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。     

原发性皮肤淀粉样变性

组织病理特征图1A为苏木精-伊红染色,组织病理表现为表皮角化过度,颗粒层和棘层肥厚,表皮突延长,真皮乳头内可见嗜酸性、无定形、含裂隙的团块状物质沉积,其中间杂真皮浅层血管周围存在灶状分布的淋巴细胞和散在的噬黑素细胞浸润.图1B为刚果红染色,显示真皮乳头内的无定形、团块状物质被刚果红染成砖红色,其下方的真皮组织染色较淡.     

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。     

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).     

结节性皮肤淀粉样变性1例

患者女,48岁。右胸部丘疹和结节4年。皮损组织病理示:真皮乳头、网状层、皮下组织及血管周围见大小不一均质性团块样物质沉积,刚果红染色示:橙红色阳性。诊断:结节性皮肤淀粉样变。     

结节萎缩性皮肤淀粉样变性

报告1例结节萎缩性皮肤淀粉样变性.患者女,60 岁.双股伸侧红褐色萎缩性斑块伴疼痛5 年.皮肤科检查:双股伸侧散在分布暗红至红褐色斑块及结节,有轻度萎缩、松弛,萎缩中心局部指压时可见疝样凹陷.皮损组织病理检查:表皮萎缩变薄,真皮全层、皮下组织及血管周围有大小不一均一红染、团块状的淀粉样蛋白沉积.真皮及皮下脂肪血管周围有较多的淋巴细胞、浆细胞浸润,其间散在少许嗜酸性粒细胞.结晶紫染色阳性,PAS染色可疑阳性.诊断:结节萎缩性皮肤淀粉样变性.     

痒疹样结节型皮肤淀粉样变性1例

患者男,54岁.双下肢伸侧痒疹性丘疹3年,加重半年,于2004年2月19日来我科就诊.3年前无明显诱因患者双下肢伸侧起丘疹,伴剧烈瘙痒,曾在当地医院就诊,诊断为结节性痒疹,予以阿维A胶囊10 mg,每日3次口服,去炎松二甲亚砜溶液外用,2个月后皮损基本好转.半年前双下肢伸侧皮损复发并加重,并逐渐扩展至背部及双前臂伸侧,伴剧烈瘙痒.体格检查：一般情况尚可,系统检查无异常.皮肤科检查：背部、双前臂及双下肢伸侧散在绿豆至黄豆大褐色结节（图1）,触之质地坚实.皮损组织病理检查：苏木精-伊红染色示真皮乳头层及网状层有均一红染的团块状淀粉样蛋白沉积,均质物与表皮间存在一窄的裂隙,在淀粉样蛋白沉积物的周围有以淋巴细胞为主的炎性细胞浸润（图2A）.结晶紫染色见大片紫红色物质沉积在真皮乳头层及网状层（图2B）.结合临床表现诊断为痒疹样结节型皮肤淀粉样变性.给予补骨脂素长波紫外线（PUVA）每日1次治疗后,皮损开始明显消退,瘙痒基本消失,随访1年未见复发.     

结节性皮肤淀粉样变性一例

目的报告1例结节性限局性原发性皮肤淀粉样变性。方法结合皮损的临床特征、组织病理、刚果红染色、偏振光显微镜检查等,明确疾病的诊断。结果患者的皮损表现为上臂、腰腹部多发黄红色隆起性结节或斑块。组织病理示:真皮乳头、网状层、皮下组织及细血管周围大小不一均质性团块样物质沉积,刚果红染色示橙红色阳性,偏振光检查可见苹果绿双折光。结论结节性限局性原发性皮肤淀粉样变性诊断明确,临床少见。     

