Rosai-Dorfman disease of the oral cavity.

First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.

Enfermedad de Rosai-Dorfman intracraneal: un infrecuente diagnóstico diferencial del meningioma. Reporte de un caso ilustrativo

La Enfermedad de Rosai-Dorfman es un trastorno infrecuente y de etiología desconocida. La presentación intracraneal es aún más rara y suele imitar la apariencia de un meningioma en las imágenes del encéfalo. Se presenta el caso de un paciente varón de 38 años que ingresa por una tumoración intracraneal, extra-axial y supratentorial asociada a déficit neurológico leve; con diagnóstico presuntivo de meningioma de la convexidad. Después de la intervención quirúrgica, el diagnóstico histológico definitivo fue de Enfermedad de Rosai-Dorfman intracraneal. Se debe considerar la Enfermedad de Rosai-Dorfman intracraneal dentro del diagnóstico diferencial de una lesión intracraneal extra-axial sugestiva de un meningioma debido a la similitud en las neuroimágenes y la presentación clínica entre ambas patologías.

Rosai-Dorfman disease is an infrequent condition with no known etiology. The intracranial presentation is even rarer, and it resembles the appearance of a meningioma in image studies. We present the case of a 38-year-old male patient who was admitted because of an extra-axial supratentorial cranial tumor, associated to mild neurological deficit. The presumptive diagnosis was convexity meningioma. After surgery, the definitive histologic diagnosis was intracranial Rosai-Dorfman disease. This condition should be considered in the differential diagnosis of an extra-axial intracranial lesion suggesting meningioma, because of similar neuroimaging results and the similar clinical picture.

Rosai-Dorfman disease of the oral cavity

ABSTRACT First described by J Rosai and R F Dorfman in 1969, Rosai-Dorfman disease (RDD) is a benign, self-limiting histiocytosis of unknown etiology. It is usually seen in the first two decades of life. The most frequent clinical presentation is painless, bilateral cervical lymphadenopathy accompanied by fever, weight loss, and an elevated ESR. However, RDD without nodal involvement is extremely rare, and the most common extranodal location is the head and neck region, mainly affecting the nasal cavity, pharynx, and paranasal sinuses. Oral location of RDD is occasional; according to our knowledge, only 17 cases of oral Rosai-Dorfman disease without lymph node involvement have been found in the literature. Because of the rarity of these isolated oral presentations, the clinical and radiological aspects need to be more studied. This article aims to present a rare case of oral Rosai-Dorfman disease without nodal involvement, detail the clinical and radiological signs, and the treatment strategy used in our patient.

Rosai-dorfman disease: a rare presentation of extranodal involvement of isolated bone. case report

ABSTRACT Introduction: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis, is a rare disorder characterized by histiocyte proliferation. Case presentation: A 33-year-old man consulted the emergency department of a tertiary care institution in Cartagena de Indias, Colombia, due to a 6-month history of progressive deformity in the frontal right side of the face, associated with pain of slow progression, without any other symptoms or dermatological involvement. There were no other major findings on physical examination and laboratory tests performed were negative. Imaging scans obtained showed extensive inflammatory involvement of the frontal bone, which led to suspect osteomyelitis as the first diagnostic possibility. A biopsy of the lesion was performed with negative cultures for bacteria, which allowed establishing a diagnosis of extranodal Rosai-Dorfman disease with isolated bone involvement. Treatment with systemic corticosteroids was indicated with poor response, so methotrexate was added, achieving an evident improvement after 2 months. Conclusions: Little is known about the manifestations of Rosai-Dorfman disease and its treatment in the adult population. The present case report contributes to expanding the literature on this topic, which can present with rare symptoms that may pose challenges for its diagnosis.

