结节性痒疹样原发性皮肤淀粉样变1例

皮肤淀粉样变是临床上的常见病 ,但以泛发性类似结节性痒疹临床表现者极为少见 ,现将我们发现的 1例报告如下。1　临床资料患者 ,女 ,34岁 ,农民。因全身泛发瘙痒性结节 10年来诊。患者 10年前无明显原因 ,双足踝部出现淡红色粟粒大丘疹、痒 ,抓好皮损扩大 ,颜色加深 ,日久腹部、背部、上肢出现同样损害 ,瘙痒加重 ,影响入眠。在当地县级医院多次诊为“结节性痒疹” ,曾口服强的松 (量不详 )及中草药治疗 ,痒感一度减轻 ,但皮损无明显消退。既往健康 ,家族中无类似疾病。体格检查 :系统检查未见异常。皮肤科情况 :躯干、四肢散在分布半球形…     

结节性皮肤淀粉样变一例

患者,男,46岁。左侧鼻唇沟、鼻翼及下颌结节1年。皮肤组织病理：真皮内可见大量嗜酸性、无定形物质沉积,甲紫染色和刚果红染色均阳性。诊断：结节性皮肤淀粉样变。给予复方倍他米松局封后结节缩小。     

结节性皮肤淀粉样变病1例

患者女,48岁。右腋下大小不等的结节样斑块十余年,皮损表现为淡黄色、质硬的斑块和结节。组织病理学检查示:表皮下有不规则的胶样物质沉积。刚果红染色阳性。诊断:结节性皮肤淀粉样变病。     

结节性原发性皮肤淀粉样变1例

患者男,36岁。右上唇须部小肿物2年余,渐增大,轻痒。组织病理:真皮全层可见较多呈淡粉红色均匀一致的团块状沉淀物,其间有裂隙;结晶紫染色淀粉样蛋白呈鲜明紫红色;透射电镜真皮内小血管周围及胶原纤维间可见纤细、排列无序的淀粉样纤维。诊断:结节性限局性原发性皮肤淀粉样变。经90锶局部贴敷治疗消退。     

结节性皮肤淀粉样变1例

报告1例结节性皮肤淀粉样变。患者男,61岁。前额发际处多发淡黄色结节性丘疹,光滑,边界清楚2年。组织病理:HE染色显示表皮萎缩变薄,表皮突消失变平;真皮全层及皮下组织有大块均一红染、团块状的淀粉样蛋白沉积;血管壁也有淀粉样蛋白沉积;刚果红染色显示橙红色阳性;偏振光检查可见浅绿色的双折光。     

限局性结节性原发性皮肤淀粉样变1例报告

患者男性，65岁脚趾长小肿物4个月，并逐渐长大，无明显痒痛感。体格检查：头、面、躯干及四肢皮肤肤色正常，未见明显斑块、结节、硬化、瘀斑及色素沉着。舌体大小及色泽正常，毛发及甲无改变。皮科情况：左足2—5趾间，右足3—4趾间内外侧缘，见散个淡紫红色绿豆至黄豆大疣状肿物，表面光滑，质地柔软，呈串珠样排列。     

原发性皮肤淀粉样变研究进展

原发性皮肤淀粉样变(PCA)是一种多病因的综合征,主要表现为真皮乳头层大量的淀粉样蛋白沉积.本病属常染色体显性遗传,可能存在遗传异质性.最新的遗传学研究将其致病基因定位于染色体1q23和染色体5p13.1-q11.2两个区域.临床上主要分为丘疹型(苔藓样)、斑疹型、结节型(肿胀型)和家族性原发性限局性皮肤淀粉样变.皮损好发于两小腿胫前、臂外侧、背部、大腿、躯干和颈部.典型的临床表现为针头大小的褐色斑点,渐变为针头样丘疹,以后发展为苔藓状或结节状或皮肤异色,可伴有剧烈瘙痒.本文就其遗传机制、临床表现、诊断及治疗等做一综述.     

鼻部结节性皮肤淀粉样变一例

正临床资料患者,男,39岁。主因上唇及鼻部红色结节、肿块3年,于2014年7月就诊。3年前,患者右鼻部出现数个淡褐红色浸润性结节及斑块,无明显自觉症状,皮损逐渐扩大并相互融合,向下蔓延至上唇部,形成凹凸不平的蜡样褐红色斑块,从未破溃(图1)。数周前曾在其他医院行皮损组织病理检查,考虑黏液性水肿性苔藓,未治疗。患者一般健康状况良好,生活基本规律,否认不良嗜好。家族中无类似疾病患者。     

原发性皮肤淀粉样变伴发白癜风1例

1病例男，7—8年前双小腿伸侧、左大腿前面出现绿豆大小的褐色半球形丘疹，表面粗糙，质硬，双小腿皮损排列成串珠状，左大腿皮损密集成片，自觉剧痒。在我省和平医院做病理确诊为原发性皮肤淀粉样变。给予口服抗组胺药及外用激素类药膏，皮损无明显改变。2年前，在皮损处出现绿豆至黄豆大小的白斑，渐融合成一大片。近1年来，在后     

