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Published on: December 15, 2011
Rosai-Dorfman disease with diffuse gastrointestinal involvement
Bulent Baran1, Cetin Karaca, Ozlem M Soyer
1Department of Gastroenterohepatology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
European Journal of Gastroenterology & Hepatology
|March 21, 2013
Summary
Rosai-Dorfman disease, a rare histiocytic disorder, can unusually affect the gastrointestinal tract. This case highlights the diagnostic challenges of atypical extranodal Rosai-Dorfman disease.
Area of Science:
- Histiocytic Disorders
- Gastrointestinal Pathology
Background:
- Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare, self-limiting histiocytic proliferative disorder.
- Typically presents in childhood with cervical lymphadenopathy and systemic symptoms, RDD is usually a nodal disease.
Observation:
- Extranodal involvement is less common, with the gastrointestinal tract being an unusual site.
- This report details an atypical RDD case with systemic symptoms and diffuse gastrointestinal involvement.
Findings:
- Diagnosis relies on clinical suspicion and histopathological examination of gastrointestinal biopsy samples.
- The diffuse gastrointestinal manifestation presented a significant diagnostic and therapeutic challenge.
Implications:
- Highlights the importance of considering RDD in cases of unexplained gastrointestinal symptoms and systemic illness.
- Underscores the need for thorough histopathological evaluation for accurate diagnosis of extranodal RDD.
- Emphasizes the diagnostic and therapeutic complexities associated with atypical gastrointestinal Rosai-Dorfman disease.
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