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

Síndrome de Gorham Stout en un paciente pediátrico. Presentación de un caso

Dentro de la clasificación de los síndromes de osteòlisis idiopática, la enfermedad de Gorham-Stout ocupa el cuarto lugar. Es un cuadro clínico caracterizado por la destrucción progresiva de tejido óseo y proliferación vascular, con angiomatosis y linfangiomatosis ósea que produce una osteòlisis progresiva del esqueleto, con pérdida de masa ósea en las áreas afectadas. Este artículo tiene como objetivo presentar un paciente pediátrico con síndrome de Gorham Stout atendido en el Hospital Provincial Pediátrico Universitario José Luis Miranda, de Villa Clara. Es una paciente femenina, de dos años de edad, con cuadro febril de una semana de evolución, aumento de volumen en región dorsal y pérdida de peso. Al examen físico se constataron palidez cutáneo-mucosa y lesión nodular indolora de tres centímetros en la región dorsal. Reapareció la fiebre y se observó marcado aumento de volumen en la región dorso lumbar. Mediante tomografía axial computarizada y resonancia magnética se evidenció osteòlisis de cuerpos vertebrales desde D8 a L2 y aumento de las partes blandas adyacentes. Las manifestaciones clínicas e imagenológicas obligaron a descartar etiologías infecciosas y neoformativas. Se descartaron las primeras y por cumplir con los criterios requeridos se concluyó como enfermedad de Gorham. Esta es una rara y peculiar condición patológica músculo-esquelética en la cual el hueso, virtualmente, se desintegra y es reemplazado por tejido conectivo vascular. Su etiología es especulativa, con una presentación clínica muy variable. Los estudios imagenológicos resultan de gran utilidad. Posee un pronóstico indeterminado a pesar de las opciones terapéuticas empleadas. Por ser un síndrome extremadamente raro se decidió la presentación del caso.

Within the idiopathic osteolysis syndromes classification, Gorham-Stout disease ranks fourth. It is a clinical picture characterized by the progressive destruction of bone tissue and vascular proliferation, with angiomatosis and bone lymphangiomatosis that produces a progressive osteolysis of the skeleton, with loss of bone mass in the affected areas. This article aims to present a pediatric patient with Gorham Stout syndrome treated at the José Luis Miranda University Pediatric Provincial Hospital in Villa Clara. A 2-years-old female patient with a one week fever evolution, increased volume in the dorsal region and weight loss. The physical examination revealed cutaneous-mucosal pallor and a three centimeters painless nodular lesion in the dorsal region. The fever recurred and a marked increase in volume was observed in the dorsal lumbar region. Computed axial tomography and magnetic resonance imaging revealed osteolysis of the vertebral bodies from D8 to L2 and an increase in the adjacent soft tissues. The clinical and imaging manifestations made it necessary to dismiss infectious and neoformative etiologies. The former were dismissed and because they fulfill the required criteria it was concluded as Gorham's disease. This is a rare and peculiar musculoskeletal pathological condition in which bone virtually disintegrates and is replaced by vascular connective tissue. Its etiology is speculative, with a highly variable clinical presentation. Imaging studies are very useful. It has an indeterminate prognosis despite the therapeutic options used. Because it is an extremely rare syndrome, the presentation of the case was decided.

Pseudoangiomatous stromal hyperplasia of the breast: a rare condition ­ from diagnosis to treatment

Pseudoangiomatous Stromal Hyperplasia (PASH) of the breast is a rare condition that consists of the proliferation of the breast myofibroblastic stromal cells, lining anastomosing vascular slit-like spaces. This condition is not considered a pre-malignant lesion and affects mainly premenopausal women. Its etiology is still uncertain, but its behavior points to a hormonal cause. It has a varied clinical presentation and can be diagnosed as an incidental finding of biopsies or with the manifestation of clinical signs and symptoms. As for the diagnosis, it can be performed with the correlation between clinical data, imaging and histopathological analysis. Due to its rare nature, there are still no prospective studies regarding treatment, but, in most cases, clinical and radiological follow-up is a safe strategy. The aim of this paper is to synthesize the data available in the literature about this condition, which, although benign in nature, can bring important aesthetic, musculoskeletal and psychological repercussions

Multicentric osteoblastoma-like: report of a rare case in the craniofacial region / Osteoblastoma-like multicêntrico: relato de um caso raro na região craniofacial

ABSTRACT The osteoblastoma-like tumor is a rare condition with limited information about its treatment in the current medical literature. The tumor histologically resembles osteoblastoma, although the imaging features are similar to those seen in primary vascular lesions. Due to the uncertainty in the biological behavior of this tumor and because it is an unusual diagnosis, treatment can be aggressive, such as amputation, en bloc resection, and/or chemotherapy. This work reports a rare case of a patient with multicentric osteoblastoma-like in the craniofacial region, treated aggressively with total resection of the lesions.

