ABSTRACT
The Rosai-Dorfman disease (RDD) is a bening entity, of unknown etiology, rare, characterized by the overproduction of histiocytes and their subsequent accumulation in the lymph nodes, which may also compromise other organs and systems. It predominantly affects young people. The histological study, which shows emperipolesis, is very indicative but not pathognomonic, but with the immunohistochemistry the diagnosis of the disease is reached, which does not have a well-protocolized treatment. Its evolution is highly variable, with spontaneous remission being a possibility, although in some cases the commitment is extensive, compromising important or vital anatomical structures that requiere vigorous treatment, which can be surgical, radiotherapy and even chemotherapy. We present a case of RDD that affected a 17-year-old girl with upper jaw and right maxillary sinus commitment, which began with loss of teeth, and subsequent appearance of a painless and deforming facial tumour that underwent surgical resection. In this case, the etiological diagnosis was not posible in the preoperative period despite multiple biopsies. An extensive review of the literature is carried out in order to a better understanding of this pathology.
La Enfermedad de Rosai-Dorfman (ERD) es una entidad benigna, de etiología desconocida, poco frecuente, caracterizada por la sobreproducción de histiocitos y su posterior acúmulo en los linfonodos, pudiendo comprometer también a otros órganos y sistemas. Afecta predominantemente a personas jóvenes. El estudio histológico, que muestra emperipolesis, es muy orientativa pero no patognomónica, aunque con la Inmunohistoquímica se llega al diagnóstico de la enfermedad que no tiene un tratamiento bien protocolizado. Su evolución es muy variable, siendo la remisión espontánea una posibilidad, no obstante, en algunos casos, el compromiso es extenso, comprometiendo estructuras anatómicas importantes o vitales que requieren de un tratamiento enérgico, pudiendo ser éste quirúrgico, radioterápico y aún quimioterápico. Se presenta un caso de ERD que afectó a una joven de 17 años, con compromiso de maxilar y seno maxilar derecho, que debutó con pérdida de piezas dentarias y posterior aparición de un tumor facial indoloro y deformante que fue sometido a resección quirúrgica. En este caso no fue posible el diagnóstico etiológico en el preoperatorio pese a múltiples biopsias. Se hace una extensa revisión de la literatura con el objeto de conocer mejor esta patología.
Subject(s)
Humans , Female , Adolescent , Maxillary Diseases/surgery , Maxillary Diseases/diagnostic imaging , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/diagnostic imaging , Tomography, X-Ray Computed , Histiocytosis, Sinus/complications , Histiocytosis, Sinus/pathology , EmperipolesisABSTRACT
Resumen La histiocitosis sinusal con linfadenopatías masivas, conocida como enfermedad de Rosai-Dorfman (ERD), es una patología poco frecuente cuya presentación clásica consiste en el desarrollo de grandes adenopatías cervicales bilaterales indoloras. La manifestación extranodal puede involucrar compromiso cutáneo, del sistema nervioso central, hematológico, óseo, de la vía aérea, entre otros. Su diagnóstico es un desafío y requiere una evaluación completa del paciente incluyendo historia clínica, examen físico, exámenes de laboratorio, imágenes y estudio histopatológico que confirme el diagnóstico. El tratamiento debe ser individualizado para cada paciente. Las alternativas incluyen la observación, corticoides sistémicos, radioterapia, quimioterapia, inmunomoduladores y cirugía. Se presenta el caso de una paciente de 65 años con antecedente de enfermedad de Rosai-Dorfman localizada en glándulas lagrimales y linfoma no Hodgkin, que comienza con obstrucción nasal bilateral progresiva refractaria a tratamiento médico, por lo que se decide realizar cirugía, cuyo estudio histopatológico confirmó enfermedad de Rosai-Dorfman.
Abstract Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare pathology, with a classic clinical presentation of painless bilateral massive cervical lymphadenopathy. The extranodal manifestations may involve skin, central nervous system, hematological, bones, and airway compromise, among others. Its diagnosis is challenging and requires a complete evaluation of the patient including medical history, physical examination, laboratory testing, imaging and histopathological study to confirm the diagnosis. Treatment should be individualized for each patient, including follow-up, systemic corticosteroids, radiotherapy, chemotherapy, immunomodulators and surgery. We present the case of a 65-year-old patient with a history of RDD located in the lacrimal glands and lymphoma, which begins with progressive bilateral obstruction refractory to medical treatment, for which it is decided to perform surgery, whose histopathological study confirmed RDD.
