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7.
Clin Lab ; 62(1-2): 241-3, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27012056

RESUMO

BACKGROUND: Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by vascular malformations mostly involving skin and gastrointestinal tract. This disease is often associated with sideropenic anemia and occult bleeding. METHODS: We report the case of chronic severe anemia in an old patient under oral anticoagulation treatment for chronic atrial fibrillation. RESULTS: At admission, the patient also presented fever and increased laboratory parameters of systemic inflammation (ferritin 308 mcg/L, C-reactive protein (CRP) 244 mg/L). A small bluish-colored lesion over the left ear lobe was observed. Fecal occult blood test was negative as well as other signs of active bleeding. Lower gastrointestinal endoscopy revealed internal hemorrhoids and multiple teleangiectasias that were treated with argon plasma coagulation. Videocapsule endoscopy demonstrated multiple bluish nodular lesions in the small intestine. Unexpectedly, chronic severe anemia due to systemic inflammation was diagnosed in an old anticoagulated patient with BRNBS. The patient was treated with blood transfusions, hydration, antibiotic treatment, and long-acting octreotide acetate, without stopping warfarin. Fever and inflammation disappeared without any acute gastrointestinal bleeding and improvement of hemoglobin levels at three-month follow up. CONCLUSIONS: This is the oldest patient presenting with chronic anemia, in which BRNBS was also diagnosed. Surprisingly, anemia was mainly caused by systemic inflammation instead of chronic gastrointestinal bleeding. However, we would recommend investigating this disease also in old subjects with mild signs and symptoms.


Assuntos
Anemia/etiologia , Anticoagulantes/uso terapêutico , Fibrilação Atrial/tratamento farmacológico , Neoplasias Gastrointestinais/complicações , Inflamação/etiologia , Nevo Azul/complicações , Doença Pulmonar Obstrutiva Crônica/complicações , Neoplasias Cutâneas/complicações , Idoso de 80 Anos ou mais , Anemia/sangue , Anemia/diagnóstico , Anemia/terapia , Fibrilação Atrial/complicações , Fibrilação Atrial/diagnóstico , Doença Crônica , Progressão da Doença , Endoscopia Gastrointestinal , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/terapia , Humanos , Inflamação/diagnóstico , Inflamação/terapia , Masculino , Nevo Azul/diagnóstico , Nevo Azul/terapia , Doença Pulmonar Obstrutiva Crônica/diagnóstico , Doença Pulmonar Obstrutiva Crônica/terapia , Fatores de Risco , Índice de Gravidade de Doença , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Resultado do Tratamento
9.
Pomeranian J Life Sci ; 62(2): 5-7, 2016.
Artigo em Polonês | MEDLINE | ID: mdl-29537205

RESUMO

The blue rubber bleb nevus syndrome or Bean syndrome is a rare disorder characterised by the presence of haemangiomas in the skin and gastrointestinal tract. These lesions are usually accompanied by chronic hypochromic anaemia resulting from gastrointestinal bleeding. The cause of this syndrome is unknown. It mostly occurs as sporadic, but autosomal dominant inheritance has also been described. The diagnosis is based on endoscopy, magnetic resonance imaging and computer tomography. Treatment is usually symptomatic.


Assuntos
Neoplasias Gastrointestinais/patologia , Nevo Azul/patologia , Neoplasias Cutâneas/patologia , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/terapia , Hemangioma , Humanos , Nevo Azul/diagnóstico , Nevo Azul/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia
14.
World J Gastroenterol ; 20(45): 17254-9, 2014 Dec 07.
Artigo em Inglês | MEDLINE | ID: mdl-25493043

RESUMO

Blue rubber bleb nevus syndrome (BRBNS) is a rare disease characterized by multiple venous malformations and hemangiomas in the skin and visceral organs. The lesions often involve the cutaneous and gastrointestinal systems. Other organs can also be involved, such as the central nervous system, liver, and muscles. The most common symptoms are gastrointestinal bleeding and secondary iron deficiency anemia. The syndrome may also present with severe complications such as rupture, intestinal torsion, and intussusception, and can even cause death. Cutaneous malformations are usually asymptomatic and do not require treatment. The treatment of gastrointestinal lesions is determined by the extent of intestinal involvement and severity of the disease. Most patients respond to supportive therapy, such as iron supplementation and blood transfusion. For more significant hemorrhages or severe complications, surgical resection, endoscopic sclerosis, and laser photocoagulation have been proposed. Here we present a case of BRBNS in a 45-year-old woman involving 16 sites including the scalp, eyelid, orbit, lip, tongue, face, back, upper and lower limbs, buttocks, root of neck, clavicle area, superior mediastinum, glottis, esophagus, colon, and anus, with secondary severe anemia. In addition, we summarize the epidemiology, clinical manifestations, diagnosis, differential diagnosis and therapies of this disease by analyzing all previously reported cases to enhance the awareness of this syndrome.