RESUMEN El tipo osteoblastoma es una afección poco común y la literatura médica actual tiene información limitada sobre su tratamiento. Es histológicamente similar al osteoblastoma, aunque las características de las imágenes son similares a las que se observan en las lesiones vasculares primarias. Por la incertidumbre de su comportamiento biológico y por tratarse de un diagnóstico poco habitual, el tratamiento puede ser agresivo, con amputación, resección en bloque y/o quimioterapia. Este trabajo reporta un caso raro de osteoblastoma multicéntrico en la región craneofacial, tratado de manera agresiva con resección total de las lesiones.

RESUMO O osteoblastoma-like é uma condição rara, e a literatura médica atual tem informações limitadas sobre seu tratamento. Ele se assemelha histologicamente ao osteoblastoma, embora as características imaginológicas sejam semelhantes às observadas nas lesões vasculares primárias. Devido à incerteza do seu comportamento biológico e por se tratar de um diagnóstico incomum, o tratamento pode ser agressivo, com amputação, ressecção em bloco e/ou quimioterapia. Este trabalho relata um caso raro de osteoblastoma-like multicêntrico em região craniofacial, tratado de forma agressiva com ressecção total das lesões.

Dental care challenges in Sturge-Weber syndrome: a case report / Desafios do tratamento odontológico na síndrome de Sturge-Weber: relato de caso

Introduction: Sturge-Weber syndrome (SWS) is a rare condition characterized by facial capillary malformation, involves ocular, neurological, and cutaneous alterations. Associated with unilateral characteristic port-wine stains, gingival growth and purple-red coloration. Aim: his case aims to report dental treatment challenges in patients with SWS and importance of oral health maintenance in these individuals. Case report: a 20-year-old woman with an established diagnosis of SWS, presented bad breath and spontaneous gingival bleeding, with gingival growth and reddish-purple spots spread to labial and alveolar mucosa, tongue, and palate. Conditioning of the patient's oral environment by supra and subgingival scraping, dental unit extraction was performed. A conservative treatment plan was adopted for management adequacy of oral environment owing to possible complications inherent to the condition. Conclusion: it is important to emphasize the importance of dental surgeon's performance in relation to a multidisciplinary health team, as well as cooperation of patient, to obtain better results from the proposed therapy.

Introdução: a síndrome de Sturge-Weber (SSW) é uma condição rara caracterizada por malformação capilar facial, envolve alterações oculares, neurológicas e cutâneas. Associada a manchas unilaterais características do vinho do porto, crescimento gengival e coloração vermelho-púrpura. Objetivo: este caso tem como objetivo relatar desafios do tratamento odontológico em pacientes com SSW e a importância da manutenção da saúde bucal nesses indivíduos. Relato de caso: paciente do sexo feminino, 20 anos, com diagnóstico estabelecido de SSW, apresentou mau hálito e sangramento gengival espontâneo, com crescimento gengival e manchas roxas avermelhadas espalhadas pela mucosa labial e alveolar, língua e palato. Condicionamento do ambiente oral do paciente por raspagem supra e subgengival, foi realizada extração da unidade dental. Foi adotado um plano de tratamento conservador para adequação do manejo do ambiente bucal devido a possíveis complicações inerentes à condição. Conclusão: é importante enfatizar a importância do desempenho do cirurgião-dentista em relação a uma equipe multidisciplinar de saúde, bem como a cooperação do paciente, para obter melhores resultados com a terapia proposta.

Síndrome de Sturge-Weber: revisión de la literatura / Sturge-Weber syndrome: literature review

El síndrome de Sturge-Weber es un trastorno neurocutáneo, congénito, esporádico e infrecuente que afecta aproximadamente a 1 de cada 20 000 a 50 0000 nacidos vivos y que se relaciona con una mutación genética activadora somática en GNAQ. Clínicamente se caracteriza por la presencia de una mácula en vino de Oporto en la piel de territorio trigeminal, angiomatosis leptomeníngea y glaucoma. Puede asociarse a diferentes manifestaciones clínicas, de las cuales las crisis epilépticas representan la manifestación neurológica más frecuente que se asocia a un deterioro cognitivo importante en estos pacientes. En el presente artículo se realiza una revisión descriptiva de la literatura sobre los aspectos etiológicos, fisiopatológicos, de clasificación, clínicos, diagnósticos y del tratamiento del síndrome de Sturge-Weber.