Subject(s)
Humans , Female , Aged , Histiocytosis, Sinus/surgery , Histiocytosis, Sinus/therapy , Histiocytosis, Sinus/diagnostic imaging , Recurrence , Tomography, X-Ray Computed/methods , Treatment OutcomeABSTRACT
Rosai-Dorfman disease (RDD) is an uncommon benign histiocytic disorder. It is found worldwide and slightly more common among in men (1.4:1), affecting individuals are with an average age of 20.6 years. Extranodal tissue involvement is documented in 25-43% of RDD patients. Here authors are discussing a case of 16-year-old male patient with the complaint of a single swelling over left forearm since one month with no lymphadenopathy. Radiology showed anechoic collection within the muscle and superficial to it. On microscopy, smear showed diffusely distributed histiocytes with cytoplasm showing intact lymphocytes with halo surrounding it (emperipolesis), without atypia. Based on cytomorphology diagnosis of Extranodal Rosai Dorfman Disease was made.
ABSTRACT
Objective@#To explore the clinical manifestations, histopathological features, diagnosis, treatment and prognosis of Rosai-Dorfman′s disease (RDD) in the maxillofacial region and to review the relevant literature in order to improve the understanding, diagnosis and treatment of oral and maxillofacial RDD.@*Methods @#The clinical manifestations, histopathological features, diagnosis, treatment, and prognosis of a patient with RDD in the maxillofacial region admitted to Shenzhen People′s Hospital were analyzed, and the literature was reviewed for analysis. @*Results@#The clinical manifestations were palpable masses of 3.5 cm × 2.0 cm × 1.0 cm in the right cheek and 3.0 cm × 2.0 cm × 1.0 cm in the right submaxillary area, with clear boundaries, good mobility, medium and hard textures, respectively, no tenderness, smooth surfaces, and no obvious nodules. On contrast-enhanced and plain CT scans of the maxilla and neck, a diffuse soft tissue shadow was seen in the right maxillofacial region with an unclear boundary and uniform density, and the contrast-enhanced scan also showed moderate and uniform enhancement. The primary diagnosis was right maxillofacial lesions. The tumor was resected surgically. The pathological report was right buccal and right submaxillary extranodal RDD. Under light microscopy, nodular lesions in the fibrous fat tissue were found, which were composed of light and deep staining areas. The light staining areas consisted of patchy, polygonal cells with large volumes and rich cytoplasm, in which lymphocytes and neutrophils could be seen stretching into the movement; the deep staining areas were composed of lymphocytes and plasma cells. IHC: S-100 (+), CD68 (+), CD163 (+), CD1a (-), CD21FDC (+), langerin (-), IgG (+), IgG4 (+). No recurrence was found 11 months after the operation. RDD is a rare, benign and self-limited tissue and cell disease and consists of multiple lesions in the maxillofacial region. Its imaging features are similar to those of lymphoma. Its pathological features are large volumes, rich cytoplasm and phagocytosis of lymphocytes and plasma cells. Generally, RDD only needs to be observed, and individuals with symptoms or the involvement of important organs need to be treated; the first choice for the extranodal type is drug treatment, with radiotherapy administered if the central nervous system is involved. Surgery is recommended if involvement of important organs and compression of the trachea are observed; chemotherapy should be used for diffuse RDD. Most patients with RDD experienced relapse or remission of the disease; a few patients died because of the involvement of important organs or complications.@*Conclusion @#The clinical manifestations of maxillofacial RDD vary and lack specific imaging features, and pathological immunohistochemistry is the gold standard for diagnosis. The etiology is not completely clear, the treatment methods are varied, and the prognosis is related to the involved range of the disease.
ABSTRACT
A 10-year-old girl presented to us with inferonasally displaced, non-axial proptosis of oculus dexter (OD), with ipsilateral, painless massive cervical lymphadenopathy of ten weeks duration. After a detailed hematological work-up and imaging, the histological evidence obtained on cervical lymph node biopsy established the diagnosis of Rosai-Dorfman disease. Although, Rosai-Dorfman disease of unilateral lacrimal gland is extremely infrequent, a high degree of suspicion is warranted in a case of young patient presenting with unilateral isolated lacrimal gland swelling and associated lymphadenopathy, wherein no other cause is found. Treatment protocol should be individualized as per the extent of systemic involvement and functional disability.
