线状Mibelli汗孔角化症1例

患者女 ,2 2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 of Mibelli and DSAP

Linear porokeratosis and disseminated superficial actinic porokeratosis (DSAP) are clinical variants of porokeratosis. We report the rare coexistence of these two variants. Porokeratosis is a genodermatosis which has at least five different clinical variants; Mibelli, linear, DSAP, plantaris palmaris et disseminata, and punctate (Wade & Ackerman, 1980). All of these manifest the distinctive peripheral keratotic ridge which histologically corresponds to the cornuid lamella. Although lesions of porokeratosis of Mibelli and linear porokeratosis are sometimes seen together in individuals, the coexistence of other porokeratotic variants is rare.     

Linear porokeratosis of Mibelli in monozygotic twin girls

Two monozygotic female twins with linear porokeratosis of Mibelli are described. One had only minimal lesions of the right elbow. The other had a linear lesion of the right arm following Blaschko's lines. Monozygotism was established using DNA fingerprinting. A single-gene defect of variable expressivity involving the clonal development of epidermal cutaneous cells according to the lines of cutaneous embryogenesis is suspected. Dithranol and carbon dioxide laser treatment are discussed.     

Porokeratosis of Mibelli: successful treatment with cryosurgery

Porokeratosis of Mibelli, a disorder of keratinization, should be treated because of the possibility of development of malignant epithelial tumors on the porokeratotic lesions. Liquid nitrogen cryosurgery is considered to be particularly useful in the treatment of precancerous and benign skin lesions. We aimed to evaluate the efficiency of this method in the treatment of porokeratosis of Mibelli. Eight patients with 20 porokeratotic lesions were treated with spray cryosurgery. Each lesion was frozen for 30 seconds with a spray tip after the keratotic borders were removed conically by sharp dissection. Eighteen of the 20 lesions were healed successfully with slight atrophy in one session. One more session was necessary for the other two lesions. Liquid nitrogen cryosurgery may be considered superior to other destructive methods in the treatment of porokeratosis of Mibelli, with advantages such as simplicity, high cure rates, low cost, short treatment period, and few complications.     

手术治疗Mibelli汗孔角化病二例

手术治疗巨大Mibelli汗孔角化症二例郭丙辰　韩娜娜　李金勇 仪晓芹患者1.女,38岁．因臀部疣状增生物22年就诊．患者于22年前右臀部出现一绿豆粒大小疣状增生物,质较硬,缓慢增长,16年前增长速度加快。25岁生一子后,曾行冷冻治疗,未见有效,增生物继续增长,在外院诊为“神经性皮炎”,药物（具体不详）治疗无效,7-8年前又行“局封”治疗,每周一次,共10次,（具体药物不详）,无效且出现瘙痒。我院就诊行病理检查示“汗孔角化症”,行“电离子机治疗”。2月后复发,渐扩展至对侧,呈片状,痒著,有时裂口疼痛,来诊。体检：一般情况尚可,双臀部见地图状皮损,边缘清,周围见堤状隆起,中央皮肤萎缩,局部有裂口,周边隆起部角化明显,周围散在类似小皮损,总面积约28×15㎝（图１）。实验室检查：血常规：白细胞4.0×10９/L,中性粒细胞49.6％淋巴细胞50.4％　．红细胞4.96×1012/L,血红蛋白141g．肝肾功能、尿常规、大便常规在正常范围。病理检查：汗孔角化症。结合临床及病理诊断：汗孔角化症。患者2.女,30岁,左足跟疣状增生物4年就诊,患者于 4年前左足跟部出现疣状增生物,渐增大,稍痒不疼,未行治疗。体检：一般情况好,左足跟部见３cm×３㎝疣状皮损,地图状外观,边缘清,角化明显,无红肿,无触疼（图２）。治疗：患者1,入院后,经积极术前准备,在骶管麻醉＋局部浸润麻醉下,行双臀部皮损扩大切除＋局部转移皮瓣修复术（图３）,扩大范围为沿皮损外缘1cm、深度至皮下组织,术后应用抗感染治疗,病人恢复顺利（图４）。随访一年无复发。患者2,门诊在局麻下,行皮损扩大切除（扩大切除范围及深度如患者1 ）,术后创面缺损行 “V-Y”成形术修补。术后口服抗生素,随访1年无复发。 　　　 图１双臀部见地图状皮损,边缘清,周围见　　　　图２患者2左足跟部见３×３㎝皮损,角化明显堤状隆起,中央皮肤萎缩,局部有裂口,周边隆起部角化明显,周围散在类似小皮损,总面积约28×15㎝　　　　　　　　　　　　　 　　　 图３患者1皮损切除+皮瓣转移修复术后　　　　　　图４患者1术后一个月　　　　　　　　　　　 讨论　Mibelli汗孔角化症系一种少见的汗孔角化病,病程缓慢,损害可持续多年不变,或经过较长的静止间隔后缓慢扩展。老人中通常在萎缩的皮肤上发生鳞状细胞癌,有时40岁的人群中也有类似发生1。金德蕙、王化秀、孙志坚2和黄朝卫、陈兴平3等也曾报道过斑块状汗孔角化症并发鳞癌的病例。目前汗孔角化症的治疗多采用冷冻、激光、磨削、维Ａ酸等,但均有不同程度的复发。鉴于此病有癌变的风险,因此,对于较大面积的汗孔角化症应尽早采取有效方法加以治疗,我们采用手术切除的方法取得较好疗效。     

Genital porokeratosis of Mibelli.

Porokeratosis of Mibelli is a disorder of epidermal proliferation in which many different clinical forms can be distinguished. Two male patients with a localized type of porokeratosis limited to the genitalia are reported. Later in life they developed an annular skin lesion with peripheral keratotic ridge. The histological examination of a biopsy specimen showed the characteristic features of porokeratosis. There was no family history of similar skin disorders and the patients were not on any drugs. Genital porokeratosis is probably underdiagnosed and we believe that these patients should be followed up on account of the precancerous potential of this disease.     

The successful off-label use of photodynamic therapy for classic porokeratosis of Mibelli: case report

Porokeratosis of Mibelli is an uncommon chronic disorder of epidermal keratinization that should be treated because it can undergo malignant change into epithelial tumors on the lesions. At the moment, it represents a therapeutic challenge for dermatologists because of the lack of standardized guidelines about the treatment. Herein, we report a case of classic porokeratosis of Mibelli treated with photodynamic therapy successfully.     

Bowen disease associated with porokeratosis of Mibelli.

A 73-year-old woman developed Bowen precancerous dermatosis in an area of porokeratosis of Mibelli on her leg. This is the fourth reported case of Bowen disease arising from porokeratosis of Mibelli. The recent literature indicates that porokeratosis of Mibelli may be due to an abnormal clone of cells, predisposing affected individuals to development of malignant neoplasms over the involved areas.