What Is Rosai-Dorfman Disease?

Rosai-Dorfman disease, also known as sinus hisliocytosis with massive lymphadenopathy (SHML), is a benign lymphoproliferative disease, first reported by Azoury and Reed in 1966. Rosai and Reed Dorfman studied it in detail and officially named it

Rhodes disease

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Rosai-Dorfman disease, also known as sinus hisliocytosis with massive lymphadenopathy (SHML), is a benign lymphoproliferative disease, first reported by Azoury and Reed in 1966. Rosai and Reed Dorfman studied it in detail and officially named it

Western Medicine Name

Sinus histiocytosis with giant lymphadenopathy

Chinese Medicine Name

Rhodes disease

English name

Rosai-Dorfman

Other name

Benign lymphoproliferative disease

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy More common in children and young people; Painless masses of bilateral cervical lymph nodes with fever, increased ESR, neutrophils, and polyclonal highballs Proteinemia; Lymph node structure is partially or incompletely destroyed, and lymphoid follicles disappear. The prominent morphology is that the lymphatic sinus is dilated obviously, and the sinus is filled with tissue cells and a small number of small lymphocytes and neutrophils. Tissue cells have obvious phagocytosis, phagocytosis of lymphocytes (lymphocytes), red blood cells, nuclear debris, lipids (foam cells), mature and differentiated tissue cells, occasionally mild atypia, rare mitotic figures, and strong S-100 protein Positive. The obvious phagocytosis of the cell, the nucleus is not grooved, twisted and defoliated, and the negative of CD1a (O10) can make it different from LCH.

Typical symptoms are painless enlargement of bilateral cervical lymph nodes with fever, elevated neutrophils, accelerated erythrocyte sedimentation, and hyperglobulinemia.

Approximately 40% of cases involve other organs other than the nodule (almost any organ can be involved), which rarely occurs on the skin alone.

Lymph nodes: single lymph nodes or multiple nodular masses, the larger diameter of which can reach 5-6cm, the texture is hard, the cut surface is more uniform, and many yellow spots are visible.

Skin: pimples or nodules, skin lesions bulging or infiltrating downward, cut surface is gray and yellow.

Intranodal: The outline of the lymph nodes is still present, the lymph follicles are atrophied, and the germinal center is not obvious. The lymphatic sinus is highly dilated, and the sinus is filled with proliferating mononuclear or multinuclear tissue cells, accompanied by lymphocytes, plasma cells, and neutrophils. Tissue cells have the same morphology and are well differentiated. Nuclei are large, vacuole-like, round or oval. The cytoplasm is richly reddish. Most of the intact lymphocytes, plasma cells, and neutrophils (emperipolesis) can be seen in the cytoplasm.

Skin: Similar to nodular lesions. A large amount of infiltration of histological cells is seen in the dermis, with infiltration of scattered lymphocytes, plasma cells, and neutrophils. Emperipolesis is common.

Rosai-Dorfman disease is a rare disease. Most cases have lymphadenopathy, so it is also called sinus histiocytosis with massive lymphadenopathy. It is very rare to be confined to the epidermis, so it is easy to misdiagnose. This article reports Rosai-Dorfman disease in a 35-year-old woman. This lesion has multiple tumors that are confined to the epidermis of the left breast. The largest tumor is 5x2x1 cm. The patient was clinically suspected of having a recurrent breast tumor because the patient had a complete tumor resection in the same area two and a half years ago. But four pathological sections were diagnosed as chronic inflammation. The diagnosis of the diagnosis was confirmed by the appearance of typical histological cells in the last pathological section. This typical spheroid cell is positive for S100 protein and phagocytoses many lymphocytes and plasma cells.

Rosai-Dorfman disease (RDD), also known as idiopathic sinus histiocytosis giant lymphadenopathy, is a rare benign disease of unknown etiology. Most are self-limiting and can subside on their own within a few months to years. A few cases have a prolonged course of disease, or they have died of multiple organ invasion.

The majority of patients were adolescents, with no gender differences.