ABSTRACT
Objective: To evaluate the clinicopathologic features of Rosai-Dorfman disease (RDD) , and elucidate the potential pathogenesis by whole exome sequencing (WES) . Methods: Clinico-pathological data of 23 RDD patients diagnosed between 2010 and 2018 in Changhai hospital were reviewed, and 9 paraffin-embedded specimens were performed for WES. Results: The median age of 23 RDD patients was 47 (10-79) years. Of them, 19 cases had extranodal lesions, 3 had nodal lesions, and 1 had nodal and extranodal lesions coincidently. All patients received surgery for lesion resection. Histiocytosis in lymph node sinuses or in extranodal tissues accompanied by lymphocyte phagocytosis are typical pathological features of RDD. Immunohistochemical staining shows histocytes are positive for S100, CD68 and CDl63, and negative for CD1a. mTOR, KMT2D and NOTCH1 mutations were detected with WES in these cases. Conclusion: Mutations in mTOR, KMT2D and NOTCH1 genes may be involved in the pathogenesis of RDD, and their clinical significance needs to be further studied.
Subject(s)
Adolescent , Adult , Aged , Child , Humans , Middle Aged , Young Adult , Histiocytosis, Sinus , Exome SequencingABSTRACT
Rosai-Dorfman disease (RDD) , also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign self-limited histiocytic proliferative disorder with undetermined etiology. There is still no specific treatment for RDD. Surgical excision is the first-line treatment, while topical and systemic therapy can be chosen for the patients who are not unfit for surgery. Classic topical therapies include topical treatment or local injection with glucocorticoids. Photodynamic therapy and laser immunotherapy are considered as promising topical treatments. Traditional systemic treatments include oral glucocorticoids, immunomodulators, acitretin, and so on. Rituximab is a new treatment option for patients with CD20-positive RDD. This review summarizes new progress in the treatment of RDD in recent years.
ABSTRACT
Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign self-limited histiocytic proliferative disorder with undetermined etiology. There is still no specific treatment for RDD. Surgical excision is the first-line treatment, while topical and systemic therapy can be chosen for the patients who are not unfit for surgery. Classic topical therapies include topical treatment or local injection with glucocorticoids. Photodynamic therapy and laser immunotherapy are considered as promising topical treatments. Traditional systemic treatments include oral glucocorticoids, immunomodulators, acitretin, and so on. Rituximab is a new treatment option for patients with CD20-positive RDD. This review summarizes new progress in the treatment of RDD in recent years.
ABSTRACT
Objective@#To evaluate the clinicopathologic features of Rosai-Dorfman disease (RDD) , and elucidate the potential pathogenesis by whole exome sequencing (WES) .@*Methods@#Clinico-pathological data of 23 RDD patients diagnosed between 2010 and 2018 in Changhai hospital were reviewed, and 9 paraffin-embedded specimens were performed for WES.@*Results@#The median age of 23 RDD patients was 47 (10-79) years. Of them, 19 cases had extranodal lesions, 3 had nodal lesions, and 1 had nodal and extranodal lesions coincidently. All patients received surgery for lesion resection. Histiocytosis in lymph node sinuses or in extranodal tissues accompanied by lymphocyte phagocytosis are typical pathological features of RDD. Immunohistochemical staining shows histocytes are positive for S100, CD68 and CDl63, and negative for CD1a. mTOR, KMT2D and NOTCH1 mutations were detected with WES in these cases.@*Conclusion@#Mutations in mTOR, KMT2D and NOTCH1 genes may be involved in the pathogenesis of RDD, and their clinical significance needs to be further studied.
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 , LaryngostenosisABSTRACT
Resumen La enfermedad de Rosai Dorfman es una entidad rara caracterizada por linfadenopatías no dolorosas de predominio cervical y submandibular. En el pasado se había considerado como una reticuloendoteliosis maligna, hasta que fue descrita como una entidad clinico-patológica, aislada en 1969 por Rosai y Dorfman. Se presenta el caso de una niña de 4 años de edad, conocida sana, con un cuadro agudo de sensación febril, masa cervical y leucocitosis. Los estudios de laboratorio no identificaron malignidad, ni infección como etiología. Recibió tratamiento con antibióticos sin resolución de su adenopatía, lo cual motivó a que se efectuara una biopsia excisional. El estudio histológico reveló una histiocitosis sinusal con linfoadenopatía masiva. Se decidió dar manejo conservador ya que tuvo una resección completa.
