丘疹性弹性纤维离解22例临床病理分析

【摘要】 目的 分析丘疹性弹性纤维离解的临床及病理学特点。方法 回顾2006年 9月至 2018年 5月在中国医学科学院皮肤病医院确诊的22例丘疹性弹性纤维离解患者的临床、病理学表现及随访情况。结果 22例患者平均发病年龄5.7岁（1～10岁）,男女比例4.5∶1,发病至确诊时间平均1.5年,无明确病因。患者皮损均无痒痛等症状,均表现为白色丘疹,直径1 ~ 10 mm,圆形、椭圆形或多角形,轻度隆起,质软,境界清晰,推挤丘疹两侧正常皮肤后丘疹表面可出现皱纹。16例（73%）皮损散在分布, 13例（59%）皮损数 < 5个;21例（95%）皮损位于躯干部位。8例皮损组织病理表现：真皮浅中层胶原纤维无明显增加,排列正常,但间隙轻度增宽;弹性纤维染色显示真皮浅中层局限性弹性纤维消失及离解。确诊后,22例均未使用任何药物治疗,其中18例自起病后皮损未继续扩大,也未出现新发皮损;4例皮损轻度扩大,但后期停止进展;16例患者出现部分缓解。结论 丘疹性弹性纤维离解是一种好发于儿童青少年的罕见弹性纤维性皮肤病,诊断需临床结合病理,无需特殊治疗,预后良好。     

结节性类弹性纤维病

报告1例结节性类弹性纤维病.患者男,70岁.因面部斑块1个月就诊.皮肤科检查:左侧面部褐青色、桔黄色橘皮样斑块,其间分布针尖大黑褐色粉刺.组织病理检查:表皮局部萎缩,真皮部分嗜碱性变,局部皮肤附属器萎缩,真皮浅中层局部可见毛囊呈囊性扩张,毛囊漏斗部角化过度,其内充满角质物.诊断为结节性类弹性纤维病.     

结节性类弹性纤维病1例

<正>临床资料患者,女性,83岁。主因面部皮肤增厚、变黄伴黑头粉刺、囊肿2年,于2010年3月19日来我科就诊。患者于2年前出现面部皮肤增厚、变黄,皮损中出现多个黑头粉刺和皮下小结节、囊肿。皮损处无自觉症状,表面无破溃。既往体健,有失眠、     

匐行性穿通性弹性纤维病

报告1例匐行性穿通行弹性纤维病.患者男,20岁.面部淡红色环形排列丘疹2年,无痒痛感,缓慢进展.皮损组织病理检查:局部表皮增生,可见穿通通道及内含嗜碱性碎片.Verhoeff-Van Gieson弹性纤维染色示真皮浅层弹性纤维增多,穿通的通道内可见弹性纤维.结合临床表现及组织病理检查结果,诊断为匐行性穿通行弹性纤维病.     

肢端角化性类弹性纤维病一例

患者女,20岁。因双手小指尺侧条状象牙白色斑2个月于2012年4月25日就诊。2个月前双手小指尺侧出现绿豆大白色角化性斑丘疹,逐渐增大并融合成条状象牙白色斑,并逐渐向上累及小鱼际尺侧中段,无自觉症状……     

丘疹性弹性纤维离解

报告1例丘疹性弹性纤维离解.患者女,24岁.因双侧前臂多发白色质硬非瘙痒性丘疹10年就诊.皮损散在分布,不能自行消退.皮损组织病理检查显示病变部位弹性纤维崩解,弹性纤维数量显著减少.     

丘疹性弹性纤维离解

报告1例丘疹性弹性纤维离解.患者女,32岁.颈部、枕部及下颌部无症状性白色丘疹3周.局部无痤疮及其他炎症性皮肤病史.皮肤科检查:下颌及颈、枕部见20余枚白色非毛囊性斑疹及丘疹,直径2～4 mm,呈圆形、星形或多角形.皮损组织病珲检查:真皮乳头层胶原轻度增生,弹性纤维断裂、减少,甚至局灶性消失.诊断为丘疹性弹性纤维离解.     

