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Updated: Aug 15, 2026
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Published on: June 15, 2020
[Kimura disease and epithelioid hemangioma: a comparative study of 12 cases]
Lei Lu1, Ren-gui Chen, Xiao-qiu Li
1Department of Pathology, Shanghai TCM-Integrated Hospital, Shanghai 200082, China.
Insights
Kimura's disease (KD) and epithelioid hemangioma (EH) are distinct conditions. KD involves lymphoid hyperplasia, while EH is a benign vascular tumor, requiring careful clinicopathologic differentiation.
Area of Science:
- Pathology
- Dermatology
- Oncology
Background:
- Kimura's disease (KD) and epithelioid hemangioma (EH) are rare conditions that can present with similar clinical features.
- Distinguishing between KD and EH is crucial for appropriate diagnosis and management.
Purpose of the Study:
- To investigate the clinicopathologic features and immunophenotypes of Kimura's disease and epithelioid hemangioma.
- To aid in the differential diagnosis between these two entities.
Main Methods:
- Evaluation of nine cases of KD and three cases of EH using light microscopy and immunohistochemistry.
- Detailed analysis of clinical presentation, histopathology, and immunophenotypic markers.
Main Results:
- Kimura's disease predominantly affects males with subcutaneous nodules and lymphadenopathy, characterized by lymphoid hyperplasia and eosinophilic infiltration.
- Epithelioid hemangioma typically affects females, presenting as small skin nodules or plaques with prominent vascular proliferation lined by epithelioid endothelial cells.
- Immunohistochemistry confirmed lymphoid markers in KD and vascular markers (CD31, factor VIII) in EH.
Conclusions:
- Kimura's disease and epithelioid hemangioma are distinct entities with differing underlying pathologies.
- KD is characterized by lymphoid hyperplasia, whereas EH is a benign vascular tumor.
- Recognizing the distinct clinical and morphologic features is essential for accurate differential diagnosis.
Objective:
To investigate the clinicopathologic features, immunophenotypes and differential diagnosis of Kimura's disease (KD) and epithelioid hemangioma (EH).
Methods:
Nine cases of KD and three cases of EH were evaluated by light microscopy and immunohistochemistry.
Results:
In this series, KD occurred predominantly in males, whereas EH had a female predilection. Both KD and EH arose most frequently in the head and neck region. However, KD usually presented as multiple subcutaneous nodules or swellings and was accompanied by lymphadenopathy in some cases. On the other hand, EH appeared only as a small skin nodule or red plaque. Histologically, both lesions may involve the dermis or subcutis. All the 9 KD cases displayed florid hyperplasia of lymphoid tissue, of which, 7 cases exhibited formation of lymphoid follicles and active germinal centers. Proliferation of post-capillary venules were seen between follicles. They were lined by plump or attenuated endothelial cells. Large number of eosinophils aggregated around the vessels or adjacent to the follicles, formation of microabscesses were observed in 3 cases. All the 3 EH cases showed prominent proliferation of vessels (capillary-sized in 2 cases and small to medium-sized in 1 case). The vessels were lined by epithelioid endothelial cells with abundant eosinophilic cytoplasm. The endothelial cells also proliferated within the lumen in 1 case and grew in sheets or cords adjacent to the vessel walls in 2 cases. Some endothelial cells contained intracytoplasmic vacuoles, suggesting formation of primitive vessels. Associated inflammatory component was noted in 2 cases. Lymphoid follicles however were not present and eosinophil infiltration was not as prominent as in KD. Immunohistochemical study in KD revealed B cells in the lymphoid follicles and mostly T cells in the interfollicular regions. In EH, the epithelioid endothelial cells showed strong reactivity to CD31 and factor VIII-related antigen.
Conclusions:
KD and EH are two distinctive entities. The former represents a lymphoid hyperplasia and the latter represents a benign vascular tumor. Recognition of the clinical characteristics and morphologic features of KD and EH is very important in making this distinction.
