Ming Wang1, Bilal Abdul-Fattah2, Cuiyan Wang3, Yun Zhao4, Xiaoying Qu5, Maher Al-Muriesh2, Xia Wang1, Li Zhu2, Yue Qian2, Changzheng Huang2, Siyuan Chen2. 1. Department of Dermatology, Shenzhen Nanshan People's Hospital, Shenzhen, China. 2. Department of Dermatology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China. 3. Department of Dermatology, Huizhou Municipal Central Hospital, Huizhou, China. 4. Department of Dermatology, General Hospital of the Yangtze River Shipping, Wuhan, China. 5. Department of Dermatology, Hospital of the People's Liberation Army, Wuhan, China.
Abstract
BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon benign fibrohistiocytic and vascular proliferation, which usually occurs as slow-growing grouped reddish-brown to purple papules and nodules on the distal extremities or face. Patients with generalized MCAH are extremely rare and to our knowledge, there are no more than 11 cases reported previously in the medical literature. OBJECTIVE: To describe the clinical, histopathologic features and immunohistochemical characteristics of all reported cases of generalized MCAH and investigate any potential clinicopathological correlations. METHODS: A systematic review of the literature was done with information collected and organized in a table. A new case report is also described in a 42-year-old female with generalized MCAH. Histopathologic and immunohistochemical features of multiple biopsies were analyzed. RESULTS: Men and women are equally affected. It is crucial to take multiple biopsies preferably from newly formed lesions to reach the correct diagnosis. The divergent results in immunohistochemistry staining for CD68 and estrogen receptor (ER) alpha necessitate further studies to reach a precise etiology and pathogenesis and secure it with certainty. CONCLUSION: Awareness of the clinicopathological hallmarks is important to avoid underdiagnosis of MCAH and the immunohistochemical features may contribute to understanding the pathogenesis of this rare disease.
BACKGROUND: Multinucleate cell angiohistiocytoma (MCAH) is an uncommon benign fibrohistiocytic and vascular proliferation, which usually occurs as slow-growing grouped reddish-brown to purple papules and nodules on the distal extremities or face. Patients with generalized MCAH are extremely rare and to our knowledge, there are no more than 11 cases reported previously in the medical literature. OBJECTIVE: To describe the clinical, histopathologic features and immunohistochemical characteristics of all reported cases of generalized MCAH and investigate any potential clinicopathological correlations. METHODS: A systematic review of the literature was done with information collected and organized in a table. A new case report is also described in a 42-year-old female with generalized MCAH. Histopathologic and immunohistochemical features of multiple biopsies were analyzed. RESULTS:Men and women are equally affected. It is crucial to take multiple biopsies preferably from newly formed lesions to reach the correct diagnosis. The divergent results in immunohistochemistry staining for CD68 and estrogen receptor (ER) alpha necessitate further studies to reach a precise etiology and pathogenesis and secure it with certainty. CONCLUSION: Awareness of the clinicopathological hallmarks is important to avoid underdiagnosis of MCAH and the immunohistochemical features may contribute to understanding the pathogenesis of this rare disease.
Authors: Anderson Alves Costa; Glaucia Ferreira Wedy; Walter Belda Junior; Paulo Ricardo Criado Journal: An Bras Dermatol Date: 2020-05-11 Impact factor: 1.896