title: A case of cutaneous Rosai-Dorfman disease refractory to imatinib therapy.
abstract: 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.
  
  
  METHODS
  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 alpha, 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(1/2) weeks and then 400 mg/d for 10 weeks) had no effect, despite the expression of platelet-derived growth factor receptor alpha 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.
authors.affiliation: Department of Dermatology, Venereology, and Allergology, Universitätsklinikum Leipzig Anstalt öffentlichen Rechts, Philipp-Rosenthal-Strasse 23-25, 04103 Leipzig, Germany. Carl.Gebhardt@mail.medizin.uni-leipzig.de
authors.forename: Carl
authors.initials: C
authors.lastname: Gebhardt
authors.forename: Marco
authors.initials: M
authors.lastname: Averbeck
authors.forename: Uwe
authors.initials: U
authors.lastname: Paasch
authors.forename: Selma
authors.initials: S
authors.lastname: Ugurel
authors.forename: Hjalmar
authors.initials: H
authors.lastname: Kurzen
authors.forename: Patrick
authors.initials: P
authors.lastname: Stumpp
authors.forename: Jan C
authors.initials: JC
authors.lastname: Simon
authors.forename: Regina
authors.initials: R
authors.lastname: Treudler
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001549:Benzamides; D014408:Biomarkers, Tumor / Q000378:metabolism; D001706:Biopsy; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D015618:Histiocytosis, Sinus / Q000175:diagnosis / Q000188:drug therapy* / Q000378:metabolism; D006801:Humans; D000068877:Imatinib Mesylate; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D010879:Piperazines / Q000008:administration & dosage*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors; D011743:Pyrimidines / Q000008:administration & dosage*; D020796:Receptor, Platelet-Derived Growth Factor alpha / Q000378:metabolism; D012867:Skin / Q000378:metabolism / Q000473:pathology; D013997:Time Factors
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D014408:Biomarkers, Tumor; D010879:Piperazines; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases; D020796:Receptor, Platelet-Derived Growth Factor alpha