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Resumo A doença veno-oclusiva pulmonar (DVOP) e a hemangiomatose capilar pulmonar são tipos raros de substratos histopatológicos dentro do espectro da hipertensão arterial pulmonar (HAP) com prognóstico muito ruim. Caracterizam-se por um processo fibroproliferativo generalizado das veias e/ou capilares de pequeno calibre com preservação das veias maiores, resultando em um fenótipo de hipertensão pulmonar pré-capilar. A apresentação clínica é inespecífica e semelhante a outras etiologias de HAP. O diagnóstico definitivo é obtido por meio de análise histológica, embora a biópsia pulmonar não seja aconselhada devido ao maior risco de complicações. No entanto, alguns achados adicionais podem permitir um diagnóstico clínico presuntivo de DVOP, especialmente história de tabagismo, uso de drogas quimioterápicas, exposição a solventes orgânicos (particularmente tricloroetileno), baixa capacidade de difusão do monóxido de carbono (DLCO), dessaturação ao esforço e evidências de doença venosa sem doença cardíaca esquerda no exame de imagem, manifestada por uma tríade clássica de opacidades em vidro fosco, linhas septais, e linfadenopatias. O transplante pulmonar é o único tratamento eficaz e os pacientes devem ser encaminhados no momento do diagnóstico, devido à rápida progressão da doença e ao prognóstico ruim. Apresentamos o caso de um homem de 58 anos com HAP com características de envolvimento venoso/capilar em que a suspeita clínica, o pronto diagnóstico e o encaminhamento precoce para transplante pulmonar foram determinantes para um bom desfecho.

Abstract Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis. We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.

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Lobular capillary hemangioma or pyogenic granuloma is a benign vascular tumor of the skin or mucous membranes. Most patients present a single lesion. It manifests clinically as an erythematous, friable, and fast-growing tumor. This report details a case with exuberant presentation in a patient with ankylosing spondylitis, using adalimumab. Factors triggering pyogenic granuloma are not well known. They may spontaneously regress, but most require treatment.

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Abstract Lobular capillary hemangioma or pyogenic granuloma is a benign vascular tumor of the skin or mucous membranes. Most patients present a single lesion. It manifests clinically as an erythematous, friable, and fast-growing tumor. This report details a case with exuberant presentation in a patient with ankylosing spondylitis, using adalimumab. Factors triggering pyogenic granuloma are not well known. They may spontaneously regress, but most require treatment.

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Fundamento: el hemangioma piógeno o granuloma piógeno, incluye neoplasias que se caracterizan por un gran número de vasos sanguíneos normales o anormales que resultan difíciles de diferenciar de malformaciones o hematomas. Es una tumoración de tejidos blandos, excepcionalmente en el tejido óseo, siendo relativamente frecuente, resulta de un desarrollo reactivo focal de tejido fibrovascular con proliferación endotelial y se les relaciona con traumas, irritación, cambios hormonales, el embarazo. Objetivo: describir la localización infrecuente y la evolución de la enfermedad. Caso clínico: paciente blanco masculino antecedentes de notar una lesión de aspecto granulomatoso en cara interna del trago, por cuyo motivo se incindió con anterioridad y por el crecimiento rápido se decidió exceresis total y biopsia, se informó la presencia de un hemangioma piógeno. Conclusiones: el hemangioma o granuloma piógeno, es una tumoración benigna de inicio brusco y de curso rápido. La irritación local de los tejidos es la causa más frecuente y el tratamiento quirúrgico es la terapéutica adecuada para la curación de la enfermedad.

Background: pyogenic hemangioma or pyogenic granuloma includes neoplasms characterized by a large number of normal or abnormal blood vessels that are difficult to differentiate from malformations or hematomas. It is a soft tissue tumor, exceptionally in bone tissue, being relatively frequent, results from a focal reactive development of fibrovascular tissue with endothelial proliferation, and is associated with trauma, irritation, hormonal changes, pregnancy. Objective: to describe the infrequent location and the evolution of the disease. Clinical case: a white male patient had a history of a granulomatous lesion on the internal side of the swallow, for which reason it was previously incinerated and for rapid growth it was decided total exceresis and biopsy reporting the presence of a pyogenic hemangioma. Conclusions: pyogenic hemangioma or granuloma is a benign tumor of rapid onset and development. Local tissue irritation is the most common cause and surgical treatment is the appropriate therapy for the cure of the disease.

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We present an interesting case report of two sarcoid-like lesions on a port-wine stain (PWS) birthmark in a Brazilian patient which on investigation proved to be cutaneous leishmaniasis.