匐行性穿通性弹性纤维病1例

患者女,16岁.颈部丘疹、脓疱,伴微痒1年,于2007年12月24日来我院门诊就诊.患者1年前于左侧颈部出现单个米粒大的暗红色丘疹,稍痒,挤压后,有少量水样物和黄色角栓样物质排出,以后丘疹不断增多.逐渐向颈前部漫延.曾在当地医院诊断为"毛囊炎".     

肢端角化性类弹性纤维病1例

肢端角化性类弹性纤维病（acrokeratoelastoidosis,AKE）是一种少见的角化性皮肤病,皮损主要发生于手足,为表面光滑半透明的角化性丘疹。现将我科诊治的1例报告如下。[第一段]     

结节性类弹性纤维病伴囊肿及黑头粉刺

报告1例结节性类弹性纤维病伴囊肿及黑头粉刺.患者男,70岁.因双侧面颊褐黄色结节、斑块10余年,伴右侧面颊溃疡1个月就诊.皮肤科检查:双侧面颊见褐青色、橘黄色斑块、结节间有多个粟粒大小的黑头粉刺,挤压结节有脂样物质溢出,右侧面颊见3 cm × 4 cm溃疡面,有少量黏稠分泌物及结痂,气味腥臭,边缘不规则;组织病理检查诊断为结节性类弹性纤维病伴囊肿及黑头粉刺.     

Acquired disorders of elastic tissue: Part II. decreased elastic tissue

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.     

Cosmetical treatments of connective tissue disorders

Connective tissue disorders (CTDs) are chronic inflammatory conditions that can lead to scarring and disfiguration. Although conventional methods are often of little benefit in cutaneous manifestations, the use of cosmetic procedures is still controversial. Concerns have also been raised concerning cosmetic treatments in CTDs, and particularly regarding lasers and fillers, due to photosensitivity and potential reactivation. This article reviews the cosmetic treatment of various CTDs under three headings ‐ lasers, fillers, and botulinum toxin.     

Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.     

Cutaneous disorders characterized by elastolysis or loss of elastic tissue

Along with collagen, elastic fibers are integral components of cutaneous connective tissue. A decrease in elastic fibers or loss thereof has been described in a number of clinically distinct skin diseases, both hereditary and acquired. In disorders associated with inflammation, elastophagocytosis is an important histological hallmark. Treatment is generally difficult.     

Immunohistochemical studies of amyloid P component in disorders of cutaneous elastic tissue

Distinctive abnormalities in the immunofluorescence/immunoperoxidase staining pattern of dermal elastic tissue were observed with antibodies to serum amyloid P component (SAP) in solar elastosis, lupus erythematosus, porphyria and pseudoxanthoma elasticum, and resembled those seen with conventional elastic tissue stains. Electron microscopy of elastotic skin revealed the presence of vacuotated disintegrating elastic fibres. Islands of amorphous microfibrillar material were surrounded by a rim of specific staining with anti-SAP, indicating an elastic tissue origin; there was no evidence for the involvement of collagen fibres in the formation of elastotic material. Immunohistochemical staining with anti-SAP, a marker for elastic fibre microfibrils, is a useful method for the investigation of cutaneous elastic tissue disorders.     

强脉冲光照射对小鼠皮肤胶原纤维、弹性纤维及透明质酸含量的影响

目的 探讨强脉冲光照射对昆明小鼠皮肤胶原纤维、弹性纤维、透明质酸含量的意义。方法用强脉冲光照射小鼠右侧背部皮肤,左侧为对照,取各时间点照射组和对照组皮肤进行组织学观察,包括HE染色、Ⅰ型和Ⅲ型胶原纤维饱和苦味酸-天狼猩红染色、弹性纤维Gomori醛品红法染色,同时提取皮肤组织,碱水解-分光光度法测定羟脯氨酸含量,放射免疫法测定透明质酸含量。结果 照射后2周,真皮厚度较对照组增加（t = 4.623,P < 0.05）,4周时达最大值,较对照组增加18.71%,8周时仍大于对照组,差异有统计学意义（t = 3.904,P < 0.05）。照射后1周,Ⅲ型胶原纤维较对照组增加40.54%（t = 5.129,P < 0.05）,照射后2周和4周,Ⅰ型和Ⅲ型胶原纤维均较对照组增多明显,至8周时,均大于对照组（P < 0.05）。照射后2 ~ 8周,弹性纤维均较对照组增多（P < 0.05）;照射后1 ~ 8周,羟脯氨酸含量均较对照组增多（P < 0.05）。照射后1 d和3 d,透明质酸含量显著增加,1 ~ 8周,渐进性降低,但均高于对照组（P < 0.05）。结论 强脉冲光照射小鼠皮肤可刺激真皮胶原纤维、弹性纤维、透明质酸含量增加。     

Ocular and cutaneous manifestations of heritable disorders of collagen and elastic tissue.

