjuvant treatment in the Septic Syndrome and its applicability are reviewed in this paper. Likewise a profile of patient that might be to the benefit of this therapy is suggested according to the current knowledge(AU)

Nódulo angiomatoso epitelioide cutáneo proliferativo / Proliferative Cutaneous Epithelioid Angiomatous Nodule

El nódulo angiomatoso epitelioide cutáneo es una proliferación vascular poco frecuente de naturaleza benigna y de reciente descripción. Clínicamente es una lesión rojiza, pequeña, papulosa o nodular, habitualmente única, de crecimiento rápido en el tronco o las extremidades de una persona adulta. Histopatológicamente se trata de una proliferación de células de hábito epitelioide y crecimiento predominantemente sólido, bien delimitada, unilobular, localizada en la dermis superficial. Es común encontrar vasos bien conformados salpicando la lesión, así como cierto infiltrado inflamatorio acompañante. Las células muestran amplios citoplasmas rosados, muchas veces con vacuolas, y núcleos vesiculosos con nucléolos prominentes. La morfología de las células es relativamente uniforme y no se encuentran atipias ni pleomorfismo, aunque no son raras las mitosis. Describimos dos nuevos casos de nódulo angiomatoso epitelioide cutáneo, el primero en una mujer embarazada de 28 años y el segundo en un varón de 27 años, ambos con todos los rasgos descritos como propios de esta entidad, pero con la peculiaridad de que presentaban un elevado índice mitósico. Discutimos el diagnóstico diferencial entre el nódulo angiomatoso epitelioide cutáneo y otras proliferaciones vasculares con citología epitelioide (AU)

The cutaneous epithelioid angiomatous nodule is an uncommon benign vascular proliferation that has only recently been described. Clinically, it usually presents as a solitary, fast-growing, small reddish papulous or nodular lesion on the trunk or limbs of adults. Histopathologic study reveals a proliferation of epithelioid cells and predominantly solid, well delimited, unilobular growth in the superficial dermis. Well defined vessels are often found permeating the lesion, which also shows a certain degree of inflammatory infiltration. The cells contain abundant pink cytoplasm, often with vacuoles, and vesicular nuclei with prominent nucleoli. The morphology of these cells is relatively uniform, without atypia or pleomorphism, although mitoses are not uncommon. We report 2 new cases of cutaneous epithelioid angiomatous nodules, the first in a 28-year-old pregnant woman and the second in a 27-year-old man. In both cases, the usual characteristics of this entity were present, but with the peculiarity of a high mitotic index. We discuss the differential diagnosis of cutaneous epithelioid angiomatous nodules with other vascular proliferations that exhibit epithelioid cytology (AU)

Botriomicosis cutánea / Cutaneous botryomycosis

La botriomicosis es una infección granulomatosa crónica supurativa, poco frecuente, caracterizada por la presencia en el estudio histológico de gránulosbasófilos que contienen los microorganismos causantes. Esta entidad puede afectar la piel, y mucosas solamente o asociarse afectación visceral.Traumatismos, cuerpos extraños y deficiencias en los mecanismos de defensa son factores predisponentes de esta enfermedad, pero también puededesarrollarse en individuos aparentemente sanos.Presentamos un caso de botriomicosis cutánea afectando la región patelar de paciente inmunocompetente, con un traumatismo local previo que precisósutura años antes como único factor predisponente. Se intenta ilustrar la dificultad que existe en ocasiones para realizar el diagnóstico de estaentidad clínica y la pobre respuesta a diversas opciones terapéuticas siendo frecuentes las recidivas (AU)

Botryomycosis is an uncommon, chronic, granulomatous, suppurative disease characterized by the presence of abscess containing granules with thecausative bacterias. This infection can involve skin alone or in association with visceral organs. Trauma, foreign bodies and immunodeficiency predisposefor the disease, but botryomycosis may develop in apparently helathy individuals.We report a woman with lesions in the patellar area following local trauma with no immunodeficiency, as a predisposing factor. We emphasize the difficultyfor the diagnosis of this entity and the limited response to different treatments (AU)

Angiomatosis bacilar por Bartonella quintana en un paciente con infección por virus de inmunodeficiencia humana / Bacillary angiomatosis caused by Bartonella quintana in an human immunodeficiency virus positive patient

We report the first case of bacillary angiomatosis due to Bartonella quintana affecting a Chilean a HIV positive patient in Chile. He was a 27 years old, heterosexual male, indigentman known to be HIV positive serological status known from September, 2003, under irregular medical control. On April, 2005, he presented a progressive abscess in the frontal region and erythematous papules in the extremities, that extended to face, thorax and mucoses, becoming nodular and violaceous lesions. Bacillary angiomatosis diagnosis was initially sustained on account of the clinical manifestations, and was confirmed by serology and Warthin Starry staining from a skin biopsy. The etiological agent was identified as Bartonella quintana through universal RPC performed from a cutaneous nodule to detect 16S rRNA gen. Azithromycin plus ciprofloxacin was started, besides of anti retroviral therapy antiretroviral, with the lesions being progressively disappearing.

