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.

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 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: Carl Gebhardt, Marco Averbeck, Uwe Paasch, Selma Ugurel, Hjalmar Kurzen, Patrick Stumpp, Jan C Simon, Regina Treudler
• Journal: Archives of dermatology (Arch Dermatol) Vol. 145 Issue 5 Pg. 571-4 (May 2009) ISSN: 1538-3652 [Electronic] United States
• PMID: 19451502 (Publication Type: Case Reports, Journal Article)

Chemical References

• Antineoplastic Agents
• Benzamides
• Biomarkers, Tumor
• Piperazines
• Pyrimidines
• Imatinib Mesylate
• Protein-Tyrosine Kinases
• Receptor, Platelet-Derived Growth Factor alpha

Topics

• Aged
• Antineoplastic Agents (administration & dosage)
• Benzamides
• Biomarkers, Tumor (metabolism)
• Biopsy
• Diagnosis, Differential
• Follow-Up Studies
• Histiocytosis, Sinus (diagnosis, drug therapy, metabolism)
• Humans
• Imatinib Mesylate
• Immunohistochemistry
• Magnetic Resonance Imaging
• Male
• Piperazines (administration & dosage)
• Protein-Tyrosine Kinases (antagonists & inhibitors)
• Pyrimidines (administration & dosage)
• Receptor, Platelet-Derived Growth Factor alpha (metabolism)
• Skin (metabolism, pathology)
• Time Factors