表皮痣一家系报道并文献复习

发布时间：2018-08-14 19:56

【摘要】：非器官样型表皮痣,简称表皮痣,又称疣状痣,是表皮角质形成细胞异常增殖形成的一种皮肤错构瘤,表现为局限或泛发的集簇性分布的肤色、棕褐色或者灰褐色的疣状丘疹,皮损可融合成边界清楚的乳头瘤样斑块,四肢的皮损大多沿Blaschko线分布。根据临床特点及皮损范围可将表皮痣分为三个亚型：局限型、泛发型和炎性线状表皮痣。本病多散发,罕见家族发病者。现报告3例有家族史的表皮痣患者并复习相关文献。患者A,男,22岁,出生后10天左右肛周皮肤出现绿豆粒大小疣状高起皮疹,之后逐年增大、增多,发展为全身多处疣状密集的褐红色丘疹,部分融合成斑块,呈带状分布。自感肛周皮损瘙痒难忍,便后尤甚。皮损活检示：表皮角化过度、角化不全,棘层增生肥厚,皮突下延交织,基底细胞色素增加；真皮浅层血管扩张,单一核细胞灶状浸润。结合临床诊断：泛发性炎性表皮痣。患者B,男,2岁,其母为A的表姐,自出生肛周即出现与A类似皮损。皮损活检示与A类似。患者C,女,系A的母亲,右侧鼻唇沟处局限性融合性黄白色角化性丘疹,自幼即有。诊断：局限型表皮痣。该家族中共3代5例罹患此病。本文通过对相关文献复习,综述了表皮痣的流行病学特点、病因及发病机制、临床分型、组织病理学特点、鉴别诊断及治疗方法。
[Abstract]:Non-organ-like epidermal nevus, also called verrucous nevus, is a skin hamartoma formed by abnormal proliferation of epidermal keratinocytes. It is characterized by localized or generalized clustered skin color, brown or grayish-brown verrucous papules, and the lesions can fuse into well-defined papillomatous plaques. Most of the skin lesions of the extremities are along Blas. The epidermal nevus can be divided into three subtypes according to the clinical features and lesion extent: localized, generalized and inflammatory linear nevus. The disease is more sporadic and rarely occurs in a family. Three cases of epidermal nevus with family history were reported and the relevant literature was reviewed. Patient A, male, 22 years old, presented with large mung bean granules on the perianal skin about 10 days after birth. The skin lesion biopsy showed that the epidermis was hyperkeratosis, incomplete keratosis, hypertrophy of the spinous layer, interlaced subcutaneous processes and increased basal cytochrome. Superficial dermal vasodilation with focal mononuclear infiltration. Combined with clinical diagnosis: generalized inflammatory epidermal nevus. Patient B, male, 2 years old, mother of A's cousin, had a skin lesion similar to A since birth around the anus. Skin biopsy showed a similar lesion to A. Patient C, female, mother of A, localized fusion of yellowish-white keratotic papules at the right nasolabial groove, i.e. at an early age. Diagnosis: localized nevus of the epidermis. There are 5 cases of this disease in this family of three generations. This paper reviews the epidemiological characteristics, etiology and pathogenesis, clinical classification, histopathological characteristics, differential diagnosis and treatment of epidermal nevus.
【学位授予单位】：山东大学
【学位级别】：硕士
【学位授予年份】：2012
【分类号】：R758.51

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