原发性系统性淀粉样变性1例

患者男,57 岁.因乏力3 年、加重半年,黄疸1 个月伴多发性皮下结节半个月入院.患者3 年前无明显诱因出现乏力,体力下降,余无不适,一直未诊治.半年前自觉上述症状加重,活动后出现胸闷,气短,心慌,无胸痛、晕厥等症状,伴腹胀、双下肢水肿,夜间常难以平卧入睡.1 个月前出现皮肤巩膜黄染,伴口干、多饮症状,半个月前渐出现全身隆起性皮下结节,无压痛、红肿、瘙痒,于多家医院就诊,均未能明确诊断,为进一步诊治,于2010 年1 月21 日来我院就诊,门诊以“多系统损害原因待查”收入院.患者无骨痛、关节痛,双下肢无水肿.起病以来精神差,饮食睡眠尚可,二便正常,体力下降,体重近2 个月下降约4 kg.2008 年发现腰椎间盘突出.2007 年9 月曾行“胆囊切除术”.家住湖北血吸虫疫区.吸烟、饮酒15 年,日均吸烟20支,白酒250 mL.否认肝炎、结核病史.     

泛发性皮肤淀粉样变性一例

临床资料患者,男,41岁。因全身对称性角化性丘疹、结节伴瘙痒6年,于2011年11月来我院就诊。6年前患者在工厂做工接触纸箱后躯干、四肢出现对称性角化性丘疹,伴瘙痒,病情逐渐加重,皮损范围扩大。曾于当地多家医院就诊,给予口服、外用药物(具体不详)治疗,瘙痒有所缓解,但每年7、8月份加重;近3年来患者在皮具厂做压膜工作后,瘙痒更为     

Localized primary cutaneous nodular amyloidosis: case report

Amyloidosis results from deposition of fibrous and insoluble amyloid protein in extracellular spaces of organs and tissues. Amyloid deposition can be localized or systemic and either primary or secondary. We report a case of localized primary cutaneous nodular amyloidosis manifested by papular-nodular, reddish-brown lesions affecting the nasal area, without evidence of systemic involvement. Immunohistochemistry showed the presence of immunoglobulin kappa light chain.     

Primary localized cutaneous nodular amyloidosis: case report and biochemical analysis of amyloid.

We report a patient with scalp lesions of primary localized cutaneous nodular amyloidosis. The extensive examination revealed no systemic involvement. Analysis of glycosaminoglycans (GAGs) in amyloid deposits showed a twofold increase as compared with normal skin, which was due to the increase in dermatan sulfate. Local disorders of GAG metabolism may be related to the amyloid fibril formation. Amyloid fibrils were purified and identified electron-microscopically, which consisted of two major 12,000- and 13,000-dalton and minor 29,000- and 48,000-dalton peptides. Western blotting analysis showed a minor 29,000-dalton peptide reactive with antibodies against both kappa and lambda light chains of immunoglobulin. There is a possibility that some components of amyloid in some cases of primary localized cutaneous nodular amyloidosis may consist of both kappa and lambda immunoglobulin light chains.     

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 and CREST syndrome: a case report and review of the literature

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.     

结节型皮肤淀粉样变性一例

患者男,58岁.5年前无明显诱因发现双足足趾肿块,并逐渐增大,无痛痒感.患者既往体健,家族中无类似病史.体检:各系统检查无明显异常.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.皮肤科情况:左足1～4趾间,右足1～5趾间内外侧见数个豌豆至核桃大小疣状肿块,最大2.5 cm×2.0 cm(左足示趾内侧),表面光滑,质地柔软,呈串珠样排列(图1a).趾间浸渍性白色.     

Primary cutaneous nodular amyloidosis associated with psoriasis

Primary cutaneous nodular amyloidosis (PCNA) presents as solitary or multiple firm, waxy nodules with a predilection for acral areas. Histologically, PCNA can be identical to myeloma‐associated systemic amyloidosis with monoclonal immunoglobulin light chain deposits. We describe a patient in whom PCNA developed in a scar in an area affected by chronic plaque psoriasis. PCNA has previously been associated with other autoimmune diseases, but to our knowledge, this is the first association with psoriasis. Interestingly, T helper (Th)17 cells, which are crucial in psoriasis pathogenesis, have recently been implicated in promotion of myeloma and plasma cell dyscrasias. The association of psoriasis and plasma‐cell light chain production in the skin, as in this case, suggests a possible role for Th17 cells in PCNA formation. The dermatopathological literature of this rare but important disease is discussed.     

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。     

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。