Abstract Rosai Dorfman's disease is a rare entity characterized by non-painful lymphadenopathies predominantly cervical and submandibular. In the past it had been considered a malignant reticuloendotheliosis, until it was described as a clinical-pathological entity, isolated in 1969 by Rosai and Dorfman. We present the case of a 4-year-old girl, known to be healthy, with an acute condition of febrile sensation, cervical mass and leukocytosis. Laboratory studies did not identify malignancy or infection as an etiology. She was treated with antibiotics without resolution of his adenopathy, which led to an excisional biopsy. The histological study revealed a sinus histiocytosis with massive lymphadenopathy. It was decided to give conservative management since it had a complete resection.
Subject(s)
Humans , Female , Child, Preschool , Histiocytosis, Sinus/diagnosis , Histiocytosis, Sinus/diagnostic imaging , Costa RicaABSTRACT
La enfermedad de Rosai-Dorfman; también conocida como linfadenopatía masiva con histiocitosis sinusal; es un raro desorden de etiología desconocida; generalmente asociado a un agrandamiento de los ganglios linfáticos superficiales y/o profundos. La mayoría de los pacientes no requiere tratamiento. Presentamos el caso de un niño de 10 meses de vida, que ingresa con una tumoración cervical izquierda de 25 días de evolución.
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare dis order of unknown etiology, generally associated with enlargement of superficial and/or deep lymph nodes. Most patients do not require treatment. We report the case of a 10 month old infant who was admitted with a left cervical tumor of 25 days duration.
Subject(s)
Humans , Male , Infant , Histiocytosis, Sinus/diagnosis , Uterine Cervical Neoplasms/diagnosis , Hypertrophy , Lymph Nodes/pathologyABSTRACT
Rosai Dorfman disease or Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, self-limiting disorder that commonly presents as massive bilateral and painless cervical lymphadenopathy. We present a case of a 6 year old male child who presented with cervical lymphadenopathy with no other complains and was diagnosed on fine needle aspiration cytology as a case of Rosai Dorfman disease. Patient was given low dose steroids to which patient responded well and became asymptomatic in 15 days.
ABSTRACT
Rosai-Dorfman disease (RDD) is a nonmalignant histiocytic disorder of unknown origin that is extremely rare. By immunohistochemistry, the RDD cells are characteristically S-100 positive and CD1a negative. Emperipolesis is a common histopathological finding, although not specific for RDD. Lymph node and cutaneous manifestations are most frequent, but diverse organs can be affected. The clinical course is unpredictable regardless of treatment. Here, we present a series of 8 cases presenting lymph node and/or cutaneous lesions. Lymph node involvement was seen in diverse regions, including mediastinal and retroperitoneal. The treatment response to steroids was diversified, and the chemotherapy response was disappointing. Associated autoimmune diseases (Sjögren syndrome and antiphospholipid syndrome) were observed in 2 patients. Regardless of therapy modality, these patients exhibited a favorable prognosis in a follow-up duration that ranged from 15 to 80 months.
ABSTRACT
Introduction Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies...
Subject(s)
Humans , Female , Child , Histiocytosis, Sinus , Lymphatic Diseases , Diagnosis, DifferentialABSTRACT
Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is an idiopathic, benign, histiocytic proliferative disease involving lymph nodes and extranodal sites. A 52-year-old woman presented with a 2-year history of multiple non-tender, erythematous nodules on her face. The initial punch biopsy showed lymphoid follicle-like aggregation of CD20 positive lymphocytes in the papillary dermis consisting of primary cutaneous marginal zone B-cell lymphoma. She underwent a 3-month course of radiotherapy, but the lesions progressed. The re-biopsy specimen revealed marked histiocytes with positive CD68 and S-100 proteins. The patient was treated with prednisolone and isotretinoin to which she responded slightly. Repeated histopathologic examinations enable us to make a definite diagnosis. Differentiating these two disorders as early as possible is crucial for appropriate treatment.
