伴抗基底膜自身抗体的皮肤异色症样淀粉样变病1例

报道1例伴抗基底膜自身抗体阳性的皮肤异色症样淀粉样变病,并对相关文献进行复习.患者女,22岁.面颈部、躯干、四肢出现瘙痒性红斑、水疱及弥漫性网状色素沉着性斑片8年余.皮损组织病理示：表皮角化过度,基底细胞液化变性,真皮乳头均一化物质沉积,结晶紫染色阳性;直接免疫荧光（DIF）示：基底膜带及乳头IgG、IgM、IgA和C3沉积.     

皮肤异色病样淀粉样变病二例

患者1,男,49岁,病程9年;患者2,女,21岁,病程3年。两例患者均表现为全身弥漫性色素沉着夹杂点状白斑,伴苔藓样丘疹,偶有水疱。家族中无类似病史。组织病理示：真皮乳头层淀粉样物质沉积。     

皮肤异色病样淀粉样变病2例

例1于17岁发病,皮损首发于四肢,家族有类似患者;例2于8岁发病,皮损首发于两前臂。2例患者临床表现均为皮肤弥漫性色素沉着伴点状色素减退斑。皮损组织病理示:真皮乳头可见类淀粉样物质沉积。     

皮肤异色病样淀粉样变1例

报告1例皮肤异色病样淀粉样变。患者，男，22岁。四肢躯干色素沉着斑，减退斑，毛细血管扩张及轻度萎缩20余年，其弟也有类似现象。病理切片行甲基紫染色，可见真皮乳头有类淀粉样物质。     

皮肤异色病样淀粉样变病1例

皮肤异色病样淀粉样变病（poikiloderma-like cutancous amyloidosis,PCA）是一种少见的皮肤病。现将我科诊治的1例报道如下。     

皮肤异色病样淀粉样变1例

报告1例皮肤异色病样淀粉样变。患者,男,47岁。全身弥漫色素沉着、色素减退斑、丘疹、水疱、瘙痒11年。皮损组织病理活检：丘疹区及水疱区均见真皮乳头,内见均质嗜伊红物质沉积,甲基紫染色阳性。根据临床表现及皮肤组织病理,诊断为皮肤异色病样淀粉样变。     

皮肤异色病样淀粉样变病1例

1病历摘要 患者女,25岁。12年前无明显诱因双胫前,背部皮肤出现苔藓样丘疹和小水疱,不痛不痒,未予以注意。后丘疹,水疱反复出现,能自行消退,消退后皮肤遗留褐色色素沉着,其上散在绿豆大色素减退斑。3个月前,双上肢又出现苔藓样丘疹和小水疱,为明确诊断于2004年6月15日来我科就诊。患者自发病以来无其他不适,否认家族中有类似疾病患者。     

皮肤异色病样淀粉样变病2例

例1.男,25岁。因全身皮肤色素沉着伴色素减退16年,于2004年3月来我院诊治。患者9岁时无任何诱因,全身皮肤变黑、轻痒,但能忍受,曾先后多次在各大医院诊治,均无效。自觉皮肤颜色逐渐加深,夏季日晒后,面部、双上臂伸侧起水疱。家族中无类似疾病史。体格检查:各系统检查均未见异常。皮肤科检查:全身皮肤呈弥漫性深褐色,夹杂散在点状色素减退斑,双上肢伸侧皮肤粗糙,上有苔藓样丘疹及针尖大水疱。皮损组织病理A:颈、胸部弥漫性色素沉着斑,夹杂大小不一的色素减退斑,表面粗糙,有鳞屑;B:上肢伸侧散在少许针帽至针尖大水疱、抓痕、血痂图1皮肤异色…     

