Extranodal manifestation of Rosai-Dorfman disease without involvement of lymph nodes.
BACKGROUND
Rosai-Dorfman disease is an uncommon sinus histiocytosis with massive lymphadenopathy. Rosai-Dorfman disease without lymphadenopathy is extremely rare. Extranodal pseudotumoral masses can occur--and have been identified and described--in the orbit, skin, bone, and upper respiratory tract. Because of its rareness, Rosai-Dorfman is seldom considered in the clinical differential diagnosis, particularly if extranodal manifestations predominate.


RESULTS
We present herein a patient with extranodal manifestation of Rosai-Dorfman disease in the orbit and parotid without typical initial lymph node involvement that reacted on steroid therapy.


CONCLUSIONS
The correct diagnosis of this entity with the knowledge that it can occur without lymphadenopathy is important. For these patients, diagnosis must be based on histologic and immunohistopathologic findings after surgical biopsy. Steroid therapy can be used for treatment.
Department of Craniomaxillofacial and Oral Surgery, University of Zurich, Zurich, Switzerland. astrid.kruse@usz.ch
Astrid L D
AL
Kruse
Carole
C
Gengler
Klaus W
KW
Grätz
Joachim A
JA
Obwegeser
D008949:Adenoma, Pleomorphic / Q000175:diagnosis; D000328:Adult; D003907:Dexamethasone / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005938:Glucocorticoids / Q000627:therapeutic use; D015618:Histiocytosis, Sinus / Q000175:diagnosis*; D006801:Humans; D007766:Lacrimal Apparatus Diseases / Q000175:diagnosis; D008198:Lymph Nodes / Q000473:pathology; D008297:Male; D008443:Maxillary Sinus / Q000473:pathology; D009916:Orbital Diseases / Q000175:diagnosis*; D010254:Paranasal Sinus Diseases / Q000175:diagnosis; D010305:Parotid Diseases / Q000175:diagnosis*
D005938:Glucocorticoids; D003907:Dexamethasone