Sturge-Weber syndrome is a rare, sporadic, congenital neurocutaneous disorder affecting approximately 1 in 20,000 to 50,000 live births that is associated with a somatic activating gene mutation in GNAQ. Clinically it is characterized by the presence of a port wine stain on the skin of trigeminal territory, leptomeningeal angiomatosis and glaucoma. It can be associated with different clinical manifestations, of which the epileptic seizures represents the most frequent neurological manifestation associated with significant cognitive impairment in these patients. This article makes a descriptive review of the literature on the etiological, pathophysiological, classification, clinical, diagnostic and treatment aspects of Sturge-Weber syndrome.

Sturge-Weber Syndrome – A Case Report

Encephalo-trigeminal angiomatosis otherwise known as struge Weber syndrome is characterised by port-wine stain. Here wereport a case with typical charecteristics of the same and description about the patients clinical presentations

Fractura patológica de cadera en edad pediátrica. Revisión de la angiomatosis quística ósea / Pathological hip fracture of pediatric age. Revision of the cystic angiomatosis of the bone

Resumen: Introducción: La angiomatosis quística ósea es una enfermedad con solamente 200 casos descritos basados en la proliferación no maligna angiomatosa en tejido óseo y vísceras. Se observa más en la pelvis, los huesos largos y la cintura escapular. La evolución clínica varía de formas autolimitantes a osteólisis agresiva masiva (Gorham-Stout). Su diagnóstico es por exclusión, con datos clínico-radiológicos (imágenes líticas) y resultados histopatológicos no específicos. Caso clínico: Nuestro estudio se basa en el caso clínico de un paciente de 14 años diagnosticado con angiomatosis quística ósea, con afección ósea diseminada, que vino a nuestro centro por el dolor y la impotencia funcional en la cadera derecha, diagnosticando una fractura pertrocantérea. Se le hizo una osteosíntesis con tornillo deslizante de la placa VERSA de cuatro agujeros. Se utilizó un injerto alogénico de hueso esponjoso de la cabeza femoral. La evolución fue satisfactoria con ambulación a plena carga por mes sin requerir medicación analgésica y control de rayos X a los ocho meses, los cuales mostraron signos de completa integración del injerto. La escala analgésica del dolor (EVA) mostró una puntuación de 2/10 por mes. Discusión: Hay controversia con respecto al tratamiento de estas fracturas por la osteosíntesis con el injerto del hueso. Nos enfrentamos a un caso sin criterios clínicos serios, pero con múltiples focos de osteólisis. Se decidió no intervenir profilácticamente la cadera contralateral, ya que es asintomática y hay diferentes patrones de evolución descritos en la literatura y los riesgos en la cirugía

Abstract: Introduction: Bone cystic angiomatosis is a disease with only 200 cases described, based on angiomatous nonmalignant proliferation, in bone tissue and viscera. It focuses on pelvis, long bones and scapular waist. Clinical evolution ranges from self-limiting forms to massive aggressive osteolysis (Gorham-Stout).Its diagnosis is of exclusion, with nonspecific clinical, radiological (lytic images) and histopathological findings. Clinical case: Our study is based on the clinical case of a 14-year-old man diagnosed with bone cystic angiomatosis, with disseminated bone involvement, who came to our center for pain and functional impotence in the right hip, diagnosing a pertrochanteric fracture. It was operated by open reduction and osteosynthesis with four-hole VERSA sliding plate screw. An allogeneic graft of cancellous bone was used from femoral head. The evolution was satisfactory, with ambulation at full load per month without requiring analgesic medication and control X-ray at eight months that showed signs of complete integration of the graft. The analgesic pain scale (visual analogue scale) showed a score of 2/10 per month. Discussion: There is controversy regarding the treatment of these fractures by osteosynthesis with bone graft. We are facing a case without serious clinical criteria, but with multiple foci of osteolysis. It was decided not to intervene prophylactically the contraleteral hip, since it is asymptomatic and there are different patterns of evolution described in the literature and risks in surgery.

