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)

Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare disease characterised by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal involvement. Orbital involvement occurs in 11% of cases and onset of the disease is typically in the first two decades of life. A benign course is usual, but in some cases blindness or fatality may result. We present a case with late onset at age 78 years and lacrimal gland involvement, and review the literature on this disease.
Orbit 2002 Jun
PMID:A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient. 1202 74

This is a case report of Rosai-Dorfman syndrome in a 36-year-old Caucasian male, involving the lacrimal gland, cervical lymph nodes, nasal and sinusal mucosa. It was successfully treated with appropriate immunosuppression. He had initially presented to the ENT surgeon with nasal and sinusal mucosal thickening and bleeding. Cervical lymph node biopsy produced a histological diagnosis compatible with Rosai-Dorfman disease. Later he developed an acute red proptotic eye. He had severe proptosis due to an enlarged lacrimal gland. He refused surgical excision of the tumour, which is suggested if there is an ocular adnexal involvement. Conservative treatment with systemic steroid resulted in the resolution of lacrimal gland swelling, nasal sinusal mucosal thickening and cervical lymphadenopathy. Previous studies have shown that patients with Rosai-Dorfman syndrome are often black males1 and require surgery.
Orbit 2001 Sep
PMID:Rosai-Dorfman syndrome affecting the lacrimal gland. 1204 17

Rosai-Dorfman disease is a rare condition characterised by massive lymphadenopathy involving the orbit in 11% of cases. Immunohistochemical analysis remains key to the diagnosis with characteristic immunohistochemical S100 staining of histiocytes and lymphocyte phagocytosis. We report the case of a 43-year-old female with recurrence of bilateral orbital Rosai-Dorfman disease following long-term immunosuppressant therapy. Long-term follow-up is advised in conservatively managed patients and surgery may offer more definitive treatment.
Orbit 2005 Mar
PMID:Rosai-Dorfman disease recurrence with bilateral orbital masses following immunosuppressant therapy. 1576 18

The presentation of RDD as an anterior subperiosteal orbital mass with bone involvement has, to the authors' knowledge, not been previously reported. We describe a case of Rosai-Dorfman disease (RDD) presenting as an anterior superolateral subperiosteal orbital mass with erosion of overlying bone mimicking eosinophilic granuloma. It was debulked using endoscopic-guided curettage and the patient was given both local and systemic corticosteroids. Careful histological analysis revealed the diagnosis of RDD and the patient remains asymptomatic and recurrence free at 16 months follow-up. Involvement of the pituitary gland, a recognized yet unusual finding in this condition was also noted. RDD should be considered in the differential diagnosis of a soft tissue mass of the superolateral orbit.
Orbit 2012 Feb
PMID:Orbital Rosai-Dorfman disease with subperiosteal bone involvement mimicking eosinophilic granuloma. 2208 1

A 45 year old female presented with painless swelling over the inner side of both eyes since one year. Magnetic resonance imaging (MRI) scan revealed well defined lesions in the bilateral lacrimal sac area with extension along the naso-lacrimal duct. Systemic work up showed polyclonal hyperglobulinemia, raised erythrocyte sedimentation rate (ESR) and anemia. The patient also had subcutaneous swelling in thoracic area over back. The histopathology of the bilateral excised tumor and fine needle aspiration cytology of thoracic swelling was consistent with features of Rosai-Dorfman syndrome.
Orbit 2012 Apr
PMID:Rosai-dorfman with bilateral involvement of lacrimal sac as extranodal disease. 2248 58

A case of a 9-year-old Black African male with right-sided proptosis is presented. The clinical interpretation was that of orbital rhabdomyosarcoma and an incisional biopsy was performed. This revealed the typical features of Rosai-Dorfman disease, featuring S100 positive non-Langerhans histiocytes with emperipolesis. There were also numerous plasma cells in the biopsy, in the more fibrotic areas. More than 40% of these plasma cells were positive for IgG4. This case represents the first case in the orbit to make the association of IgG4 plasma cell positivity with Rosai-Dorfman disease. Potential associations are discussed, and the relevance of this finding to ophthalmic pathology interpretation and differential diagnosis are highighted.
Orbit 2013 Oct
PMID:A case of orbital Rosai-Dorfman disease with IgG4 positive plasma cells. 2387 15