摘要：患者女，22岁。自幼于左面部出现褐色角化性油腻斑，逐渐扩大波及上半身。家族中父亲及一挛生妹妹身上有较轻的类似皮损。查体右眼轻度斜视。皮疹呈环形堤状隆起，褐色角化斑，部分皮疹匐行性延长呈条索状或地图状，与肢体长轴呈平行排列，背部左侧呈单侧分布，其余部位对称分布。组织病理检查示汗孔角化症。

Linear porokeratosis Mibelli: A case report

Chu JingjinTong PanzhuoLi WeijuLi HongfeiWei LiankunZheng Maorong

(Department of Dermatology, The Second Affiliated Hospital， Hebei Medical University,050000)

Abstract A 22 year- old female presented oily brown keratinized macula at left face at birth which increased in size with age. In her family her father and her twin sister had the similar mild lesions. The right eye had slightly oblique vision. The lesions were keratinized, ring , dammed and swelled up, part of those crawled to striplike and maplike and paralleled with the long axes of the limbs. The lesions of left back were distributed only on one side, the other lesions were symmetrical. The characteristics of the histopathologic feature of the lesions confirmed the diagnosis. old female presented oily brown keratinized macula at left face at birth which increased in size with age. In her family her father and her twin sister had the similar mild lesions. The right eye had slightly oblique vision. The lesions were keratinized, ring , dammed and swelled up, part of those crawled to striplike and maplike and paralleled with the long axes of the limbs. The lesions of left back were distributed only on one side, the other lesions were symmetrical. The characteristics of the histopathologic feature of the lesions confirmed the diagnosis.

Key words Porokeratosis, linear

患者女，22岁。主诉头部、面部、颈部、躯干及双上肢淡褐色角化性斑疹19年，于1998年2月26日收入院。患者自3岁始无明显诱因于左颊部出现一钱币大小的褐色斑，周边呈环状隆起，中央低平，表面有角化性油腻性鳞屑。皮疹逐渐扩展，增多，波及头部、面部、颈部、躯干及双上肢(见图1)。近10年来停止发展，但原有皮损干燥、皲裂。右手无名指由于长期慢性炎症致角化肥厚，手指活动受限。曾分别于1995年、1997年先后2次做面部皮肤磨削术，术后半年皮损仍恢复到术前状态。既往史及个人史无异常发现。家族中其父亲及一挛生妹妹身上有类似皮损，但均较轻，呈散在孤立分布的小片皮损。入院体格检查：体温36.6℃，脉搏80次/min，呼吸20次/min，血压13/8kPa。右眼有轻度斜视。心肺(－)，肝脾未触及，余未见明显异常。皮肤科情况：头部、面部、颈部、双上肢及躯干密布淡褐色斑疹及斑片，一种皮疹为散在、环形、堤状隆起的角化性褐色斑丘疹；另一种皮疹则在上述环形斑丘疹的基础上匐行性延长，多呈条索状或图案状，与肢体长轴呈平行排列。背部皮疹位于左侧，呈单侧分布，其余部位对称分布。但腹部以下未见皮疹。皮疹表面有不同程度的角化，覆有油腻样鳞屑及结痂，周边隆起，中央低平或轻度凹陷，以“湖堤状”。右手无名指角化皲裂，指甲污黄肥厚，活动受限(见图1)。入院后查血、尿、粪常规及肝、肾功能均正常，胸部X线拍片、腹部B超、心电图检查均正常。两次病理活检：第一次取右上臂皮疹活检示：表皮上覆疏松角层，未见鸡眼样角化不全栓，部分乳头瘤样细胞增生，真皮浅层散在淋巴细胞及较多噬色素细胞浸润,呈疣状痣样表现。第2次取颈前皮疹活检示：表皮轻度角化过度，可见两处角化不全栓，角栓下方粒细胞消失，棘细胞变性。符合汗孔角化症。

图1左上肢内侧椭圆形、条索状淡褐色环状皮损，与肢体长

轴一致，右手第4、5指红斑角化、脱屑

讨论：

患者单个环形、堤状隆起的褐色角化性斑丘疹，呈典型Mibelli汗孔角化症，其条索状皮疹多符合Mibelli在1968年提出的汗孔角化症皮损的6种分型中的一种——单侧线状型。但本患者皮疹既有单侧分布，又有双侧分布，皮疹既有Mibelli单个典型环状斑丘疹，又有条状图案状，组织病理既有疣状痣样表现，又有汗孔角化症之表现，但未见类似扁平苔藓样皮疹及白细胞破碎性血管炎之表现。鉴于其上述特别之处，建议将此型称为线状汗孔角化症。

(收 稿 1999- 01- 20 修 回 1999- 05- 04)