一般型皮肤异色病样淀粉样变11例分析

目的:分析一般型皮肤异色病样淀粉样变(PLCA)的临床特点。方法:收集总结我院11例经组织病理确诊的一般型皮肤异色病样淀粉样变患者的临床资料(病史、皮损特点、治疗及转归等)进行分析。结果:11例均为成人期发病。首发部位均在四肢,9例日晒后加重,7例伴有苔藓样丘疹,3例伴有水疱,3例有家族史。结论:在临床中,表现为皮肤异色、苔藓样丘疹,首发于四肢,无智力低下和身材矮小,需考虑一般型PLCA的可能。     

皮肤异色病样淀粉样变

皮肤异色病样淀粉样变是一种家族性原发性皮肤淀粉样变。表现为躯干、四肢的网状色素沉着和色素减退。现将最近我科诊治的1例皮肤异色病样淀粉样变报道如下：     

皮肤异色病样淀粉样变病

皮肤异色病样淀粉样变是原发性皮肤淀粉样变的少见类型,国内外关于本病的报道较少;多种因素参与皮肤异色病样淀粉样变发病,明确诊断需组织病理的支持,必要时需行电镜观察,且需与有皮肤异色样变表现的其他疾病相鉴别.基于已报道的病例,就该病病因、临床特点、组织病理、诊断及鉴别诊断等方面进行探讨.     

Anosacral cutaneous amyloidosis: a study of 10 Chinese cases

BACKGROUND: Primary cutaneous amyloidoses are rare in Western countries, but are relatively common in Taiwan. Anosacral cutaneous amyloidosis is a rare type of primary cutaneous amyloidoses, first reported in Japanese patients. PATIENTS/METHODS: In the present study, we investigated the age of onset, sites of involvement, associated systemic diseases, and histopathological findings in 10 cases of anosacral cutaneous amyloidosis seen during the past 27 years. RESULTS: In previous reports the aetiology of anosacral cutaneous amyloidosis was thought to be a senile change, but half of our patients developed the disease before the age of 60 years. Based on our histopathological findings, apoptosis may be the initial event causing amyloid deposition, although the precise mechanism causing apoptosis needs further investigation. Three patients were found to have diabetes mellitus, but any relationship to anosacral cutaneous amyloidosis is unclear. CONCLUSIONS: No cases of this cutaneous disorder have been reported in the Western literature; there seems to be a racial difference accounting for the disease, although the precise factor is not clarified yet. The disease could easily be misdiagnosed as lichen simplex chronicus, postinflammatory hyperpigmentation or tinea cruris; therefore, a thorough history, a careful physical examination and a skin biopsy is needed to establish a firm diagnosis.     

原发性结节性皮肤淀粉样变研究进展

原发性结节性皮肤淀粉样变是原发性皮肤淀粉样变的最少见亚型,其特征为蜡样光泽的黄棕色结节或斑块,组织病理改变为在真皮、皮下及血管壁弥漫性淀粉样蛋白沉积。研究发现,结节性淀粉样变可能与创伤、IL-6家族细胞因子受体基因突变、免疫功能异常等有关。手术切除、电干燥法、刮除术、冷冻术、局部糖皮质激素注射和皮肤磨削术为结节性淀粉样变的主要治疗手段。近年来研究发现局部注射淀粉样蛋白抗体的免疫疗法有望成为该病新的有效的治疗手段。     

Case of primary localized cutaneous amyloidosis with protean clinical manifestations: lichen, poikiloderma-like, dyschromic and bullous variants

Primary localized cutaneous amyloidosis (PLCA) commonly presents as macular and lichen variants. We present a case of a 27-year-old Chinese woman with cutaneous features of the rarely reported poikiloderma-like, dyschromic and bullous forms of PLCA, and the commoner lichen variant. There were no syndromic associations or systemic involvement, and the various morphological subtypes occurred in isolation from one another. We review the clinical spectrum of PLCA, highlight its protean clinical manifestations in this patient, and discuss its postulated pathogenesis in relation to its histopathological features.     

