Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a patient with steroid responsive cutaneous Rosai-Dorfman disease (RDD) associated with bilateral anterior uveitis and hypothyroidism. RDD is a rare histiocytic disorder of unknown aetiology. There is characteristic histology in which large pale-staining histiocytes exhibit emperipolesis and stain positive for S-100 protein. This condition is thought to be a reactive process rather than a neoplastic one and viral and immune mediated causes have been postulated.
Clin Exp Dermatol 2002 Jun
PMID:Steroid responsive cutaneous Rosai-Dorfman disease associated with uveitis and hypothyroidism. 1213 69

Rosai-Dorfman disease (RDD; sinus histiocytosis with massive lymphadenopathy) and Langerhans cell histiocytosis (LCH) are two different yet pathogenetically related histiocytic disorders. While systemic and localized forms have been identified in both diseases, each has its own characteristic histological, immunohistochemical and ultrastructural profile. Rarely, either RDD or LCH can also occur in the context of certain malignant neoplasms. However, the coexistence of RDD and LCH has never been described. We report a case of cutaneous RDD in which a focus of LCH was found. Clinical and laboratory examinations revealed no evidence of extracutaneous involvement of RDD or LCH. We believe that this is the first report of such a coexistence, and the possible pathogenesis is discussed.
Br J Dermatol 2002 Oct
PMID:Coexistence of localized Langerhans cell histiocytosis and cutaneous Rosai-Dorfman disease. 1236 28

Rosai-Dorfman disease (RDD), previously known as sinus histiocytosis with massive lymphadenopathy, is a benign, idiopathic histiocytic proliferative disorder. It commonly affects lymph nodes, but any organ of the body may be involved. Histological findings include characteristic large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting cytophagocytosis. Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but stain negatively for CD1a. On electron microscopy, Birbeck granules are absent. RDD limited to the skin is rare, less than 30 cases having been reported to date. We present five further cases of purely cutaneous RDD. Three presented as solitary nodules and one as a large, well-circumscribed plaque. The fifth patient, who was HIV positive, had a rosacea-like facial eruption.
Clin Exp Dermatol 2003 Jan
PMID:Five cases of cutaneous Rosai-Dorfman disease. 1255 21

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.
Arch Dermatol Res 2003 Dec
PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

Presented is a case of a 31-year-old woman with cutaneous Rosai-Dorfman disease, which is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. It primarily involves lymph nodes but can affect extranodal sites such as the skin, where it typically appears as indistinct erythematous papules and nodules. On histopathologic examination, emperipolesis is a consistent finding. The etiology is unknown, and treatment is based on clinical manifestations.
Dermatol Online J 2004 Nov 30
PMID:Cutaneous Rosai-Dorfman disease. 1574 82

Rosai-Dorfman disease in soft tissue without nodal disease has been recognized as a distinct clinicopathologic entity. It may represent a diagnostic challenge and the natural history and optimal treatment has not been well clarified. We investigated a patient in whom Rosai-Dorfman disease was confined to the subcutis of the abdominal wall and recurred after incomplete excision. Complete resolution was achieved by wide surgical excision with negative margins. Pathologic examination confirmed the diagnosis of extranodal Rosai-Dorfman disease. The patient is disease-free after 1 year of follow-up. Despite the possibility that spontaneous remission may occur, our results suggest that when anatomically feasible, complete excision can be a treatment option for persistence or recurrence of exclusively extranodal disease. Larger case series and longer follow-up are needed to assess the long-term efficacy in these patients.
J Eur Acad Dermatol Venereol 2005 Nov
PMID:Subcutaneous Rosai-Dorfman disease: is surgical excision justified? 1626 85

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management.
Indian J Dermatol Venereol Leprol
PMID:Rosai-Dorfman syndrome. 1639 60

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a benign idiopathic histiocytic proliferative disorder that commonly involves the lymph nodes but secondarily may involve the skin. However, purely cutaneous disease without lymphadenopathy or internal organ involvement rarely may occur. We present case reports of three patients who presented with asymptomatic nonspecific enlarging skin nodules without evidence of lymphadenopathy or internal disease. Histopathologic examination of skin lesions in all patients showed proliferation of large histiocytes with phagocytosed inflammatory cells characteristic of Rosai-Dorfman disease. However, the diagnoses of dermatofibroma, other spindle cell neoplasm, infectious granulomatous process, and other xanthohistiocytic proliferations were also considered due to the presence of storiform spindle cells and foamy cells in the first case. One patient experienced regression during a course of oral steroids, while another patient cleared spontaneously. In the absence of massive lymphadenopathy characteristic of Rosai-Dorfman disease, the diagnosis of purely cutaneous Rosai-Dorfman disease may be complicated by the rarity, non-specific clinical appearance of skin lesions, and broad histopathological differential diagnosis of this disorder. A high index of suspicion of the clinician and pathologist is often required.
Dermatol Online J 2006 Jan 27
PMID:Cutaneous Rosai-Dorfman disease. 1663 76

A 63-year-old woman developed multiple nodules at the site of a recently biopsied proliferation of CD 68+ histiocytes. Clinical lymphadenopathy was not present on physical examinaton or on computed tomography scans. A subsequent skin biopsy specimen showed changes consistent with Rosai-Dorfman disease. Rosai-Dorfman disease is a benign, histiocytic proliferative disorder also known as sinus histiocytosis with massive lymphadenopthy. A particularly rare, extranodal, purely cutaneous form has been described. Spontaneous regression tends to occur over months to years, with surgical intervention remaining the most effective treatment. Documented success with a number of other therapies has been reported, which include systemic high-dose thalidomide in extensive cutaneous disease.
Dermatol Online J 2008 May 15
PMID:Cutaneous Rosai-Dorfman disease. 1862 44

We present herein a Japanese case of Rosai-Dorfman disease (RDD) in which cutaneous manifestations completely remitted after treatment with low-dose oral corticosteroid. A 69-year-old Japanese man presented with a 1-year history of enlarged submandibular lymph nodes and subsequent nasal and pharyngeal bleeding. RDD was diagnosed based on biopsy results from a lymph node in the left parotid region. The patient had also noted several skin eruptions that repeatedly appeared and disappeared on the face and arms. Biopsies were taken from skin eruptions on the face and cuboidal fossa. Both biopsy specimens showed dense, well-demarcated infiltration of histiocytes, lymphocytes and multinucleated giant cells from just under the epidermis to the subcutaneous tissue. These histiocytes were positive for CD68 and S-100, but negative for CD1a, and some displayed emperipolesis. Given the histopathological findings and the fact that the patient was suffering from RDD, skin lesions were diagnosed as cutaneous manifestations of RDD. Cutaneous lesions gradually began to persist concomitant with enlargement of extranodal lymphadenopathy in the nasopharyngeal area. Increasing respiratory obstruction prompted a trial with oral prednisolone commencing at 0.4 mg/kg per day. Both the lymphadenopathy and skin lesions responded quickly. Within 3 months, all his skin lesions disappeared completely with almost complete resolution of lymphadenopathy. Twelve months after the beginning of oral prednisolone therapy, slight recurrence of mucosal and cutaneous lesions appeared, but disappeared quickly with an increase in prednisolone to 0.3 mg/kg per day. Low-dose prednisolone appeared very effective in the case of RDD.
J Dermatol 2009 Apr
PMID:Successful treatment of Rosai-Dorfman disease with low-dose oral corticosteroid. 1934 64


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