弹性假黄瘤样真皮乳头层弹性组织溶解症一例并文献复习

<正>临床资料患者,女,74岁。主因乳房下、腹部、躯干两侧、颈部皮损进行性增多半年余,于2013年1月17日就诊。患者于半年前无明显诱因乳房下出现粟粒至绿豆大淡黄色丘疹,无明显自觉症状,未予积极治疗。逐渐于腹部、躯干两侧及颈部出现新发皮损,进行性增多、增大。自起病以来患者一般情况可,精神、睡眠、食欲可。否认家族中有类似患者。体格检查:一般情况良好。系统检查未见明显异常。皮肤科检查:颈部、乳房下、腹     

儿童弹性纤维假黄瘤

报告1例儿童弹性纤维假黄瘤。患儿男,9岁。颈部及腹部丘疹3年。皮肤科检查:颈部及腹部可见成片密集分布的淡黄色粟粒至米粒大丘疹,呈网状排列。皮损组织病理检查:真皮上部和中部可见弹性纤维变性、断裂,排列不规则,呈团块状或颗粒状改变。弹性纤维Verhoeff-Van Gleson染色:真皮可见大量弹性纤维变性、断裂、卷曲,呈碎片或颗粒状。诊断:弹性纤维假黄瘤。     

弹性纤维性假黄瘤1例

患者女,26岁。10余年前颈外侧皮肤出现淡黄色小丘疹,皮疹渐向周边蔓延,累及腋下及腹股沟部。眼底检查为双眼视神经乳头周围有形行色素纹,后极部见血管纹样改变,黄斑部色素沉着。组织病理:真皮中下部弹性纤维明显增多而紧密,可见轻度嗜碱性染色及部分钙盐沉积。诊断为弹性纤维性假黄瘤。     

弹性纤维性假黄瘤1例

患者女,35岁。颈部、双侧腋下密集淡黄色扁平丘疹10余年。组织病理学检查示真皮大量波纹状、扭曲、断裂的深嗜碱性纤维成分。弹性纤维染色可见变性弹性纤维。诊断:弹性纤维性假黄瘤。     

弹性纤维性假黄瘤1例

报告1例颈部弹性纤维性假黄瘤,患者女,41岁,颈部多发性肤色或淡黄色扁平丘疹40余年,偶觉瘙痒。组织病理学检查示真皮网状层中部有多量波纹状,扭曲,断裂或不规则形状的深嗜碱性纤维成分,诊断为弹性纤维性假黄瘤。     

弹性纤维性假黄瘤1例

<正>临床资料患者,女,41岁。因颈部出现橙黄色丘疹伴蚁走感、紧绷感,于2009年4月28日来我科门诊就诊。患者于12年前,无意中发现颈部出现散在的点滴状黄色丘疹,无明显不适感,未予治疗,丘疹逐渐增     

弹性纤维假黄瘤一例

临床资料患者,女,36岁。主因颈部皮损10余年,于2012年10月19日来我院就诊。患者10年前无明显诱因颈部出现多发性淡黄色扁平丘疹,后渐隆起并融合成片状,弥漫至整个颈部,因无痛痒等不适,未进行治疗。患者体健,否认有类似疾病家族史。体检检查:一般状况可,心、肺、腹未见明显异常。皮肤科检查:颈部可见密集     

弹性纤维性假黄瘤一家系2例

弹性纤维性假黄瘤是一种临床上较少见的疾病，现将我们诊治的一家系2例患者报告如下。     

弹性纤维假黄瘤并发雀斑一家系报告

先证者(Ⅱ4).女,40岁.因颈部丘疹15年余,于2007年8月12日就诊.患者约25岁时颈部起少量黄色丘疹,以后逐年增多,皮肤逐渐松弛,形成褶皱,近2年尤其明显,无明显自觉症状,未曾治疗过.近2年来,劳累后双眼常感发胀,但无明显视力下降.患者从十余岁起,面部出现多发性黑色斑点及斑片,逐渐增多布满整个面部.父母健在,非近亲结婚.     