RESUMEN Introducción. La enfermedad de Rosai-Dorfman, también conocida como histiocitosis sinusal, es un trastorno poco frecuente caracterizado por la proliferación de histiocitos. Presentación del caso. Hombre de 33 años quien consultó al servicio de urgencias de una institución de tercer nivel de Cartagena de Indias, Colombia, por una deformidad de progresión lenta que apareció 6 meses atrás en el costado frontal derecho de la cara y generaba dolor. Al examen físico no hubo hallazgos mayores y los estudios paraclínicos fueron normales o negativos. Se obtuvieron imágenes que evidenciaron extenso compromiso inflamatorio/infeccioso del hueso frontal, por lo que se sospechó de osteomielitis y se realizó biopsia de la lesión que permitió establecer diagnóstico de enfermedad de Rosai-Dorfman variante extranodal con compromiso óseo aislado. El paciente recibió tratamiento con corticoide sistémico sin obtener respuesta, por lo que se adicionó metotrexato, con lo cual se logró mejoría a los 2 meses. Conclusiones. Las manifestaciones de la enfermedad de Rosai-Dorfman en población adulta son poco conocidas, además no hay mucha información sobre su tratamiento, por lo que el presente caso contribuye a ampliar la literatura sobre esta enfermedad que puede presentarse con sintomatología completamente inusual.

Enfermedad de Rosai-Dorfman primaria ósea

La enfermedad de Rosai-Dorfman es una patología poco común que suele presentarse con adenopatías cervicales indoloras. El compromiso óseo como presentación extraganglionar es aún menos frecuente. Se presenta el caso de una mujer de 38 años con 3 meses de dolor y limitación funcional en miembro inferior derecho. La radiografía simple y tomografía computarizada mostraron la presencia de una lesión ósea lítica en la metáfisis y epífisis distal del fémur derecho. Finalmente, el estudio histopatológico e inmunohistoquímico de la lesión determinó que se trataba de un caso de enfermedad de Rosai-Dorfman primaria ósea.

Rosai-Dorfman disease is a rare pathology that usually presents as painless cervical adenopathies. Bone compromise as an extra nodal presentation is even more uncommon. We present the case of a 38 years old woman with a 3 months history of pain and functional limitation of her right lower limb. X-ray and CT images showed a lytic bone lession at the distal metaphysis and epiphysis on her right femur. Histological and immunohistochemical study of the lession determined that it was a case of Rosai-Dorfman disease with bone involvement.

Enfermedad de Rosai-Dorfman (ERD) con compromiso exclusivo del sistema nervioso central. Presentación de caso y revisión de literatura / Rosai-Dorfman Disease (RDD) With Isolated Compromise of the Central Nervous System. Case Report and Literature Review

La enfermedad de Rosai-Dorfman (ERD) o histiocitosis sinusal con linfadenopatía masiva (HSLM) es una entidad histiocítica benigna rara, que usualmente afecta los ganglios linfáticos. Se han descrito algunos casos en el sistema nervioso central, y son excepcionales los que aparecen sin afección nodular concomitante. La falta de patrones imaginológicos cerebrales típicos puede llevar a una cirugía por un diagnóstico erróneo, con causas atribuidas a un probable origen maligno. Usualmente, el diagnóstico histopatológico se realiza después del procedimiento quirúrgico. Esta entidad clínico-patológica carece de consenso en diagnóstico, curso clínico y tratamiento. Se presenta un caso de ERD con extensión intracraneal, con la lectura retrospectiva de los estudios imaginológicos.

Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a rare benign histiocytic disorder, usually affecting the lymph nodes. Intracranial involvement is an uncommon variant of the disease; however, intracranial lesions without concomitant nodular involvement is exceptional. The lack of typical brain imaging patterns can lead to surgery due to misdiagnosis, with causes attributed to a probable malignant origin. Histopathological diagnosis is usually made after the surgical procedure. There is no consensus related to diagnosis, clinical course and treatment of this disease. A case report is presented on isolated intracranial RDD, with a retrospective reading of the imaging studies.

Solitary extranodal Rosai-Dorfman disease of the mandible: an exceedingly rare presentation

Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.

Solitary extranodal Rosai-Dorfman disease of the mandible: an exceedingly rare presentation.

Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.

Generalized erythematous and scaly plaques and papules: a rare case of Rosai-Dorfman disease accompanied by multiple myeloma

Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.

Histological variability and the importance of clinicopathological correlation in cutaneous Rosai-Dorfman disease

Abstract: Rosai-Dorfman disease is a benign histiocytic proliferative disorder of unknown etiology. The disease mainly affects lymph node tissue, although it is rarely confined to the skin. Here, we describe a 53-year-old woman with purely cutaneous Rosai-Dorfman disease. The patient presented with a large pigmented plaque on her left leg, and sparse erythematous papules on her face and arms. A complete clinical response was achieved with thalidomide, followed by recurrence at the initial site one year later. The histological examination displayed the typical features of Rosai-Dorfman disease in the recent lesions but not in the older lesions. In the setting of no lymphadenopathy, the histopathological features of Rosai-Dorfman disease are commonly misinterpreted. Therefore, awareness of the histological aspects present at different stages, not always featuring the hallmark microscopic signs of Rosai-Dorfman disease, is particularly important for a correct diagnosis of this rare disorder.