Ocular and cutaneous stigmata are hallmarks of the heritable disorders of collagen and elastic tissue. Awareness of the oculocutaneous features is essential both to aid diagnosis and to direct management of affected patients. Although the angioid streaks of PXE, blue sclerae of OI, ocular fragility of EDS and ectopia lentis of Marfan syndrome are the best-known ocular signs of these disorders, other relevant ophthalmologic symptoms often accompany such classic findings.     

Treatment of fibroelastolytic papulosis with fractionated carbon dioxide laser

Fibroelastolytic papulosis (FEP) is an acquired elastic tissue disorder that presents as white-to-yellow papules and plaques usually occurring on the neck. Although the lesions are often asymptomatic, their appearance may be distressing to patients. FEP has been treated with topical tretinoin in one case report (1 Wang AR, Lewis K, Lewis M, Robinson-Bosom L. Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum- like papillary dermal elastolysis? J Cutan Pathol. 2009;36: 1010–1013.[Crossref] , [Google Scholar]). Other reports have not mentioned treatment for this rare disorder (1–6 Wang AR, Lewis K, Lewis M, Robinson-Bosom L. Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum- like papillary dermal elastolysis? J Cutan Pathol. 2009;36: 1010–1013. Rongioletti F, Rebora A. Fibroelastolytic patterns of intrinsic skin aging: pseuodoxanthoma-elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Dermatology. 1995;191:19–24. Shimuzu H, Kimura S, Harada T, Nishikawa T. White fibrous papulosis of the neck: a new clinicopathologic entity? J Am Acad Dermatol. 1989;20:1073–1077. Balus L, Amantea A, Donati P, Fazio M, Giuliano MC, Bellocci M. Fibroelastolytic papulosis of the neck: a report of 20 cases. Br J Dermatol. 1997;137:461–466. Jagdeo J, Ng C, Ronchetti IP, Wilkel C, Bercovitch L, Robinson-Bostom L. Fibroelastolytic papulosis. J Am Acad Dermatol. 2004;51:958–964. Ohnishi Y, Tajima S, Ishibasi A, Inazumi T, Saski T, Sakaoto T. Pseudoxanthoma elasticum-like papillary dermal elastolysis: reposrt of four Japanese cases and an immunohistochemical study of elastin and fibrillin-1. Br J Dermatol. 1998;139:141. ). We present a case of FEP successfully treated with a fractionated carbon dioxide (CO₂) laser.     

Pseudoxanthoma elasticum: report of two cases

Pseudoxanthoma elasticum is a rare inherited multisystem disorder that ischaracterized by a pathological mineralization of the elastic connective tissue,which involves predominantly the skin, eyes and cardiovascular system. Its cause lieson mutations in the ABCC6 gene, which lead to reduction or absence of thetransmembrane transport ADP dependent protein (MRP6), causing an accumulation ofextracellular material and subsequent deposition of calcium and other minerals in theelastic tissue. The authors report two cases of pseudoxanthoma elasticum, emphasizingits major clinical features and the importance of early diagnosis of the disorder,aiming for adequate therapeutic management of associated complications.     

Disulphide reaction staining for the identification of integumental elastic fibres

Based on specific methods (Sippel-APM-chromotropic acid technique; IC3-PE-maleimide fluorescence reaction) and skin samples of four domesticated mammals (dog, cattle, horse, pig), disulphide groups were demonstrated in the elastic component of the basement membrane of the epidermis, the elastic fibre system of the dermis, the elastic components of the connective tissue sheath of hair follicles, apocrine tubular glands, and sebaceous glands, and of the connective tissue surrounding the cutaneous muscle. The results are discussed regarding the relation of this reaction staining to the presence of microfibrils (fibrillin) in the elastic fibres.