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Rosai Dorfman disease--a clinico-pathological presentation
1Department of Medicine, King George's Medical University, Lucknow.
The Journal of the Association of Physicians of India
|November 21, 2007
Summary
Rosai Dorfman disease (RDD), a rare disorder, can present as a nasal mass and generalized lymphadenopathy. Diagnosis is confirmed by histopathology showing lymphophagocytosis, highlighting RDD as an uncommon cause of enlarged lymph nodes.
Area of Science:
- Pathology
- Oncology
- Immunology
Background:
- Rosai Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is an uncommon benign proliferative disorder.
- RDD typically presents with painless lymphadenopathy, often in the cervical region, and may be accompanied by systemic symptoms.
Observation:
- A 45-year-old female patient presented with a nasal mass and generalized lymphadenopathy.
- Histopathological examination of the affected tissues revealed lymphophagocytosis, also referred to as emperipolesis.
Findings:
- The observed lymphophagocytosis is a characteristic feature consistent with a diagnosis of Rosai Dorfman disease.
- The case highlights the diverse clinical presentations of RDD, including extranodal involvement such as a nasal mass.
Implications:
- This case underscores the importance of considering RDD in the differential diagnosis of patients presenting with generalized lymphadenopathy and unusual masses.
- Understanding the clinical and histological features of RDD is crucial for accurate diagnosis and management of this rare condition.
