[Cutaneous Rosai-Dorfman disease].
OBJECTIVE
To investigate the clinicopathologic feature, immunophenotype and differential diagnosis of cutaneous Rosai-Dorfman disease (CRDD).


METHODS
Clinical manifestation, morphologic features and immunohistochemical staining were studied in 8 cases of CRDD.


RESULTS
All 8 patients presented with multiple papules, nodules and/or coalescent patches or plaques distributing over the extremities or trunk, without lymphadenopathy or other systemic abnormalities. Microscopically, the lesions were located intradermally and/or subcutaneously. CRDD was characterized by the presence of S-100 positive histiocytic cells exhibiting emperipolesis, accompanying with infiltration of mixed inflammatory cells. Fibrosis, somewhere in vague storiform pattern due to stromal responses, with distribution of individual neutrophil microabscess was seen in cases with a long course of illness. Dilated vascular spaces in dermis containing numerous large typical histiocytes were seen in 2 cases.


CONCLUSIONS
CRDD is a benign, persistent proliferative disease of histiocytes. Systemic involvement is rare, outcome favorable. It should be differentiated from other types of histiocytosis, dermatofibrosarcoma protuberans, xanthoma and lymphoproliferative disorders. Immunohistochemical staining for S-100 protein and CD68 is helpful in making a correct diagnosis.
Kong
Yun-yi
YY
Department of Pathology, Cancer Hospital, Fudan University, Shanghai 200032, China. kyydb@yahoo.com.cn
Lu
Hong-fen
HF
Zhu
Xiong-zeng
XZ
Wang
Jian
J
Shi
Da-ren
DR
Kong
Jin-cheng
JC
D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D015214:Antigens, Differentiation, Myelomonocytic / Q000378:metabolism; D003937:Diagnosis, Differential; D005260:Female; D015618:Histiocytosis, Sinus / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D009418:S100 Proteins / Q000378:metabolism; D012871:Skin Diseases / Q000378:metabolism / Q000473:pathology* / Q000601:surgery; D000097382:CD68 Molecule
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D009418:S100 Proteins; C067980:CD68 antigen, human; D000097382:CD68 Molecule