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1.
Rev. otorrinolaringol. cir. cabeza cuello ; 78(4): 426-430, dic. 2018. ilus
Article in Spanish | LILACS | ID: biblio-985750

ABSTRACT

RESUMEN La enfermedad de Rosai-Dorfman (ERD) o histiocitosis sinusal con linfadenopatía masiva es una enfermedad infrecuente, de etiología desconocida caracterizada por linfadenopatías cervicales masivas bilaterales. El compromiso extraganglionar puede ocurrir en diferentes sitios, incluida la vía aérea, en la cual la localización nasosinusal es la más frecuente, pero puede comprometer también otros sitios de la vía aérea superior. Dentro de éstos, el compromiso laríngeo es muy poco frecuente. Se presenta el caso de una paciente de 82 años con antecedentes de ERD localizada en los ganglios linfáticos cervicales en remisión espontánea, que desarrolló posteriormente una obstrucción subaguda de las vías respiratorias. La tomografía computarizada y la nasofaringolaringoscopía mostraron lesiones subglóticas que obstruían severamente las vías respiratorias. Se realizó una traqueostomía de emergencia y biopsia de las lesiones, confirmando el diagnóstico de ERD extranodal.


ABSTRACT Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease of unknown etiology characterized by bilateral massive cervical lymphadenopathy. Extranodal involvement can occur in different sites including airway where nasosinusal involvement is common but this disease may occur in other sites of the upper airway. Laryngeal involvement is rare. We present the case of an 82-year-old female patient with a history of Rosai-Dorfman disease located in cervical lymph nodes in spontaneous remission that subsequently develops airway obstruction. Computed tomographic scan and nasopharyngolaryngoscopy showed subglottic lessons that severely obstructed the airway. Emergency tracheotomy and biopsy were performed, confirming the diagnosis of extranodal RDD. Patient was observed and tracheostomy was maintained with good tolerance.


Subject(s)
Humans , Female , Aged, 80 and over , Histiocytosis, Sinus/diagnosis , Laryngeal Diseases/diagnosis , Airway Obstruction/surgery , Tracheostomy , Tomography, X-Ray Computed , Histiocytosis, Sinus/surgery , Laryngeal Diseases/surgery , Laryngostenosis
2.
Rev. chil. cir ; 67(1): 65-69, feb. 2015. ilus
Article in Spanish | LILACS | ID: lil-734741

ABSTRACT

Background: Rosai Dorfman disease is uncommon and consists in a benign fibrous and hematopoietic tissue proliferation, usually located in the head or neck. It is self-limited and the treatment is the surgical excision of the lesion. Case Report: We report a 69 years old woman consulting for a palpable right breast nodule with a mammography and mammary ultrasound informed as BIRADS 4 and 5, respectively. A core biopsy was informed as a mammary Rosai Dorfman disease. Two years later, due to a lesion growth, the patient was subjected to a partial mastectomy. The pathological study of the surgical piece confirmed the diagnosis of the core biopsy. This disease may resemble a malignant disease and the clue for its diagnosis is the pathological study.


Introducción: La enfermedad de Rosai Dorfman es una entidad infrecuente, que consiste en la proliferación benigna del tejido fibroso y hematopoyético ubicado generalmente en cabeza y cuello. Se trata de una enfermedad benigna, autolimitada, cuyo tratamiento generalmente se limita a la resección quirúrgica, con elevada tasa de recidiva. Objetivo: Exposición de un caso de Enfermedad de Rosai Dorfman mamario, inicial-mente sugerente de patología maligna. caso clínico: Mujer 69 años derivada de atención primaria por nódulo palpable en mama derecha, mamografía y ecografía mamaria BIRADS 4 y 5, respectivamente. Biopsia Core informa Enfermedad de Rosai Dorfman mamaria. A los dos años de diagnóstico se objetiva lesión de mayor tamaño. Se realizó mastectomía parcial con biopsia de pieza quirúrgica compatible con diagnóstico de Biopsia Core previa. Discusión: La similitud clínica de esta enfermedad con patologías malignas mamarias, asociado a su baja prevalencia, dificultan la pesquisa de casos como el expuesto. En los estudios de imágenes raramente se sospecha. La clave del diagnóstico son los hallazgos histológicos en el que característicamente se observa emperipolesis con tinciones inmunohistoquímicas positivas para S 100 y negativo para CD 1. Dado que es una enfermedad benigna y autolimitada su tratamiento generalmente se limita a la resección quirúrgica. Este permite extirpar el tumor en estudio, realizar el diagnóstico, y aliviar la sintomatología producida por el efecto masa de la lesión. A pesar de lo anterior la recidiva es alta, por lo que el seguimiento debe ser a largo plazo.