Síndrome de Stuger- Weber (SSW) Experiencia en cuatro pacientes con episodios de déficit motor transitorio (DM) / Stuger-Weber syndrome (SSW) Experience in four patients with episodes of transient motor deficit (DM)

El síndrome de Sturge Weber es un trastorno neurocutáneo congénito, esporádico, causado por una mutación somática activadora en el gen GNAQ, con una incidencia de uno cada 20.000-50.000 nacidos. Se caracteriza por la presencia de una mancha facial color vino de Oporto, angiomatosis leptomeníngea y glaucoma. La manifestación neurológica más común son las convulsiones, que suelen comenzar en los primeros meses de vida. Un 25% de los pacientes con angioma leptomeníngeo desarrolla episodios de déficit motor (EDM), que pueden durar entre horas y días en recuperarse totalmente. Los estudios de neuroimagen permiten visualizar la angiomatosis leptomeníngea, ayudando al diagnóstico del síndrome de Sturge Weber. El pronóstico de la enfermedad dependerá de la extensión de la malformación leptomeníngea y del grado de afectación ocular. Existe consenso de que el uso de aspirina (AAS) a bajas dosis es bien tolerado, seguro y reduce el número de convulsiones y EDM, mejorando el pronóstico neurológico a largo plazo de estos pacientes

Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder caused by a somatic activating mutation in GNAQ; it affects 1 in every 20,000 to 50,000 newborns. It is characterized by a facial Portwine stain, leptomeningeal angiomatosis, and glaucoma. Seizures are the most common neurological manifestation and typically present in the first months of life. 25% of patients with leptomeningeal angiomatosis develop stroke like epsisodes, transient motor deficit lasting hours to days up to recovery. Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma.There is a consensus that low-dose aspirin is safe, well tolerated and reduces seizures and stroke like episodes in Sturge-Weber syndrome patients. Low-dose aspirin may improve Sturge-Weber syndrome patients' neurologic outcomes

Advances in angiomatosis of breast with vertical infiltration / 医学研究生学报

Angiomatosis is a benign tumor of mesenchymal origin that can vertically infiltrate adjacent tissues. Most reported cases of angiomatosis were in the head and neck, internal organs and tissues, limb muscles, bones, etc.. However, there were only a few cases of breast angiomatosis reported in China. Angioangiosis is rare with high local recurrence rate. Clinical manifestations and pathological diagnosis are easy to be confused with other breast diseases, therefore easy to be misdiagnosed and mistreated. The current study reviewed relevant literature on angiotomasis. Clinical manifestations, auxiliary examinations, pathology, etc. of breast angiomatosis were introduced along with other disease that need to be differentially diagnosis from. In this paper, current progress of diagnosis, treatment, recurrence related risk factors of angiomatosis were summarized, in the hope of improving the clinicians’ understanding and provide basis for further diagnosis and treatment.

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.

Síndrome de Sturge Weber: relato de caso / Sturge-Weber Syndrome: a case report

A Síndrome de Sturge-Weber é uma síndrome rara, não hereditária e de etiologia desconhecida. É caracterizada por proliferações vasculares harmatomatosas, nos tecidos do cérebro e face e ocorre em cerca de 1/50.000 nascimentos. Este trabalho relata o caso de uma paciente de 7 anos com Síndrome de Sturge-Weber e aborda as etapas de seu tratamento junto com uma equipe multidisciplinar na APAE-Santo Ângelo, de 2001 até 2009(AU)

The Sturge-Weber syndrome is a rare, nonhereditary syndrome of unknown etiology. It is characterized by hamartomatous vascular proliferations in the brain and face tissues and occurs in about 1/50,000 live births. This paper reports the case of a 7-year-old patient with Sturge-Weber syndrome, and addresses the steps for its treatment with a multidisciplinary team in the APAE-Santo Ângelo from 2001 until 2009(AU)

Angiomatosis bacilar por Bartonella Henselae en un paciente con SIDA: Presentación de un caso y revisión de la literatura / Bacillary angiomatosis due to Bartonella Henselae in an AIDS patient: Report of a case and literature review

Se presenta un paciente con enfermedad VIH/SIDA avanzada, que desarrolló un cuadro de angiomatosis bacilar por Bartonella henselae, con manifestaciones cutáneas, mucosas, sistémicas y que respondió favorablemente al tratamiento con antibióticos más la terapia antirretroviral.

We report a case of a patient with advanced HIV/AIDS disease who developed a bacillary angiomatosis due to Bartonella henselae with cutaneous, mucosae, systemic compromise and a good response to the antimicrobial therapy plus highly active antiretroviral therapy.