Rosai-Dorfman disease affecting the maxilla.

Rosai-Dorfman disease (RDD), formerly called sinus histiocytosis with massive lymphadenopathy, is a non-neoplastic proliferative histiocytic disorder with behavior ranging from highly aggressive to spontaneous remission. Although the lymph nodes are more commonly involved, any organ can be affected. This study aimed to describe the features and the follow-up of a case of extranodal RDD. Our patient was a 39-year-old woman who was referred with an 11-month history of pain in the right maxilla. On clinical examination, some upper right teeth presented full mobility with normal appearance of the surrounding gingiva. Radiographic exams showed an extensive bone reabsorption and maxillary sinus filled with homogeneous tissue, which sometimes showed polypoid formation. An incisional biopsy demonstrated a diffuse inflammatory infiltrate rich in foamy histiocytes displaying lymphocytes emperipolesis. Immunohistochemistry showed positivity for CD68 and S-100, and negativity for CD3, CD20, and CD30. Such features were consistent with the RDD diagnosis. The patient was referred to a hematologist and corticotherapy was administrated for 6 months. RDD is an uncommon disease that rarely affects the maxilla. In the present case, the treatment was conservative, and the patient is currently asymptomatic after 5 years of follow-up.

Case for diagnosis

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.

Rosai-Dorfman disease presenting with extensive cutaneous manifestation - Case report / Doença de Rosai-Dorfman com manifestação cutânea extensa - Relato de caso

Rosai-Dorfman disease is a benign, self-limited, idiopathic proliferative histiocytic disorder. It was first described in 1969 by Rosai and Dorfman. In its typical form the disease is characterized by extensive cervical lymphadenopathy associated with fever, polyclonal gammopathy and leukocytosis with neutrophilia. The skin is the most common site affected. Extranodal manifestations have been reported in 43% of cases. In this study, we report an atypical case of Rosai-Dorfman disease in a female with massive cutaneous manifestation on the thigh, associated with a minimal lymphadenopathy limited to the regional inguinal lymph nodes.

A doença de Rosai-Dorfman é uma desordem benigna, autolimitada, idiopática, decorrente da proliferação de histiócitos. Foi descrita pela primeira vez por Rosai e Dorfman em 1969. Tipicamente, a doença se caracteriza por extensa linfadenopatia cervical, febre, gamopatia monoclonal e leucocitose com neutrofilia. Manifestações extranodais são encontradas em 43% dos casos, sendo a pele o sítio extranodal mais comumente afetado. No trabalho, apresentamos um caso de doença de Rosai-Dorfman com apresentação cutânea exuberante na coxa direita e discreto comprometimento linfonodal limitado à cadeia regional.

Rosai - Dorfman disease: a rare entity diagnosed at autopsy.

Rosai-Dorfman disease (RDD) or Sinus histiocytosis with massive lymphadenopathy is a rare and benign histiocytic proliferative disorder first described by Juan Rosai and Ronald Dorfman in 1969, whose etiology remains unknown. Since then, many cases were reported in the literature. The disease primarily involves the lymph nodes, and is characterized by painless, bilateral cervical lymphadenopathy accompanied by fever, night sweats, malaise and weight loss, reason why many patients are clinically misdiagnosed as malignant lymphoma. In some cases, extranodal involvement may be present. Leukocytosis, elevated erythrocyte sedimentation rate, and hypergamaglobulinemia are often present. The authors report a case of a 52-year-old female patient admitted to the hospital with the diagnosis of pneumonia and progressed to multiple organs failure and death. During the hospitalization an attempt to diagnose a lymphoproliferative disease trough an axillary lymph node biopsy was disappointing. The autopsy was crucial for the diagnosis, illustrating a severe and unusual presentation of Rosai-Dorfman disease.

