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lüll Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor A literature review Kroumpouzos G; Demierre MFActa Derm Venereol 2002[]; 82 (4): 292-6Purely cutaneous Rosai-Dorfman disease is exceptional. The disease is characterized histologically by large, proliferating histiocytes exhibiting inflammatory cells within their cytoplasm (emperipolesis). We present here a case of purely cutaneous generalized disease in which the routine histopathology was suggestive of an inflammatory pseudotumor. Positivity for S-100 protein, alpha1-antitrypsin, alpha1-antichymotrypsin, lysozyme, Mac387 and CD68 proteins, and negativity for CD1a protein confirmed the diagnosis of Rosai-Dorfman disease. The rarity of this case lies in the presence of conspicuous inflammatory pseudotumor-like histopathologic changes, masking an otherwise typical sinus histiocytosis cell infiltrate. This unusual presentation of the disease requires a high index of suspicion by clinicians and pathologists.|Adult[MESH]|Antigens, CD/analysis[MESH]|Antigens, CD1/analysis[MESH]|Antigens, Differentiation, Myelomonocytic/analysis[MESH]|Diagnosis, Differential[MESH]|Female[MESH]|Granuloma, Plasma Cell/*pathology[MESH]|Histiocytosis, Sinus/*pathology[MESH]|Humans[MESH]|Immunohistochemistry[MESH]|Muramidase/analysis[MESH]|S100 Proteins/analysis[MESH]|Skin Diseases/*pathology[MESH]|alpha 1-Antichymotrypsin/analysis[MESH]|alpha 1-Antitrypsin/analysis[MESH] |