增厚型葡萄酒色斑24例临床病理学特征

Hypertrophic port-wine stain:a clinicopathological analysis of 24 cases

目的探讨增厚型葡萄酒色斑(port-wine stain,PWS)的临床及病理学特征、诊断及鉴别诊断。方法收集并分析2012至2018年河南省人民医院确诊的增厚型PWS 24例,复查相关临床及病理学资料,同时采用免疫组织化学和组织化学染色方法检测相关组织成分,并复习文献。结果24例增厚型PWS中,发病部位包括头颈部(20例)、四肢(2例)、躯干(2例)。临床表现主要为无痛性缓慢增长的红色或紫红色斑块、结节,隆起于表面皮肤,呈增厚结节状。镜下可见畸形血管呈蜂窝状、丛状或簇状生长方式,弥漫累及真皮、皮肤附属器、皮下脂肪组织,以及深部骨骼肌;畸形血管管腔扩张,且管腔直径均≥100μm,其中>400μm者18例,均为晚期病变;真皮浅层处以畸形毛细血管为主,数量多少不等,深部可见血管壁增厚、纤维组织增生,弹力纤维及Masson染色提示为畸形静脉样管腔,个别病例含有少量畸形动脉样管腔,且均未见血管内皮细胞增生;其中19例合并表皮萎缩,6例合并慢性脉管炎或表皮溃疡,4例合并毛细血管瘤,4例合并皮脂腺增生,2例合并表皮乳头状增生,2例合并血管角皮瘤。结论PWS是一种较常见的先天性毛细血管畸形,组织学上畸形毛细血管数量减少且位于浅表部位,深部血管管壁增厚以静脉畸形样特征为主,且弥漫累及真皮层及深部骨骼肌,需要与婴幼儿血管瘤、海绵状血管瘤、血管角皮瘤等进行鉴别诊断。

Objective To investigate the clinical and pathologic features,diagnosis and differential diagnosis of hypertrophic port-wine stain(PWS).Methods Cases of hypertrophic PWS,collected from Henan Provincial People′s Hospital between 2012 and 2018,were retrospectively analyzed for their clinical and pathologic features,immunophenotype and histochemical data,and the relevant literature was reviewed.Results Twenty-four cases of PWS were included in this cohort,located in the head and neck region(20 cases),limbs(2 cases),and trunk(2 cases).The clinical presentations were mainly red or purple-red plaques or slow growing,painless nodules,or thickened and raised above the skin surface.Microscopically,deformed blood vessels showed honeycomb-like,plexiform or cluster-like growth pattern,and diffusely involved the dermis,skin appendages,subcutaneous fat tissue,and deep skeletal muscles;The vascular lumen of the deformed vessels was dilated(≥100μm in diameter),and in 18 cases the lumen was greater than 400μm.The superficial dermis mainly contained few deformed capillaries.The deep wall showed thickening of blood vessel wall and fibrous tissue hyperplasia.Elastic fiber and Masson staining indicated abnormal venous vessel,which in some cases contained small amount of abnormal arterioid vessel,without vascular endothelial cell proliferation in all cases.In 24 cases,19 cases had epidermal atrophy,6 with vascular chronic inflammation or epidermal ulcer,4 with capillary hemangioma,4 with sebaceous gland hyperplasia,2 with epidermal papillary hyperplasia and 2 with vascular keratomas.Conclusions PWS is a common congenital capillary malformation.The number of histologically deformed capillaries is reduced and they usually locate in the superficial part.The deep vascular wall is increased with thick venous malformation,diffusely involving the dermis and deep skeletal muscle.Furthermore,PWS needs to be differentiated from infantile hemangioma,cavernous hemangioma and vascular keratomas.