Subject(s)
Humans , Female , Aged , Breast Diseases/surgery , Breast Diseases/diagnosis , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/diagnosis , Diagnosis, Differential , Mastectomy, Segmental , Breast Neoplasms/diagnosis
3.
An. bras. dermatol ; 89(1): 171-172, Jan-Feb/2014. graf
Article in English | LILACS | ID: lil-703529

ABSTRACT

Cutaneous Rosai-Dorfman disease is a rare, lymphoproliferative disease. It is benign and self-limited, only involves skin and subcutaneous tissue and typically occurs as histiocyte-rich inflammatory infiltrates, manifesting as erythematous to brown papules, plaques, or nodules, without predilection for site. The authors describe a case of cutaneous Rosai-Dorfman disease in a 72-year-old man who presented erythematous and scaled plaque on the right neck for three months without systemic symptoms. Owing to local involvement, the patient received a surgery to exsect the lesion completely and remained asymptomatic with no signs of recurrence at the 9-month follow up.


Subject(s)
Humans , Male , Aged , Skin Diseases/pathology , Histiocytosis, Sinus/pathology , Skin/pathology , Skin Diseases/surgery , Histiocytosis, Sinus/surgery , Neck/pathology
4.
Pakistan Journal of Medical Sciences. 2013; 29 (4): 1065-1067
in English | IMEMR | ID: emr-130378

ABSTRACT

Rosai-Dorfman disease [RDD] is rare and characterized by histiocytic proliferation and massive cervical lymphadenopathy. About 40% of patients have extra-nodal involvement. Ophthalmic involvement is seen in 10% of cases. A case of orbital Rosai Dorfman disease in a 58 years old woman is presented here, who was misdiagnosed as orbital inflammatory disease initially. The patient did not respond to a course of oral prednisolone. Then complete surgical excision of the mass was performed and the histopathological examination was consistent with a diagnosis of RDD


Subject(s)
Humans , Female , Histiocytosis, Sinus/therapy , Histiocytosis, Sinus/surgery , Prednisolone , Orbital Diseases
5.
An. bras. dermatol ; 85(5): 687-690, set.-out. 2010. ilus, tab
Article in Portuguese | LILACS | ID: lil-567831

ABSTRACT

A doença de Rosai-Dorfman, também denominada histiocitose sinusal com linfadenopatia maciça, é histiocitose de células não Langerhans, idiopática e de curso benigno. Descrita em 1969, caracteriza-se por linfadenomegalia não dolorosa, sendo a cadeia cervical a mais envolvida, além de febre, perda de peso e sudorese. O envolvimento extranodal ocorre em 43 por cento dos casos, em que múltiplos sítios podem ser acometidos. Já foram descritos casos exclusivamente extranodais, inclusive formas limitadas à pele. Relata-se um caso de doença de Rosai-Dorfman extranodal cutânea pura, devido à raridade dessa apresentação clínica.


Rosai-Dorfman disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a non-Langerhans cell histiocytosis with a benign course and unknown etiology. It was described in 1969 as a painless cervical lymph node enlargement in association with fever, weight loss and sweating. Extranodal disease has been reported in 43 percent of cases, with involvement of multiple organs. Purely extranodal Rosai-Dorfman disease has been already reported, including forms restricted to the skin. This paper reports a case of purely cutaneous Rosai-Dorfman disease, which is of interest in view of the rarity of this condition.


Subject(s)
Adult , Female , Humans , Histiocytosis, Sinus/pathology , Biopsy , Histiocytosis, Sinus/surgery , Immunohistochemistry , Skin/pathology
7.
An. bras. dermatol ; 84(3): 275-278, jul. 2009. ilus
Article in English, Portuguese | LILACS | ID: lil-521753

ABSTRACT

Doença de Rosai-Dorfman é uma doença benigna e autolimitada. Tem etiologia desconhecida e foi descrita pela primeira vez por Rosai e Dorfman em 1969. As manifestações clássicas incluem linfadenopatia cervical usualmente acompanhada por febre, leucocitose com neutrofilia e hipergamaglobulinemia policlonal. Apesar de a pele ser o principal sítio extranodal da doença, sua manifestação restrita à pele costuma ser bastante rara com poucos casos descritos na literatura. O trabalho tem como objetivo relatar um caso de Doença de Rosai-Dorfman cutânea com revisão da literatura onde são abordados os diferentes aspectos do diagnóstico e do tratamento dessa doença.