52例原发性皮肤淀粉样变病因分析

目的：明确原发性皮肤淀粉样变患者发病相关因素。方法：对2018年3月至2019年1月我院52例原发性皮肤淀粉样（PCA）变患者及104名正常对照的资料进行分析。结果：多因素 Logistic回归分析显示原发性皮肤淀粉样变的发病因素中日光暴晒、搔抓、过敏为危险因素,OR值分别为3.044,9.254,4.653,P均<0.05。合并疾病中,湿疹、荨麻疹、过敏性鼻炎发病率在PCA患者与104名健康正常对照间比较,差异有统计学意义（P<0.05）。结论：日光暴晒、搔抓、过敏与原发性皮肤淀粉样变的发病有关,患者易合并湿疹、荨麻疹、过敏性鼻炎。     

原发性结节型皮肤淀粉样变性

报告1例原发性结节型皮肤淀粉样变性。患者男,41岁,双侧鼻孔缘下斑块、结节4年就诊,无自觉症状。组织病理检查:表皮基底细胞轻度液化变性,真皮浅、深层及皮下组织胶原纤维间可见嗜酸性物沉积,血管壁见类似改变。结晶紫和刚果红染色阳性,PAS染色弱阳性,偏振光显微镜下呈苹果绿双折光。诊断:原发性结节型皮肤淀粉样变性。     

结节、斑疹双相型皮肤淀粉样变一例

患者女,40岁,腰、腹部结节14年,无自觉症状,进行性加重;上背部色素性斑疹,痒十余年,于1989年来我院首诊,给予手术切除并行病理检查,诊断为结节型皮肤淀粉样变.最近因腰、腹部出现新的结节、斑块,背部出现褐色斑疹,痒,再次来我院就诊.既往体健,家族中无类似病史.皮肤科检查:双肩胛间见片状褐色斑疹,轻度肥厚(图1);腰、腹、臀部数个大小不等的结节、斑块,黄豆粒至红枣大小,呈黄红色,具蜡样光泽,部分区域有萎缩、紫癜及色素沉着,质硬,无触痛(图2).     

Extensive nodular cutaneous amyloidosis: an unusual presentation

Amyloidosis is characterized by the deposition of a group of unrelated proteins leading to changes in tissue architecture and function. The nodular variant is the rarest form of the cutaneous amyloidoses. We report a patient with localized nodular amyloidosis without systemic amyloid involvement or paraproteinaemia after 6 years of follow-up. The unusual aspects of our case were a plaque presentation rather than nodular, and the disseminated pattern observed.     

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。     

原发性皮肤淀粉样变皮肤镜特征分析

目的 描述原发性皮肤淀粉样变的常见皮肤镜特征,探讨皮肤镜在原发性皮肤淀粉样变辅助诊断中的应用价值.方法 收集2014年4月至2016年12月在北京协和医院皮肤科门诊就诊并行皮肤镜检查的原发性皮肤淀粉样变患者17例,分析45处皮损的皮肤镜特征.结果 原发性皮肤淀粉样变常见的皮肤镜特征为存在中心区域(可为白色、棕色或瘢痕样结构)和多种形态的色素结构及亮白色条纹.45处皮损(100％)皮肤镜下均观察到中心区域.14处(31％)苔藓样皮损仅有白色中心区域,5处(11％)苔藓样皮损同时具有白色中心区域和瘢痕样结构.8处(18％)斑状皮损仅有棕色中心区域,6处(13％)苔藓样皮损和17处(38％)斑状皮损同时具有白色及棕色中心区域.所有皮损均有多种形态的色素结构.4处(9％)苔藓样皮损有亮白色条纹.结论 皮肤镜在原发性皮肤淀粉样变的辅助诊断中具有良好的应用价值.     

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.     

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.     

原发于头皮的皮肤淀粉样变一例

原发性皮肤淀粉样变皮损常见于双侧胫前、背部及大腿,也可见于阴茎及面颈部,原发于头皮者少见,诊治具有一定的难度。现报道1例我科诊治原发于头皮的皮肤淀粉样变患者。给予0.1%维A酸外用头皮,治疗2个月,皮损变化不明显,瘙痒缓解,目前进一步随访中。     

Primary localized cutaneous nodular amyloidosis successfully treated with cyclophosphamide

Primary localized cutaneous nodular amyloidosis (PLCNA) is a rare subtype of localized cutaneous amyloidosis and can be associated with various connective tissue disorders. It can be difficult to treat and past therapies include surgical excision, dermabrasion, electrodessication and curettage, cryotherapy and laser therapy. We present a case of a middle‐aged woman with PLCNA associated with CREST (calcinosis, Raynaud phenomenon, oesophageal motility disorders, sclerodactyly and telangiectasia) syndrome and Sjögren's syndrome responding to cyclophosphamide with no new amyloid deposits and resolution of skin ulceration after many years of resistance to drug therapy. It is important to monitor these patients for progression into systemic amyloidosis.