Unmasking Rosai-Dorfman disease in a woman with cervical lymphadenopathy: a histopathological insight

Rosai-Dorfman disease (RDD) also called as sinus histiocytosis with massive lymphadenopathy is a rare benign proliferative self?limiting disease of the macrophage?histiocyte cell family. Its etiology is unknown and presents with massive lymphadenopathy. We hereby present a case of a female presenting with massive cervical lymphadenopathy, evening rise in temperature and weight loss. It was initially misdiagnosed as tuberculous cervical lymphadenopathy as it is the most common cause of cervical lymphadenopathy in India due to high prevalence of tuberculosis. It was treated with anti-tuberculous drugs, but did not resolve. Eventually neck biopsy was done which showed characteristic histopathologic features including emperipolesis and S100 positivity on immunohistochemistry. The patient was managed conservatively with observation and symptomatic management. Patient underwent spontaneous remission in a few months. The absence of any specific clinical features caused diagnostic difficulty, delaying the diagnosis. This report highlights the importance of considering RDD in the differential diagnosis of cervical lymphadenopathy, particularly in tuberculosis-endemic regions.

Extranodal rosai dorfman disease: a case report of single soft tissue cystic lesion

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.

Treatment of Rosai-Dorfman disease / 中华皮肤科杂志

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.

Treatment of Rosai-Dorfman disease / 中华皮肤科杂志

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.

Enfermedad de Rosai-Dorfman: A propósito de un caso / Rosai-Dorfman disease: Case report

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.

Rosai-Dorfman disease: a report of eight cases in a tertiary care center and a review of the literature

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.

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease): A Case Report and Literature Review

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...

Cutaneous Rosai-Dorfman Disease (RDD) Mimicking Primary Cutaneous Marginal Zone B-cell Lymphoma / 대한피부과학회지

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.

A Case of Rosai-Dorfman Disease with Highly Elevated Serum Ferritin

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.

Rosai-Dorfman Disease.

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.

A Case of Cutaneous Rosai-Dorfman Disease Manifesting as Multiple Tumors / 대한피부과학회지

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic, benign, self-limited, histiocytic proliferative disease confined mainly to cervical lymph nodes. The disease is usually accompanied by massive bilateral lymphadenopathy, fever, elevated erythrocyte sedimentation rate, leukocytosis with neutrophilia, and polyclonal hypergammaglobulinemia. Although cutaneous manifestations are observed in 10% of patients, pure cutaneous RDD without any nodal or systemic involvement is very rare. We report a case of pure cutaneous RDD occurring in a 37-year-old man with asymptomatic erythemaotus to yellowish multiple widespread nodules and tumors on the face and trunk. Histopathologic examination characteristically showed a diffuse infiltrate composed of large histiocytes exhibiting emperiopolesis and mixed inflammatory cells including many plasma cells. Immunohistochemically, these histiocytes were positive for S-100 protein and CD68, but negative for CD1a. During 2 years of follow-up, the patient showed neither nodal nor systemic involvement of the disease and the skin lesions gradually disappeared. The final diagnosis was made by typical cutaneous manifestations without nodal or extranodal involvement and histopathological findings compatible with RDD.

Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph node sinuses and lymphatics in extranodal sites. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. A 26-yr-old man presented with painless palpable lymph node in cervical area. Radiographic studies revealed pleural effusion with lymphadenopathy and calcification in mediastinum. The cervical lymph node biopsy showed dilated sinuses filled with histiocytes with clear cytoplasm. The cells stained positive with CD68 and S-100. These cytologic and immunohistochemical findings were considered consistent with the diagnosis of SHML.

A Case of Rosai-Dorfman Disease Treated with Hydroxychloroquine / 대한피부과학회지

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy (SMHL) is a benign histiocytic proliferative disorder of unknown etiology. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. Histopathologic examinations showed characteristic large histiocytes exhibiting emperipolesis. On immunohistochemical stains, histiocytes are positive for CD68 and S-100 protein, but negative for CD1a. The lymph node involvement is typical, but it may also involve other systemic organs in one third of cases such as skin, upper respiratory tract, bone and so on. Patients with purely cutaneous Rosai-Dorfman disease are of older age at onset of the disease with a reversed male/female ratio, so cutaneous Rosai-Dorfman disease is recognized as a distinct entity from Rosai-Dorfman disease. We present a 48-year-old woman with an asymptomatic erythematous indurated plaque with yellowish papules on her left cheek and erythematous papules on her left infraorbital area, diagnosed as cutaneous Rosai-Dorfman disease at first. But 3 months later, she developed a palpable lymph node on her left submandibular area. She responded to high-dose hydroxychloroquine treatment.

Two Cases of Cutaneous Rosai-Dorfman Disease / 대한피부과학회지

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, idiopathic histiocytic proliferative disorder affecting lymph nodes as well as extranodal sites. It is accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. Although cutaneous involvement in RDD is common, a purely cutaneous disease is very rare. Histologic findings show characteristic large, pale, histiocytic cells exhibiting cytophagocytosis (emperipolesis). Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but negative for CD1a. The etiology is unknown, although it is thought to be a reactive disorder rather than neoplastic. We report two cases of Rosai-Dorfman disease showing involvement limited to the skin.