Rosai-Dorfman disease is a self-limited benign disease. Rosai and Dorfman first described it in 1969, and the etiology of the disease remains unknown. Main manifestations are cervical adenopathy associated with fever, leukocytosis with neutrophilia and polyclonal gamaglobulinemia. Although the skin is the most common site of extra nodal disease, Rosai-Dorfman disease restricted to the skin is very rare with only a few cases described in the literature. This paper reports a rare case of cutaneous Rosai-Dorfman with skin as the sole site, and reviews the controversies of diagnosis and treatment.


Subject(s)
Adult , Humans , Male , Histiocytosis, Sinus , Histiocytosis, Sinus/pathology , Histiocytosis, Sinus/surgery , Skin Diseases/pathology , Skin Diseases/surgery
8.
Rev. cuba. estomatol ; 45(1)ene.-mar 2008. ilus
Article in Spanish | LILACS, CUMED | ID: lil-507111

ABSTRACT

La enfermedad de Rosai Dorfman es un desorden multisistémico de etiología y patogénesis desconocidas, pero se piensa que representa un proceso reactivo histiocítico a un agente infectante en lugar de una neoplasia u otra condición primaria. Se han informado en la literatura menos de 1 000 casos que han estado asociados con disímiles enfermedades. Se hace referencia de un caso clínico atendido en la República Democrático Federal de Etiopía en el año 2003 con compromiso panglandular y diagnósticos previos inciertos hasta su conclusión definitiva(AU)


Rosai-Dorfman syndrome is a multisystemic disorder of unknown aetiology and pathogenesis, but it is thought that it represents a reactive histiocytic process to an infective agent instead of a neoplasia or other primary condition. Less than 1 000 cases associated with different diseases have been reported in literature. Reference is made to a clinical case cared for in the Federal Democratic Republic of Ethiopia in 2003 with panglandular compromise and uncertain previous diagnoses until its definitive conclusionRosai-Dorfman syndrome is a multisystemic disorder of unknown aetiology and pathogenesis, but it is thought that it represents a reactive histiocytic process to an infective agent instead of a neoplasia or other primary condition. Less than 1 000 cases associated with different diseases have been reported in literature. Reference is made to a clinical case cared for in the Federal Democratic Republic of Ethiopia in 2003 with panglandular compromise and uncertain previous diagnoses until its definitive conclusion(AU)


Subject(s)
Humans , Male , Adolescent , Salivary Glands/injuries , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/diagnosis , Review Literature as Topic
9.
Rev. cuba. oftalmol ; 10(1/2): 5-11, ene.-dic. 1997. ilus, graf
Article in Spanish | LILACS | ID: lil-217726

ABSTRACT

En esta comunicación se presenta el estudio clinicopatológico de un caso con afectación masiva de ambas órbitas como única manifestación de la enfermedad de Rosai-Dorfman. En el examen clínico se constató una exoftalmía maligna bilateral, en la TAC realizada se pudo apreciar la ocupación de las órbitas por múltiples masas tumorales. En el estudio histológico de esta tumoraciones se comprobó una marcada fibrosis y proliferación de los histocitos, varios de ellos cargados de núcleos linfoides sin desintegrarse, lo que constituye la emperipolesis; cuadro histológico que corresponde a la enfermedad de Rosai-Drofman. En este paciente se realizó un tratamiento quirúrgico por vía anterior para eliminar las masas tumorales de las órbitas. Actualmente recibe tratamiento con quimioterapia, esteroides y radioterapia externa. Es de interés señalar que éste es el primer caso reportado en Cuba con las características antes descritas


Subject(s)
Humans , Male , Middle Aged , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/therapy , Cuba
10.
P. R. health sci. j ; 14(2): 145-9, jun. 1995.
Article in English | LILACS | ID: lil-176822

ABSTRACT

We present the case of a 5-year-old boy who was referred to the Neurosurgical Service of the San Jorge Children's Hospital for evaluation of right eye proptosis. At the age of one year the diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) had been rendered on a cervical lymph node biopsy. The right orbit mass causing the proptosis was the result of extranodal involvement of the orbital tissue by SHML. The clinical and pathologic features of this entity are discussed


Subject(s)
Child, Preschool , Humans , Male , Orbital Diseases/diagnosis , Histiocytosis, Sinus/diagnosis , Biopsy , Diagnosis, Differential , Orbital Diseases/pathology , Orbital Diseases/surgery , Lymph Nodes/pathology , Histiocytosis, Sinus/pathology , Histiocytosis, Sinus/surgery , Neck , Orbit/pathology , Puerto Rico , United States Virgin Islands/ethnology
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