Acometimento orbitário na doença de Rosai-Dorfman / Orbital involvement in Rosai-Dorfman disease

A doença de Rosai-Dorfman (DRD) ou histiocitose sinusal com linfadenopatia maciça é uma entidade clínica idiopática, rara e benigna, caracterizada pela proliferação histiocitária com linfofagocitose. Geralmente se apresenta com linfoadenomegalia cervical, no entanto pode haver acometimento extranodal, sendo a região orbitária um dos locais de possível acometimento. Na maioria dos casos é uma doença autolimitada com melhora espontânea, entretanto pode ser necessária exérese cirúrgica da lesão ou tratamento com corticosteróides e radioterapia.Neste trabalho, relatamos o caso de um paciente masculino de 29 anos, com queixa de tumoração em pálpebra inferior direita por 6 meses e história de acometimento sinusal prévio pela doença de Rosai Dorfmann. Ao exame oftalmológico apresentava proptose de olho direito e aumento de volume de pálpebra inferior direita. O exame de tomografia computadorizada de órbitas evidenciou lesão sólida extraconal em órbita direita. Após a exérese da lesão houve melhora importante do quadro clínico. O exame histopatológico associado à imuno-histoquímica confirmou o envolvimento orbitário na DRD. Revisamos também o quadro clínico, diagnóstico diferencial e tratamento desta afecção.

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare idiopathic and benign clinical entity, characterized by histiocytic proliferation with linfofagocitosis. It usually presents with cervical lymphadenopathy although extranodal involvement may occur. The orbital region is one of the most common extranodal sites. It is usually a self-limiting disease with spontaneous resolution but surgical excision, corticosteroids and radiotherapy may be necessary. We describe the case of a 29-years-old male patient complaining of an orbital mass sensation for 6 months and a history of previous sinus involvement from Rosai-Dorfman disease. Ophthalmic examination showed proptosis of the right eye and swelling of right lower eyelid. Computed tomography of the orbits disclosed a solid extraconal lesion in the inferior right orbit. After surgical excision there was a significant clinical improvement. Histopathologic examination confirmed the diagnosis of Rosai-Dorfman disease. We also review the clinical picture and differential diagnosis of this condition.

Rosai-Dorfman disease with spontaneous resolution: case report of a child.

Rosai-Dorfman disease is a self-limiting condition caused by histiocyte proliferation within the sinusoids of lymph nodes and in extranodal tissue. It is a rare disease, particularly in children, that progresses with extensive lymphadenopathy. This paper reports on the case of a 2-year-old child with progressive cervical lymphadenopathy associated with persistent fever and radiological findings suggestive of lymphoma. Histopathological and immunohistochemistry studies of a lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Both lymphadenopathy and fever resolved spontaneously.

Rosai-Dorfman disease with spontaneous resolution: case report of a child

Rosai-Dorfman disease is a self-limiting condition caused by histiocyte proliferation within the sinusoids of lymph nodes and in extranodal tissue. It is a rare disease, particularly in children, that progresses with extensive lymphadenopathy. This paper reports on the case of a 2-year-old child with progressive cervical lymphadenopathy associated with persistent fever and radiological findings suggestive of lymphoma. Histopathological and immunohistochemistry studies of a lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Both lymphadenopathy and fever resolved spontaneously.

Doença de Rosai-Dorfman cutânea / Cutaneous Rosai-Dorfman Disease

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.

Histiocitose Sinusal / Sinus Histiocytosis / Histiocytosis del sino

O autor relata o caso de um paciente com linfadenopatia cervical maciça, febre, astenia e sudorese com evolução de três semanas. Iniciada investigação em Unidade de Saúde de Sapiranga ­ RS, Brasil ­ diagnosticou-se histiocitose sinusal (doença de Rosai-Dorfman), sendo realizado manejo inicial e, posteriormente, encaminhado o paciente para serviço de referência.

The author reports the case of a patient with cervical Lymphadenopathy, fever, fatigue and sweating with evolution of three weeks. Initiated research in Health Unit of Sapiranga-RS, Brazil-diagnosed sinus Histiocytosis (Rosai-Dorfman disease), being carried out initial management and subsequently forwarded to the patient referral service.

El autor divulga el caso de un paciente con cervical linfadenopatía, fiebre, fatiga y sudoración con la evolución de tres semanas. Inició la investigación en salud unidad de Sapiranga-RS, Brasil-diagnosticada sino Histiocitosis (enfermedad de Rosai-Dorfman), se llevó a cabo la gestión inicial y posteriormente remitido al servicio de remisión de pacientes.