A Case of Diffuse Dermal Angiomatosis / 대한피부과학회지

Diffuse dermal angiomatosis is an acquired, benign vascular proliferation with poorly circumscribed, violaceous, and livedoid plaques with frequent ulceration. Histologically, there is diffuse and interstitial proliferation of CD31-positive endothelial cells and myofibroblasts within the dermis. Endothelial atypia, mitoses, and vasculitis are lacking. Here we describe a 52-year-old male who presented with erythematous and purpuric patches on the trunk and lower extremities for 2 years. Histologic examination demonstrated a dense proliferation of endothelial cells in the papillary and reticular dermis. Small vascular spaces and extravasated erythrocytes were noted. Immunohistochemical staining using anti-CD31 antibody was positive.

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.

A case of bacillary angiomatosis developed at a burn site.

Bacillary Angiomatosis (BA) is frequently seen in patients with human immunodeficiency virus (HIV)-induced immunodeficiency. Our patient was a case that developed granuloma-like lesions in the area of a burn, 8 days after being burnt on the upper right arm by scalding water. No indication of immune deficiency was observed and no history of direct contact with cats was evident. By the sixth day of the patient's admission to our clinic, some of the lesions had reached a diameter of 2.5 cm. An excision biopsy was carried out from the lesions present on the patient. Electron microscopy revealed solitary bacilli located close to the capillary wall. Oral erythromycin treatment was implemented at 250 mg, 4 times a day for 2.5 months. Within this period of treatment, the lesions regressed completely, and a complete cure was achieved. This case demonstrates that BA must be considered in the differential diagnosis of both HIV-infected and immunocompetent patients.

Sturge-Weber syndrome: a case report

Introduction: The Sturge-Weber Syndrome, also known as encephalotrigeminal angiomatosis, is a rare vascular neurocutaneous alteration. The main clinical features of this syndrome are facial vascular cutaneous naevus, usually unilateral, which often follows the outline distribution of trigeminal nerve. Objective: To report a clinical case of Sturge-Weber Syndrome in a 29-yeral-old male patient who presented oral manifestations related to the syndrome. Case report and conclusion: The patient reported that he had presented a cutaneous vascular nevus on the face during childhood as well as epileptic crisis episodes. However, he had no ophthalmic alterations. Sturge-Weber syndrome is a systemic condition commonly affecting oral cavity through vascular lesions, therefore, dentists' knowledge is extremely important to provide an adequate dental treatment without complications.

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.

Síndrome de Sturge-Weber e suas repercussões oculares: revisão da literatura / Sturge-Weber syndrome and its ocular effects: a review

A síndrome de Sturge-Weber (SSW) é uma desordem neuro-oculocutânea, rara e congênita. Esta facomatose, também conhecida como angiomatose encéfalotrigeminal, é definida pela tríade clássica: hemangiomas cutâneo, meníngeo e ocular. Apesar de sua apresentação típica, formas incompletas não são incomuns. O glaucoma está frequentemente presente e seus mecanismos fisiopatológicos permanecem incertos. Talvez por isso, o manejo clínico e cirúrgico do glaucoma associado à síndrome de Sturge-Weber (GSSW) seja um desafio para a prática oftalmológica, muitas vezes com resultados desapontadores. Apresentamos uma revisão da literatura com ênfase no GSSW, sua patogênese e perspectivas terapêuticas.

The Sturge-Weber syndrome (SWS) is a rare congenital neuro-oculocutaneous disorder. This phacomatosis, also known as encephalotrigeminal angiomatosis, is defined by the classic triad: cutaneous, meningeal and ocular hemangiomas. Despite its typical presentation, incomplete forms are not uncommon. Glaucoma is often present and their pathophysiological mechanisms remain uncertain. Pherhaps this is why the clinical and surgical management of glaucoma associated with Sturge-Weber syndrome (GSSW) is a challenge for the ophthalmic practice, often with disappointing results. We present a literature review with emphasis on GSSW, its pathogenesis and therapeutic perspectives.

Bacillary angiomatosis with atypical clinical presentation in an immunocompetent patient.

Bacillary angiomatosis is a recently described infectious disease that usually affects immunosupressed hosts with a previous history of contact with cats. We report a rare case of bacillary angiomatosis in an immunocompetent 59-year-old woman with no history of previous exposure to cats, and atypical clinical features (fever and subcutaneous nodules with ulceration on the left ankle). Histopathology of the lesion showed extensive ulceration and reactive tumor-like vascular proliferation of the blood vessels with swollen endothelial cells and an inflammatory infiltrate including neutrophils and lymphocytes in the dermis and subcutis. Staining with the Warthin-Starry method demonstrated the presence of clustered bacilli located in the extracellular matrix adjacent to the proliferating endothelial cells. Diagnosis was confirmed with the detection of Bartonella spp. DNA in the affected skin and in bone marrow using polymerase chain reaction.