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Hemangioma é o tumor mais comum da infância, frequentemente situado na cabeça e pescoço. A órbita é frequentemente acometida e indica intervenção precoce e agressiva para evitar sérias complicações visuais. Reportam-se dois casos, nos quais há impedimento da visão no primeiro e, no segundo, um hemangioma profundo acomete áreas adjacentes, confirmado por exame radiológico. Demonstra-se sucesso terapêutico após corticoterapia sistêmica agressiva, evitando sequelas visuais permanentes, além do resultado estético satisfatório. O tratamento de escolha é o corticosteroide oral, devendo ser conduta individualizada e com bom seguimento clínico dos possíveis efeitos adversos.

Hemangioma is the most common tumor of childhood and is commonly located on the head or neck. The orbit is often affected and early and aggressive intervention is required to prevent serious visual complications. This paper reports on two cases. In the first case, the patient's vision was impaired, while in the second case a deep hemangioma affecting adjacent areas was confirmed radiologically. Treatment with aggressive systemic corticotherapy was successful, thus avoiding permanent damage to the patients' vision. Furthermore, esthetic outcome was satisfactory. The treatment of choice is oral corticosteroids and management should be individualized and should include careful follow-up to monitor possible adverse effects.

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Hemangiomas are the most common benign tumors of infancy. Despite their self-limited course, infantile capillary hemangiomas can impair vital or sensory functions as vision and cause cosmetic deformity. The usual treatments include oral/intralesional steroids, alpha interferon, cytotoxins, pulsed dye laser and cosmetic surgery resection. These treatments are not free of multiple complications and toxic side effects. This report describes the case of a 3-month-old female baby with progressively increasing hemangioma of the left upper eyelid impinging over the visual field. The hemangioma promptly responded to low-dose oral propranolol. A clinical response was noticed few days after the beginning of the treatment, with regression to 1/4 of its original size in 45 days of treatment, and to less than 1/10 after 8 months, free of any major side effects.

Hemangiomas são os tumores benignos mais comuns durante o primeiro ano de vida. Apesar do seu curso autolimitado, os hemangiomas capilares podem prejudicar funções vitais ou sensoriais como a visão e causar alteração estética. O tratamento usual inclui esteróides orais ou intralesionais, interferon alfa, citotoxinas, laser e ressecção cirúrgica. Entretanto estes tratamentos não estão livres de complicações e efeitos adversos. Este relato descreve o caso de um bebê feminino de 3 meses com um hemangioma rapidamente progressivo na pálpebra superior esquerda, causando obstrução no eixo visual. O hemangioma respondeu rapidamente a uma baixa dose oral de propranolol. A resposta clínica foi notada poucos dias após o início do tratamento, com regressão a 1/4 do seu tamanho original após 45 dias de tratamento, e a menos de 1/10 após 8 meses, sem ter apresentado nenhum efeito adverso.

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Los hemangiomas de tipo capilar son los tumores de la infancia más frecuentes, principalmente en niños menores de un año de edad y usualmente afectan la cabeza y el cuello. Por lo general, están solitarios; sin embargo, aproximadamente un 20% de los niños con grandes hemangiomas cervicofaciales tendrán una de las anomalías asociadas en el síndrome de PHACES, un raro síndrome neurocutáneo de predominio en el sexo femenino, con características mayores, como malformaciones cerebrales en la fosa posterior, hemangiomas de tipo capilar, anomalías arteriales, coartación de aorta, defectos cardiacos y anormalidades oculares. Cuando se asocia con hendidura esternal o rafe supraumbilical, es referido como síndrome de PHACES. Se presenta el caso de una niña de cuatro años de edad con hemangioma facial congénito asociado con anomalías vasculares cerebrales y de la fosa posterior.

Capillary hemangiomas of infancy are the most common childhood tumors, mainly in children under 1 year old, and they usually involve the head and neck. They are usually solitary, but about 20% of the children with large cervicofacial hemangiomas will have one of the anomalies associated with PHACES syndrome. PHACES is a rare neurocutaneous syndrome with female predominance and features such as: brain malformations in the posteriorfossa, hemangiomas, arterial anomalies, coarctation of the aorta, heart defects, and ocular abnormalities. When associated with sternal slit and/or supraumbilical Raphe, it is referredto as PHACES syndrome. The case of a 4-year-old child with congenital facial hemangioma associated to the posterior fossa and with cerebral vascular anomalies is presented.

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