特殊部位的原发性皮肤淀粉样变性3例

报告3例部位特殊的原发性皮肤淀粉样变性。3例患者皮损分别发生于外耳、乳房及额部,由小而密集的褐色斑疹或丘疹组成,或呈苔藓样改变,不伴或伴有瘙痒。皮损组织病理检查示:真皮乳头层淀粉样蛋白沉积,刚果红和结晶紫染色阳性。诊断:原发性皮肤淀粉样变性。     

A monoclonal anti-keratin antibody reactive with amyloid deposit of primary cutaneous amyloidosis

Specimens from cutaneous amyloidoses (lichen amyloidosis and macular amyloidosis) were stained immunohistochemically with monoclonal anti-keratin antibodies. One monoclonal antibody raised against hair keratin (HKN-6) reacted with the amyloids of both primary amyloidoses. Another monoclonal antibody, HKN-2, did not decorate the amyloid deposit. HKN-6 did not stain the interfollicular epidermis, but HKN-2 did. The possible explanations of these findings are 1) amyloid deposits contain keratin protein modulated to react with HKN-6; 2) amyloid deposits contain a protein unrelated to keratin protein, but reactive to HKN-6.     

Suggestive linkage of familial primary cutaneous amyloidosis to a locus on chromosome 1q23

BACKGROUND: Large or deteriorated skin defects are sometimes life threatening. There is increasing evidence that adult stem cells are useful for tissue regeneration. Human mesenchymal stem cells (hMSCs) are self-renewing and are potent in differentiating into multiple cells and tissues. OBJECTIVES: To investigate the effects of hMSCs in cutaneous wound healing. METHODS: Wound healing was studied in an hMSC-populated porcine skin substitute, using a nude rat model to minimize immune reactions. Full-thickness skin and soft tissue defects of 1.5 x 1.5 cm in size, including the panniculus carnosus, were excised and covered with hMSCs and basic fibroblast growth factor (bFGF)-soaked skin substitutes and an evaluation was made of wound size, histology and protein expression at 3, 7 and 42 days after injury. RESULTS: The wound size was significantly smaller in the hMSC-treated groups (P < 0.01) and any dose of bFGF (1, 10, 100 microg) enhanced the healing (P < 0.01). The re-epithelialization markers integrin alpha3 and skin-derived antileucoproteinase were remarkably increased with the presence of bFGF in a dose-dependent manner, while the mesenchymal cell surface markers CD29 and CD44 were downregulated in a time-dependent manner. Human pancytokeratin, which does not cross-react with rat antigens, was observed by Western blotting at 38 kDa and 42 kDa from the hMSC-treated tissues on day 7. The expression levels were elevated by 10 microg bFGF (P < 0.01). The immunohistochemical expression of human pancytokeratin was only observed in the hMSC-treated groups. CONCLUSIONS: These data suggest that hMSCs together with bFGF in a skin defect model accelerate cutaneous wound healing as the hMSCs transdifferentiate into the epithelium.     

Nodular localized primary cutaneous amyloidosis: a long-term follow-up study

We present long-term follow-up data on patients with nodular localized primary cutaneous amyloidosis (NLPCA) seen at the St John's Institute of Dermatology between 1968 and 1999. This is the largest clinical follow-up study of this type of amyloid to date. Based on these cases we estimate the rate of progression of NLPCA to systemic amyloidosis to be only 7%, much lower than the 50% rate currently quoted in the literature.     

Primary cutaneous amyloidosis of the auricular concha: case report and review of published work

A 70-year-old Japanese female developed tiny papules on her bilateral ears 2 years previously. A histological study of a biopsy specimen revealed that amorphous materials were present in the widened dermal papillae. Because these materials were positive for both Congo red and Dylon, we diagnosed the lesion as primary cutaneous amyloidosis of the auricular concha. Immunohistochemically, the amyloid substance stained positively with 34betaE12 (cytokeratin 1/5/10/14), suggesting that it had an epidermal origin. Seven reported cases of this unique disorder were also reviewed.