弹性纤维性假黄瘤3例

3例患者均为女性。无明显自觉症状,各系统检查未见明显异常。皮肤科情况：3例患者颈部、腹部等不同部位可见米粒至黄豆大小的黄色丘疹、斑块,大致沿皮纹分布,其外观如鹅卵石样,表面光滑,界限尚清,触之柔软,未见皮肤松弛。皮损组织病理示：表皮大致正常,真皮中部可见弹性纤维变厚、断裂、排列紊乱,呈嗜碱性颗粒状或碎片状。结合皮损特征和病理诊断：弹性纤维性假黄瘤。     

Pseudoxanthoma elasticum-like papillary dermal elastolysis developed in early middle age

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a rare acquired elastolytic disorder characterized by papules that resemble pseudoxanthoma elasticum, and it typically affects elderly women. Histopathological examination shows atrophic epidermis and band-like loss of elastic tissue in the papillary dermis. The pathogenesis is assumed to be related to intrinsic aging because it affects elderly people and shows the loss of elastic tissue. We report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis in early middle age presenting typical clinical and histopathological findings. The patient was a 41-year-old woman who had had her lesions for 10 years. We propose that younger patients, hitherto unknown, can be affected by this disorder and suggest that mechanisms other than intrinsic aging are involved in its pathogenesis.     

Acquired idiopathic cutaneous elastolysis of the papillary and mid-dermis: distinct but related conditions. Report of two cases

Among the acquired idiopathic cutaneous elastolytic disorders, pseudoxanthoma elasticum-like papillary dermal elastolysis (PDE) and mid-dermal elastolysis (MDE) are conditions not frequently reported in the literature.
We report 2 cases: the first an older woman with typical papular and plaque-like lesions on both sides of her neck and shoulders and the second a 42-year-old woman with fine wrinkles scattered all over the Hunk, shoulders and abdomen. Histological examination of the first case revealed loss of the elastic fibers limited to the papillary dermis, instead in the second case the loss was observed in the mid-dermis.
We discuss the possibility that PDE and some cases of mm-inflammatory MDE may represent distinct but related entities in a "spectrum" of acquired elastolysis and that they may be an expression of intrinsic aging.     

获得性全身性弹性组织离解症(国内首例)

获得性全身性弹性组织离解症为一罕见系统性疾病,系由结缔组织中弹力纤维碎裂、紊乱、减少,导致进行性皮肤松弛,伴发内脏异常。现报告国内首例患典型获得性全身性弹性组织离解症的36岁女性病例1例,并予以讨论。     

Mid Dermal Elastolysis: Case Report and Review of the Literature

A new case of typical mid dermal elastolysis was studied with electron microscopy. Elastic fibers were irregularly branched or lumpy, but subcomponents of these fibers were often normal. In some fibers, loose assembly of skeleton fibrils and aggregation of dense substances were observed. Phagocytosis of normal and abnormal elastic fibers by dermal macrophages was observed.     

Acute Urticaria Associated with Thyroid Papillary Carcinoma: A Case Report

Although there are several studies showing the association between cancer and urticaria, the mechanisms by which these events occur are not yet known. In this report, a case of acute urticaria with a diagnosis of thyroid papillary carcinoma is presented. Disappearance of treatment-resistant urticarial lesions after thyroidectomy suggests that this association was not a coincidence. The fact that urticaria which was nonresponsive to treatment disappeared spontaneously as a result of tumorectomy, strongly suggests that this association is not a coincidence. To our knowledge, this is the first report of the coexistence of acute urticaria and thyroid papillary carcinoma. This case provides further support that detailed history taking and a thorough physical examination are of paramount importance.     

Intravascular Papillary Endothelial Hyperplasia on the Penis: Report of a Case

An 82-year-old Japanese man with intravascular papillary endothelial hyperplasia on the penis is described. To the best of our knowledge, this is the first report of a patient who developed this tumor on his penis.     

Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case

Fibroelastolytic papulosis is a rare, acquired fibroelastolytic disorder that presents clinically as white‐to‐yellow papules and plaques most commonly occurring on the neck of elderly patients. The term fibroelastolytic papulosis encompasses two closely related conditions previously described as pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE) and white fibrous papulosis of the neck (WFPN). Here we present a case of a 78‐year‐old white female with a several‐year history of numerous, asymptomatic 2–3 mm yellowish, non‐follicular papules distributed symmetrically over the posterior neck, axillae, arm and antecubital fossae. Histopathologic examination revealed thickened and clumped elastotic fibers admixed with thick, sclerotic appearing collagen bundles in the mid and deep reticular dermis. Rare melanophages, loss of vertically oriented elastic fibers and scattered elastotic globes were noted in the papillary dermis. Based on the shared clinicopathologic features showed in this case, strong consideration should be made for the additional inclusion of papillary dermal elastosis as existing along the disease continuum of fibroelastolytic papulosis. This occurrence of fibroelastolytic papulosis shows unique histopathologic findings of pseudoxanthoma elasticum‐like PDE, papillary dermal elastosis and WFPN, further supporting the theory that these entities exist as variants along the fibroelastolytic papulosis spectrum.     

Focal Dermal Hypoplasia (Goltz Syndrome): A Case Report Showing a Wide Variety of Systemic and Oral Manifestations

Focal dermal hypoplasia (FDH), also known as Goltz syndrome, consists of an unusual genodermatosis that affects tissues of ectodermal and mesodermal origin and various organs and systems, especially skin, bones, eyes, and oral cavity. While systemic manifestations of FDH have been well documented, the oral manifestations have not been extensively discussed. We present a 22-year-old female patient with history of FDH that showed a variety of systemic and oral manifestations. FDH was diagnosed at birth based on cutaneous alterations. Extra and intraoral examination showed facial asymmetry, lip and perioral atrophy, upper lip papilloma, malocclusion, enamel hypoplasia, and gingival hyperplasia. Mucosal lesions, periodontal diseases, and malocclusion were treated by oral surgery, periodontal therapy and orthodontic treatment, respectively. Although FDH is an uncommon syndrome, health professionals should be aware of its systemic and oral manifestations to establish an early diagnosis and adequate treatment.     

多发性毛盘瘤国内首例报告

目的 报告1例多发性毛盘瘤。患者男,31岁。主诉右下肢出现多发性高梁米粒至小豆大小坚实性皮色丘疹15年,左膝关节、左小腿部同样皮损近1年,无自觉症状。方法和结果 组织病理显示表皮棘层肥厚,真皮上部为细网状胶原纤维增殖和局灶性黏蛋白沉积,细小血管和神经纤维成分增多,弹力纤维增殖限局于毛囊和表皮突周围。增生毛囊见于病变两侧边缘,呈衣领状向下延伸。超微结构示Merkel细胞-轴突复合体位于表皮下基底板上方,真皮上部见有髓神经纤维。血管超微结构有的基底板显示层状结构,时见血管壁纤维成分增殖,管壁增厚。结论 本病罕见,系来源于毛盘的一种错构瘤。     

汗管样小汗腺癌国内首报

患者女,56岁。右口角斑块2年余。皮肤科情况:右口角见一2.2cm×1.4cm浸润性肤色斑块,中央偏淡红色,表面不平,边界清楚,与皮肤黏连,质硬。组织病理检查示:真皮中下部及皮下组织见多数基底样瘤细胞团块,瘤细胞形成巢状、条索状或蝌蚪样肿瘤团块,伴有大量管腔及囊腔样结构形成,部分腔内可见嗜酸性均一物质。间质明显增生,可见透明变性和黏液变性。未见角质囊肿及筛状结构形成。免疫组化染色示:肿瘤细胞上皮膜蛋白(EMA)和癌胚抗原(CEA)阳性,S100蛋白、角蛋白10(CK10)、角蛋白20(CK20)和GCDFP-15(gross cystic disease fluid protein-15)均阴性。诊断:汗管样小汗腺癌。本病临床罕见。治疗采用Mohs手术切除。