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Carl Gebhardt, MD; Marco Averbeck, MD; Uwe Paasch, MD; Selma Ugurel, MD; Hjalmar Kurzen, MD; Patrick Stumpp, MD; Jan C. Simon, MD; Regina Treudler, MD

Arch Dermatol. 2009;145(5):571-574.

Background  Rosai-Dorfman disease is a non–Langerhans cell histiocytosis that recently has been treated successfully with imatinib mesylate in a patient with a systemic variant of the disease.

Observations  We describe a 69-year-old man with cutaneous Rosai-Dorfman disease manifesting as progressive, deeply infiltrated skin lesions. Histopathologic examination of the lesions demonstrated dense dermal infiltrate positive for CD68, stabilin-1, and S-100, but not for CD1a. The histiocytes were positive for platelet-derived growth factor receptor , the target molecule for imatinib. During the 5-year course of the disease, multiple therapeutic approaches (tuberculostatic drugs, topical and systemic glucocorticoids, thalidomide, isotretinoin, and methotrexate) did not result in significant improvement. Imatinib mesylate therapy (600 mg/d for 2 weeks and then 400 mg/d for 10 weeks) had no effect, despite the expression of platelet-derived growth factor receptor on the histiocytes.

Conclusions  Failure of imatinib therapy in our patient may be due to a lack of functioning target molecules, the therapy protocol, or the course of the disease. Cutaneous and systemic variants of Rosai-Dorfman disease may be different clinical entities or at least may respond differently to tyrosine kinase inhibitors.

Author Affiliations: Departments of Dermatology, Venereology, and Allergology (Drs Gebhardt, Averbeck, Paasch, Simon, and Treudler) and Radiology (Dr Stumpp), Universitätsklinikum Leipzig Anstalt öffentlichen Rechts, Leipzig, and Department of Dermatology, Venereology, and Allergology, University Medical Center Mannheim, Ruprecht Karl University of Heidelberg, Mannheim (Drs Ugurel and Kurzen), Germany.

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