DIAGNOSIS AND MANAGEMENT OF EXTRANODAL ROSAI-DORFMAN DISEASE IN NASAL SINUSES / 解放军医学杂志

Objective To evaluate the clinical manifestations, pathological features, diagnosis, differential diagnosis and treatment of extranodal Rosai Dorfman disease(RDD) in paranasal sinuses. Methods The clinical manifestations, pathological findings, and imaging data of a rare case of extranodal RDD in the paranasal sinuses, who had received repeated surgical interventions, were analyzed. Results The lesion was situated in the paranasal sinuses, It had been repeatedly removed and misdiagnosed. Physical examination showed a red spherical mass in the nasal cavity. CT scan of the sinuses revealed a 5cm 5 cm soft tissue opacification of the right nasal cavity and paranasal sinuses. There was also erosion of the lateral wall of the maxillary sinus, the medial wall of orbit, parts of pterygopalatine fossa, and infratemporal fossa. Through the previous incision, all visible tumours in the lateral nasal cavity, paranasal sinuses, pterygopalatine fossa and orbital base were again removed. The tumour was attached to the orbital periosteum. The orbit was not removed, but the tumour was stripped off as completely as possible. Microscopic examination revealed intermingling spindled shaped and polygonal histiocytes, admixing with few plasma cells. The infiltrating histiocytes had large round or oval nuclei. The cytoplasm was abundant, granular or vacuolated and foamy. Immunohistochemical studies showed that the histiocytes were strongly reactive with antibodies against S100 protein and Vimetin. Conclusion The diagnosis of extranodal RDD in the paranasal sinuses should be differentiated with rhinoscleroma, eosinophilic granuloma, plasmacytoma, or fibrous histiocytoma. The identification of the distinctive histocytes which are typically immunoreactive to S 100 and CD68 can be quite helpful for correct diagnosis. Surgical excision supplemented by chemotherapy and radiotherapy, may be the optimal treatment, and long term follow up is essential.

Histiocitosis sinusal con linfadenopatía masiva (Enfermedad de Rosai Dorfman) / Sinusal histiocytosis with massive lymphadenopathy: report of a case

Se presenta el caso de una Joven de 14 años con adenomegalias cervicales bilaterales masivas, con diagnóstico clínico de linfoma y cuyo estudio anatomopatológico demostró los hallazgos morfológicos de la histiocitosis sinusal con linfadenopatía masiva. Esta entidad debe tenerse en cuenta en todo diagnóstico diferencial de adenomegalias masivas en niños o pacientes jóvenes, pues el curso clínico y el pronóstico en general son benignos.

The case of a 14 year-old girl with massive, bilateral, cervical adenopathies is reported. The Initial diagnosis was a Iymphoma but histological study revealed typical changes of sinusal histiocytosis with massive Iymphadenopathy. The patient was treated with acyclovir and two months later left adenopathies had considerably decreased while the right ones remained unchanged. This disease should be taken Into account in the differential diagnosis of massive adenopathies in children or young people. Its clinical course and prognosis are usually benign

A Case of Sinus Histiocytosis with Massive Lymphadenopathy / 대한피부과학회지

Sinus histiocytosis with massive lymphadenopathy(SHML) is a benign, generally selflimited pseudolymphomatous disease that typically appears with cervical massive lymphaclenopathy. Extranodal involvement including skin occurs in the 28% of the cases. We report a case af SHML in 51 year-old male who had several, prominent firm masses ranging from 1-10cm in the cervical, axillary, inguinal areas and multiple, plum colored nodules and plaques in the face, trunk for about 10 years. The histopathological findings of cervical lymph node, facial nodule showed dense heavy infiltration of large histiocytes with abundant pale eosinophilic cytoplasm in the subcapsular and medullary sinuses of lymph node and dermis of skin. No atypical cells suggesting malignancy is seen in the infiltrates. The patient had been treated with combination of prednisolone and vinblasstine, but he expired 1 month later.

Sinus Histiocytosis with Massive Lymphadenopathy : Report of A Case with Perineal Involvement / 대한비뇨기과학회지

The term "sinus histiocytosis with massive lyrnphadenopathy," introduced in 1969 by Rosai and Dorfman, refers to a newly recognized disease entity characterized by painless cervical lymphadenopathy, fever, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. The typical course is one of insidious onset, protracted duration of the active disease state, and eventual spontaneous remission, occasionally with subsequent recurrences. Lymph nodes other than those in the cervical area may be involved, and extranodal involvement can occur (such as in the orbit, skin, or respiratory tract) Clinically, this entity may closely simulate malignant lymphoma or nonneoplastic conditions with lymphadenopathy. We present a case in which this disease process involved the perineum.