Destructive Sinusopathy and Middle Ear Involvement in Behçet’s Syndrome

Martins, Henrique; Santos, Samir Cezimbra dos; Lourenço, Jorge; Araujo, Jesús A.

doi:10.1007/0-306-48382-3_96

Cited by 10 publications

(10 citation statements)

References 2 publications

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“…Martins et al 10 published in an unheard-of fashion a likely association between Behçet's Syndrome and rhinosinusopathy in a male patient (47 years old) that also developed chronic bilateral middle ear otitis and CT scan findings were similar to those of the patient of this study (predominantly maxilla-ethmoidal with destructive characteristics). They concluded, however, due to the inexistence of a reliable histological marker, that clear documentation of such association should be postponed.…”

Section: Discussionsupporting

confidence: 72%

“…The vasculitis found in Behçet's Syndrome is characterized by necrotizing inflammatory response of the micro vessels (preferably venules which have build up fragmented neutrophils and lymphocytes) and due to the fact that all lesions are in the same evolution stage and were classified in the group of "Immune Response Vasculitis" 10 . Poor perfusion resulting from spasmodic contraction of arterial vessel certainly is one of the major consequences of vasculitis that also causes deficient venous drainage 2 .…”

Section: Discussionmentioning

confidence: 99%

“…Since Behçet's Syndrome is rare, data about it are not significantly supported by literature both quantitatively and qualitatively in order to define, characterize and mainly relate it to Otorhinolaryngology 5 . Martins et al 10 reported the first case in 2003 with association between significant chance and development of rhinitis with destructive sinus disease with Behçet's syndrome.…”

Section: Introductionmentioning

confidence: 99%

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Rinossinusite em paciente com síndrome de Behçet

Alcântara

Júnior²,

Mocellin

et al. 2005

Rev. Bras. Otorrinolaringol.

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A condição conhecida como Síndrome de Behçet foi inicialmente descrita por H. Behçet, um dermatologista turco. O seu componente principal são lesões aftosas recorrentes em mucosa oral. Grupos como os asiáticos são mais propícios ao seu desenvolvimento. No continente americano, ela é relativamente rara. Além de lesões orais, os pacientes podem desenvolver ulcerações genitais, uveítes, vasculite pustular da pele, sinovites e meningoencefalites. O diagnóstico é baseado na pronunciação de critérios maiores e/ou menores internacionalmente propostos, e na combinação entre eles. O tratamento é desafiador, devendo dirigir-se ao principal órgão envolvido, exigindo terapias combinadas com freqüência. O quadro clínico do paciente deste caso não deixa dúvidas quanto ao diagnóstico da síndrome de Behçet. No presente trabalho, a vasculite é evidenciada pela ausência de sangramento ao manueseio de áreas potencialmente sangrantes. E a ela atribuiu-se tanto o desencadeamento quanto as complicações da rinossinusite (abscesso periorbitário), haja visto seus efeitos de má perfusão e drenagem no tecido em questão. A rinossinusite é uma manifestação potencial da síndrome de Behçet. Os profissionais médicos devem estar atentos à sua apresentação e prestar maiores cuidados para sua resolução, que é caracteristicamente difícil.

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Section: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Rinossinusite em paciente com síndrome de Behçet

Alcântara

Júnior²,

Mocellin

et al. 2005

Rev. Bras. Otorrinolaringol.

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“…La mise en évidence de signes de vascularite sur les premières biopsies a évoqué une manifestation rhino-sinusienne de la maladie de Behçet. En 2003, Martins et al ont rapporté le premier cas d'une association entre une rhinosinusite destructive et le syndrome de Behçet chez un patient de 47 ans [7]. En outre, Alcântara LJ et al ont rapporté un cas de rhinosinusite compliqué d'abcès périorbitaire qui serait lié à la vascularite nécrosante de la maladie de Behçet.…”

Section: Discussionunclassified

Un lymphome T/NK extra-ganglionnaire de type nasal se présentant comme une cellulite orbitaire

Amri¹,

Zalagh²,

Benariba³

2018

Pan Afr Med J

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Le lymphome extra-ganglionnaire T/NK de type nasal est une forme rare des lymphomes malins non hodgkinien. Elle pose un problème diagnostique à cause des symptômes peu spécifiques. Nous rapportons un cas de lymphome T/NK simulant une cellulite orbitaire. Il s'agit d'une patiente de 46 ans, suivie pour maladie de Behçet. Admise dans un tableau clinique de cellulite orbitaire. Le scanner du massif facial montrait une pansinusite avec infiltration orbitaire. En l'absence d'amélioration sous traitement antibiotique. Un traitement chirurgical avec biopsie a été réalisé. L'examen histologique a mis en évidence des signes de vascularite inflammatoire et la patiente a été mise sous corticothérapie avec amélioration clinique. Devant la récidive la patiente a bénéficié d'une reprise chirurgicale avec des biopsies de l'ethmoïde qui ont mis en évidence un lymphome T/NK de type nasal. Le traitement a consisté en une radio-chimiothérapie et la patiente est décédée dans les deux mois. Les lymphomes T/NK sont agressifs, ils touchent essentiellement les cavités naso-sinusiennes. Ils sont responsables d'une angio-destruction et de la nécrose qui rendent les symptômes peu spécifiques et les biopsies souvent négatives, posant un problème de diagnostic différentiel. Le traitement repose sur la radiothérapie et la chimiothérapie et le pronostic reste réservé.

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“…13 A 47-year-old man with rhinitis was found, on computed tomography (CT) scanning, to have destruction of the turbinates, a septal perforation and pansinusitis. The authors attributed this destructive sinusopathy to Behçet's disease, after excluding Wegener's granulomatosis.…”

Section: Paranasal Sinus Featuresmentioning

confidence: 98%

Ear, nose and throat manifestations of Behçet's disease: a review

2008

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Behçet's disease is usually considered to be a condition affecting the oral cavity, eyes and genitals. This article shows that most patients will also exhibit other ENT symptoms, hearing loss in particular. Indeed, Behçet's disease may present with features other than the classic triad of symptoms. Raised awareness of the clinical features within the head and neck region will hopefully enable early diagnosis and treatment of this potentially serious condition.

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Destructive Sinusopathy and Middle Ear Involvement in Behçet’s Syndrome

Cited by 10 publications

References 2 publications

Rinossinusite em paciente com síndrome de Behçet

Rinossinusite em paciente com síndrome de Behçet

Un lymphome T/NK extra-ganglionnaire de type nasal se présentant comme une cellulite orbitaire

Ear, nose and throat manifestations of Behçet's disease: a review

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