Assuntos
Neoplasias Gastrointestinais , Nevo Azul , Neoplasias Cutâneas , Anemia Ferropriva/diagnóstico , Anemia Ferropriva/epidemiologia , Anemia Ferropriva/terapia , Transfusão de Sangue , Diagnóstico Diferencial , Endoscopia Gastrointestinal , Feminino , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/epidemiologia , Hemorragia Gastrointestinal/terapia , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/epidemiologia , Neoplasias Gastrointestinais/terapia , Hematínicos/uso terapêutico , Humanos , Ferro/uso terapêutico , Pessoa de Meia-Idade , Nevo Azul/diagnóstico , Nevo Azul/epidemiologia , Nevo Azul/terapia , Valor Preditivo dos Testes , Prognóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/epidemiologia , Neoplasias Cutâneas/terapia , Tomografia Computadorizada por Raios X
15.
Gen Dent ; 62(5): e22-6, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25184728

RESUMO

Melanocytic nevi are the most common benign proliferations of melanin-producing cells in Caucasians; up to 30 lesions can be seen in an adult individual. Lesional cells are usually superficially located in the epidermis-superficial dermis. Blue nevi are less common. They represent benign proliferations of fusiform dermal melanocytes in the submucosa or deep dermis. These cells contain abundant melanin granules. The blue color is due to the deep location of the lesional cells and the Tyndall effect. In the oral cavity, both melanocytic and blue nevi are rare; the hard palate being the site of predilection. The risk of malignant transformation of blue nevi is unknown; this is mainly due to the rarity of reported oral lesions. Therefore, the reporting of such cases is key to a better understanding and possibly predicting the clinical behavior of intraoral blue nevi. This article presents 2 patients with blue nevi in the oral cavity, and reviews the differential diagnosis of solitary pigmented lesions, including oral melanoma. The importance of proper diagnosis and treatment of oral pigmentations in general, and of melanocytic nevi in particular, is underlined in order to help the general dentist manage patients presenting with such lesions.


Assuntos
Doenças da Boca/diagnóstico , Nevo Azul/diagnóstico , Adulto , Diagnóstico Diferencial , Humanos , Masculino , Doenças da Boca/terapia , Nevo Azul/terapia
16.
Mod Pathol ; 27(11): 1468-78, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24743221

RESUMO

Melanomas arising in association with blue nevi or mimicking cellular blue nevi comprise a relatively rare and heterogeneous group of melanomas. It remains controversial which prognostic indicators predictive of outcome in conventional cutaneous melanomas are applicable to this type of melanoma. Here, we describe the clinical and histopathologic features of 24 melanomas arising in association with blue nevi and correlate these with clinical outcome. The mean patient age was 49 years (range: 23-85) with a slight female predominance (15 females:9 males). The most common anatomic locations included the head and neck region (50%), the trunk (21%), and the buttock/sacrococcygeum (17%). Histologically, the tumors were typically situated in the mid to deep dermis with variable involvement of the subcutis, but uniformly lacked a prominent intraepithelial component. The mean tumor thickness (defined as either the standard Breslow thickness or, if not available due to the lack of orientation or lack of epidermis, the largest tumor dimension) was 20.9 mm (range: 0.6-130 mm). The mean mitotic figure count was 6.5/mm(2) (range: 1-30/mm(2)). Perineural invasion was common (38%). Follow-up was available for 21 cases (median 2.1 years). The median overall survival, recurrence-free survival, time to local recurrence, and time to distant recurrence were 5.2, 0.7, 2.6, and 1.6 years, respectively. Logistic regression analyses demonstrated a significant association between tumor thickness and recurrence-free survival (hazard ratio=1.02 per mm; P=0.04) and reduced time to distant metastasis (hazard ratio=1.03 per mm; P=0.02) with a similar trend toward reduced time to local recurrence (hazard ratio=1.02 per mm; P=0.07). No other parameters (age, anatomic location, mitotic figures, lymphovascular or perineural invasion, or type of associated blue nevus) emerged as significant. In addition, we provide a comprehensive review of 109 cases of melanoma blue nevus type described in the English literature and summarize our findings in this context.


