The differential diagnosis of Destombes–Rosai–Dorfman disease includes both malignant and nonmalignant diseases, such as granulomatosis with polyangiitis, Langerhans cell histiocytosis, Langerhans cell sarcoma, lymphoma, sarcoidosis, IgG4-related disease, and tuberculosis. The disease is diagnosed by biopsy of affected tissues. Microscopic examination of stained specimens will show histiocytes with lymphocytes and possibly other types of cells trapped within them, a phenomenon known as emperipolesis. Upon immunohistochemical staining, the histiocytes will be positive for S100, CD68, and CD163 but negative for CD1a. Classification In 2016 the Histiocyte Society proposed a classification of histiocytoses into five groups designated by letters: "C", "H", "L", "M", and "R". Group "R" included<unk>  and "miscellaneous noncutaneous, non-Langerhans cell histiocytoses".<unk>  itself was classified into "Familial", "Classical (nodal)", "Extranodal", "Neoplasia-associated", and "Immune disease-associated" subtypes.
Rosai–Dorfman disease
Mowat–Wilson syndrome
Akureyri disease
Wilson–Mikity syndrome
Wilson's disease
BENTA disease
Batten disease
Keshan disease