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cutaneous Rosai-Dorfman disease

Tuesday 16 December 2003

Rosai-Dorfman disease (RDD), previously known as sinus histiocytosis with massive lymphadenopathy, is a benign, idiopathic histiocytic proliferative disorder.

Rosai-Dorfman Disease (RDD) is an idiopathic reactive proliferation of distinctive histiocytes that have abundant cytoplasm and commonly exhibit intracytoplasmic ingestion of inflammatory cells (emperipolesis).

The histiocytes are immunopositive for S100 protein and are typically associated with an infiltrate of lymphocytes, plasma cells, and neutrophils.

The classic clinical presentation is massive enlargement of (usually) cervical lymph nodes with a histologic appearance that mimics exaggerated sinus histiocytosis.

RDD can also involve extra-nodal sites and skin involvement is common either as part of disseminated disease or as a result of primary disease.

Synopsis

- solitary nodules
- large, well-circumscribed plaque
- large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting cytophagocytosis

Immunohistochemistry

- S-100+
- CD68+
- CD1a-

Electron microscopy

- Birbeck granules are absent

References

- Pitamber HV, Grayson W. Five cases of cutaneous Rosai-Dorfman disease. Clin Exp Dermatol. 2003 Jan;28(1):17-21. PMID: 12558621