Cutaneous Rosai-Dorfman disease: histopathological presentation as inflammatory pseudotumor. A literature review.
Purely 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.
D000328:Adult; D015703:Antigens, CD / Q000032:analysis; D018949:Antigens, CD1 / Q000032:analysis; D015214:Antigens, Differentiation, Myelomonocytic / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D006104:Granuloma, Plasma Cell / Q000473:pathology*; D015618:Histiocytosis, Sinus / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D009113:Muramidase / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D012871:Skin Diseases / Q000473:pathology*; D000514:alpha 1-Antichymotrypsin / Q000032:analysis; D000515:alpha 1-Antitrypsin / Q000032:analysis; D000097382:CD68 Molecule
D015703:Antigens, CD; D018949:Antigens, CD1; D015214:Antigens, Differentiation, Myelomonocytic; D009113:Muramidase; D009418:S100 Proteins; D000514:alpha 1-Antichymotrypsin; D000515:alpha 1-Antitrypsin; C419393:CD1a antigen; C067980:CD68 antigen, human; D000097382:CD68 Molecule