Mutreja Deepti, Purohit Abhishek, Singh Pawan K, Pati H P
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.
Indian J Pathol Microbiol. 2012 Oct-Dec;55(4):566-8. doi: 10.4103/0377-4929.107824.
Systemic mastocytosis is a rare clonal disorder characterized by mast cell infiltration of one or more organs, with or without skin involvement. Leonine facies is a rare presentation and corresponds to the morphologic manifestation of diffuse dermal infiltration of the face as a result of long standing disease. Bone marrow aspiration and biopsy findings in a 60-year-old woman, who had extensive cutaneous infiltration due to systemic mastocytosis, resulting in 'leonine facies,' are described, and causes of leonine facies are discussed.
系统性肥大细胞增多症是一种罕见的克隆性疾病,其特征为一个或多个器官有肥大细胞浸润,可伴有或不伴有皮肤受累。狮面是一种罕见的表现,对应于长期疾病导致面部弥漫性皮肤浸润的形态学表现。本文描述了一名60岁女性因系统性肥大细胞增多症导致广泛皮肤浸润而出现“狮面”的骨髓穿刺和活检结果,并讨论了狮面的病因。