Assuntos
Melanoma/patologia , Nevo Azul/patologia , Neoplasias Cutâneas/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biópsia , Intervalo Livre de Doença , Feminino , Humanos , Modelos Logísticos , Masculino , Melanoma/genética , Melanoma/mortalidade , Melanoma/secundário , Melanoma/terapia , Pessoa de Meia-Idade , Mutação , Invasividade Neoplásica , Recidiva Local de Neoplasia , Neurônios/patologia , Nevo Azul/genética , Nevo Azul/mortalidade , Nevo Azul/secundário , Nevo Azul/terapia , Valor Preditivo dos Testes , Modelos de Riscos Proporcionais , Proteínas Proto-Oncogênicas B-raf/genética , Fatores de Risco , Neoplasias Cutâneas/genética , Neoplasias Cutâneas/mortalidade , Neoplasias Cutâneas/terapia , Fatores de Tempo , Carga Tumoral , Adulto Jovem
17.
Pract Neurol ; 14(5): 360-2, 2014 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-24614007

RESUMO

A 62-year-old woman presented with stabbing pain over her left temple radiating to her left cheek when bending forwards or coughing. Neurological examination was normal. There were many cutaneous venous prominences over her body. CT and MR brain scans showed multiple venous anomalies and venous occlusive disease of the left sylvian fissure and superior sagittal sinus. We excluded arteriovenous malformation and dural fistulae with cerebral angiography. Following a clinical genetics assessment, we diagnosed blue rubber bleb naevus syndrome (BRBNS) and gave amitriptyline for her pain. There are only 200 cases of BRBNS in the literature, and central nervous system involvement is rarer still. The syndrome involves multiple cutaneous and visceral venous malformations. Most appear to be sporadic though a few have autosomal dominant inheritance. Although rare, BRBNS represents an important differential diagnosis for patients presenting with multiple and/or multisystem vascular malformations.


Assuntos
Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Neurologia , Nevo Azul/patologia , Nevo Azul/terapia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Sistema Nervoso Central/patologia , Feminino , Hemangioma Cavernoso/patologia , Humanos , Angiografia por Ressonância Magnética , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Tomógrafos Computadorizados
19.
Pol Merkur Lekarski ; 33(196): 226-8, 2012 Oct.
Artigo em Polonês | MEDLINE | ID: mdl-23272612

RESUMO

Blue rubber bleb nevus syndrome (Bean syndrome) is a rare disease characterized by the presence of multiple vascular malformation of rubber-like consistence. This disease is of a genetic origin and most often is caused by sporadic mutation, however, exist reports on autosomal dominant type of heritance. Nevi are most frequently met in the skin and alimentary tract but may be present in all organs and tissues. The most frequent symptom of Bean syndrome is anaemia due to ferrum deficiency, which is a result of chronic hemorrhagia from vascular malformations in the alimentary tract. Vascular anomalies on the skin are usually asymptomatic. Other symptoms are less frequent and depend on the localisation of vascular changes and therefore patients with Bean syndrome require meticulous analysis of reported multiple - specialistic medical care.


Assuntos
Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/terapia , Nevo Azul/diagnóstico , Nevo Azul/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/terapia , Neoplasias Gastrointestinais/genética , Humanos , Nevo Azul/genética , Neoplasias Cutâneas/genética
20.
Ann Vasc Surg ; 24(8): 1136.e1-5, 2010 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21035709

RESUMO

We present the case of a 9-year-old boy diagnosed with blue rubber bleb nevus syndrome, who showed a very large left cervical cystic lymphangioma. He was previously subjected to various treatments for lesions in the intestinal tract including blood transfusions for anemia, sclerosis, enterotomies or resections. The tumor was resected without any complications and the anatomopathologic report confirmed this diagnosis. The blue cavernous hemangioma syndrome (or blue rubber bleb nevus syndrome) is a rare disease characterized by cavernous angiomas involving the skin and gastrointestinal tract. Several cases of cystic lymphangiomas associated with this syndrome have been published recently and lymphomatous differentiation has been identified in the cells of cutaneous lesions. Given their common embryological origin, we underscore the importance of bearing in mind that it is possible for different types of vascular malformations to coexist in the same patient.


Assuntos
Neoplasias de Cabeça e Pescoço/complicações , Linfangioma Cístico/complicações , Criança , Neoplasias Gastrointestinais/complicações , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Neoplasias de Cabeça e Pescoço/patologia , Neoplasias de Cabeça e Pescoço/cirurgia , Humanos , Linfangioma Cístico/patologia , Linfangioma Cístico/cirurgia , Imagem por Ressonância Magnética , Masculino , Nevo Azul/complicações , Nevo Azul/patologia , Nevo Azul/terapia , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Resultado do Tratamento
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