Subject(s)
Female , Humans , Middle Aged , Biopsy , Dermis , Diagnosis , Histiocytes , Histiocytosis, Sinus , Isotretinoin , Lymph Nodes , Lymphocytes , Lymphoma, B-Cell, Marginal Zone , Prednisolone , Radiotherapy , S100 ProteinsABSTRACT
Background: Oral squamous cell carcinoma is the most common neoplasm and comprises of approximately 80% of the cancers occurring in the oral cavity. The role of the host response to this neoplasm has been recognized, and for many years the regional lymph node in tumor-bearing hosts has been considered as an anatomic barrier to the systematic dissemination of tumor cells. Morphological evaluation of the regional nodes has aided in understanding the immune response. Aim: The current study was carried out to observe the morphological changes occurring in the regional lymph nodes and to evaluate whether these features could be helpful in assessing the immunological status of the patient, and thereby, the prognosis of the patient. Materials and Methods: The study was based on lymph nodes from 63 patients with oral squamous cell carcinoma, who underwent radical neck dissection or modified neck dissection. In the lymph node, four morphological patterns were observed that included lymphocyte predominance, germinal center predominance, mixed pattern (sinus Histiocytosis), and an unstimulated pattern. The cases were then divided into four groups according to the predominant immunoreactivity pattern based on the World Health Organization (WHO) standardized system for reporting human lymph node morphology. Results: Revealed that risk of metastases to cervical lymph nodes in patients with lymphocyte predominance was less (28.6%) when compared to the high risk of metastases with germinal center predominance (68%), and these results were statistically significant (P < 0.05). Patients with a mixed pattern showed less risk of metastases (45.4%), while those with an unstimulated pattern had increased risk of metastases (66.6%), but the results were not statistically significant. It was also found that in the positive nodes, germinal center hyperplasia (50.2%) was the predominant pattern. Conclusion: The present study revealed that patients with lymphocyte predominance had less risk of metastases and patients with germinal center predominance had a high risk of metastases to the lymph node.
Subject(s)
Capillaries/pathology , Carcinoma, Squamous Cell/immunology , Carcinoma, Squamous Cell/pathology , Carcinoma, Squamous Cell/secondary , Endothelial Cells/pathology , Endothelium, Vascular/pathology , Forecasting , Germinal Center/pathology , Histiocytosis, Sinus/pathology , Humans , Hyperplasia , Lymph Nodes/immunology , Lymph Nodes/pathology , Lymphatic Metastasis/immunology , Lymphatic Metastasis/pathology , Lymphocytes/pathology , Macrophages/pathology , Mouth Neoplasms/immunology , Mouth Neoplasms/pathology , Neck Dissection/methods , Prognosis , Risk FactorsABSTRACT
Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease is a rare disorder characterized by proliferation of distinctive histiocytes within lymph node sinuses and lymphatics, sometimes involving extranodal sites. However, clinical suspicion is difficult and there is also a lack of useful diagnostic markers for this disorder prior to histological confirmation. High elevation of serum ferritin is known to be a useful diagnostic marker for various hematologic diseases, including hemophagocytic lymphohistiocytosis and lymphoma. Here, we report a case of fever of unknown origin that presented along with highly elevated serum ferritin (5,780 ng/mL), and was finally diagnosed as Rosai-Dorfman disease by lymph node biopsy.
Subject(s)
Adult , Female , Humans , C-Reactive Protein/analysis , Ferritins/blood , Histiocytosis, Sinus/blood , L-Lactate Dehydrogenase/blood , Lymph Nodes/pathology , Positron Emission Tomography Computed TomographyABSTRACT
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/pathologyABSTRACT
Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with massive lymhadenopathy (SHML) is a rare, benign proliferative disorder of histiocytes, sometimes showing familial incidence. It is mainly characterized by painless bilateral cervical lymph node enlargement and is often associated with fever and leucocytosis. It presents in the first or second decades of life, though any age group can be affected. Without the awareness about RDD, the diagnosis of RDD is unexpected especially in South East Asian Countries where certain lymphadenopathies such as tuberculosis, metastatic nasopharayngeal carcinoma and lymphomas are common. RDD is commonly missed and so its revision would seem worthwhile, this case illustrating the disappearance of the lymphadenopathy without treatment.