进行性交叉性偏侧萎缩症1例

患者男,25岁。左侧颌面部缓慢萎缩十余年,右侧头部皮肤萎缩变硬2年。皮肤科情况:患者左侧颌面部、舌肌、前胸及右下肢多处皮肤肌肉萎缩并变薄,肤色略深;右侧头顶颅骨略凹陷,头皮变薄,呈带状分布,延及右前额及右眼眶,触之皮革样硬,表面光滑,伴患处头发及眉毛脱落;右上臂内侧及左侧背部可见淡褐色片状斑块;右足拇趾明显短缩。皮损组织病理示:表皮略变薄,基底细胞层色素颗粒增多;真皮胶原纤维增生,部分均质化,真皮血管周围灶状淋巴细胞浸润。诊断:进行性交叉性偏侧萎缩症。

A Case of Progessive Crossed Hemiatrophy

A 25-year-old man presented with slow atrophy on the left side of jaw over 10 years,and with rigid skin atrophy on the right side of the head for 2 years.Dermatology examination showed:multiple thinned shriveled skin and muscle could be found on the left side of jaw,tongue muscl,chest and the right leg.The complexion of the lesions became slightly deeper.A slightly umbilicate skull on the right side of the head which extened to the right forehead and eye as the zonal distribution.The scalp got thinner,Smoother or harder with hair and the right eyebrow shed.The hazel flake plaque could be found on the left side of back and the inside of the right upper arm.The right foot toe was distinctly shortening.Histopathology showed the epidermis got slightly thinner with the basal cell layer of pigment particles increased.Perivascular foci-like lymphocytes infiltrated and the increased collagen fibers were homogenization partially in the dermis.The final diagnosis was progessive crossed hemiatrophy.