Reportamos el primer caso de angiomatosis bacilar por Bartonella quintana en un paciente con infección por VIH en nuestro país. Este corresponde a un hombre de 27 años, heterosexual, indigente, seropositivo para VIH conocido desde septiembre de 2003, en control irregular. En abril de 2005, el paciente desarrolló un aumento progresivo de volumen en la región frontal y aparición de pápulas eritematosas en las extremidades, que luego se extendieron a la cara, tórax y mucosas, tornándose nodulares y violáceas. El diagnóstico de angiomatosis bacilar se planteó inicialmente por el cuadro clínico del paciente, siendo confirmado por serología y tinción de Warthin Starry positiva en la biopsia de piel. El agente causal se identificó como Bartonella quintana por RPC universal para el gen del 16S ARNr de un nódulo cutáneo. Se inició terapia antimicrobiana con azitromicina y ciprofloxacina, además de terapia antiretroviral, con desaparición de las lesiones en forma progresiva.

Angiomatosis bacilar: resolución espontánea? / Bacillary angiomatosis: spontaneous resolutión?

La angiomatosis bacilar es una enfermedad infecciosa oportunista con manifestaciones sistémicas, no conocidas antes de la epidemia del virus de la inmunodeficiencia humana (HIV). Se manifiesta usualmente en pacientes inmunodeprimidos con recuentos de linfocitos T CD4+ inferiores a 200 células /µL y su evolución, sin tratamiento, cuenta con elevados índices de mortalidad. Presentamos un paciente de sexo masculino, de 27 años, con antecedentes de SIDA, que había iniciado HAART 18 días antes de su derivación a nuestro servicio. Al examen físico se observaban lesiones generalizadas de 1 mes de evolución, compatibles clínica e histológicamente con angiomatosis bacilar, que resolvieron en forma espontánea en 4 semanas.

Angiomatose bacilar pós-transplante renal: apresentação de um caso / Bacillary angiomatosis after kidney transplantation

A bartonela causa a Doença da arranhadura do gato, mas em imunossuprimidos pode levar à doença sistêmica, denominada angiomatose bacilar.Descrição do caso: Masculino, 15 anos, transplantado há 3 anos, em uso de FK, MMF e prednisona com função renal preservada. Refere tumoraçãoavermelhada e dolorosa na região mandibular esquerda há 30 dias, com febre elevada há 7 dias. Esta em regular estado geral, descorado, 39ºC, commassa submandibular de 4cm de diâmetro, consistência carnosa com vesículas na superfície e hepato-esplenomegalia. Bx da massa compatível com Angiomatose Bacilar. Evoluiu bem com Cloranfenicol e Eritromicina. Apresentou elevação da creatinina sérica, sendo feita hipótese de rejeição e realizadopulso de metilprednisolona com normalização da função renal. Conclusões: Como a soroprevalência de Bartonela, em gatos, é elevada em nosso meio, deve-se estar alerta para seu diagnóstico. Pacientes imunossuprimidos podem apresentar formas sistêmicas graves de infecções comuns que, pelararidade, podem ser diagnosticadas tardiamente e carrear maior morbidade.

Bartonela sp causes cat scratch disease but in immunosuppressed hosts it can be associated with a systemic disease named bacillary angiomatosis.Description of case: A 15-year old boy with a successful kidney transplant for 3 years had been treated with FK, MMF and prednisone. He reported ared and painful mass in the left submandibular area for 30 days, and high fever for the last 7 days. He looked sick, pale and had a submandibular mass of 4cm in diameter with vesicles in its surface and hepatosplenomegaly. Biopsy of the mass was suggestive of bacillary angiomatosis. He was treated withchloramphenicol and erythromycin and had a favorable course. An increase of creatinine, interpreted as rejection, was successfully managed with a pulseof methylprednisolone. Conclusions: As the prevalence of bartonelosis in cats is elevated in our country, physicians should be aware of that diagnosticpossibility. Immunocompromised hosts may present rare systemic forms of common diseases that can be diagnosed late and produce sequels.

Nódulo angiomatoso epitelioide cutáneo. ¿Variante superficial y extrafacial de hemangioma epitelioide? / Cutaneous epithelioid angiomatous nodule. Is it a superficial and extrafacial variant of epithelioid hemangioma?

El nódulo angiomatoso epitelioide cutáneo (NAEC), esuna lesión vascular recientemente descrita que muestrahallazgos histológicos distintivos y un curso evolutivobenigno. Clínicamente suele presentarse como un nódulo opápula eritematosa de reciente aparición, predominantementelocalizados en tronco y extremidades; y a pesar, delas aparentes diferencias clínico-patológicas, no es posiblecon certeza descartar que pueda tratarse de una variantepoco usual de hemangioma epitelioide (HE)

Cutaneous epithelioid angiomatous nodule (CEAN), is arecently described vascular lesion with distinct histologicalfeatures and apparently benign behaviour. The lesion is predominantlylocated in the trunk and extremities. It appearsas a small erythematous papule or nodule with a short history.Despite the apparent clinicopathologic differences, thepossibility that CEAN represent a very unusual variant ofepithelioid hemangioma can not absolutely be excluded