黏液水肿性苔藓1例

报告1例黏液水肿性苔藓或硬化性黏液水肿。患者男，45岁。面部皮肤弥漫性肿胀、硬化并有多发隆起性斑块，鼻根部斑块隆起明显，形成一纵行皮嵴，颈部、躯干、双上肢近端泛发苔藓样丘疹。常规实验室检查无异常。皮肤组织病理示真皮内胶原纤维及成纤维细胞增多，胶原束间黏蛋白沉积。     

泛发型黏液水肿性苔藓

报告l例黏液水肿性苔藓。患者女,47岁。面部及全身皮肤出现弥漫性丘疹4年余,手指屈曲障碍1年余。颈部、躯干及四肢泛发苔藓样丘疹,面部皮肤肿胀、硬化,鼻根部斑块隆起较明显。皮损组织病理检查示真皮内胶原纤维及成纤维细胞增多,胶原束间黏蛋白沉积,阿辛蓝染色阳性。给予糖皮质激素和甲氨蝶呤治疗,效果显著。     

无副蛋白血症的风团型粘液水肿性苔藓

粘液水肿性苔藓(LM)的特点是在正常甲状腺人的正常皮肤中发生的多数性粘蛋白沉着。临床上可分成4种类型;1)泛发性苔藓样丘疹,其下组织变硬,又称为硬化性粘液水肿。2)躯干和四肢疏散分布的丘疹。3)局限或广泛苔藓样斑块。4)风团样斑块和结节。各型中基本组织变化是在真皮胶原束之问有粘液样物质沉积,这种物质已证实为透明质酸。LM的病因和发病机理不明。     

粘液水肿性苔藓1例

粘液水肿性苔藓１例姜海燕，丁连英，金非，赵桂兰粘液水肿性苔藓是一种以皮肤内纤维母细胞增生、酸性粘多糖过多沉积、胶原破碎为特征的少见病。我们见到１例，现报告如下。患者，女，３８岁。１８年前发现额、面、颈部皮肤起粟粒大淡红色扁平丘疹，逐渐融合成片、增厚，...     

席纹状胶原瘤

报告1例席纹状胶原瘤。患者男,46岁。背部肿物40年余,缓慢增长,无自觉症状。无系统性疾病病史。皮损组织病理检查示表皮轻度萎缩,真皮内可见一边界清楚的结节,无包膜,病变均匀、局限,由大量透明样变的胶原束组成,胶原束之间见许多裂隙,排列成席纹状。诊断:席纹状胶原瘤。     

面部发疹性胶原瘤伴太田痣一例

报告面部皮肤发疹性胶原瘤1例。患者女,30岁。面部出现多发性扁平丘疹、结节4年。皮损为正常皮色或红色,直径2－10mm,无自觉症状,无家族史。组织病理检查示：胶原显著增厚,胶原束宽大,弹力纤维减少、碎裂。诊断为发疹性皮肤胶原瘤。     

73例女性硬化性苔藓临床及病理分析

 目的:分析73例女性硬化性苔藓患者的临床表现及组织病理特征。方法:回顾性分析本科诊治的73例女性硬化性苔藓患者的临床资料。结果：73例患者中，50岁以上者34例（46.58%），14～50岁者28例（38.36%），小于14岁者11例（15.07%)。皮损好发于外阴部位。基本病理改变为角化过度、基底层空泡变性、真皮浅层水肿，伴胶原均质化透明样变性,其下方常有不同程度的淋巴细胞及组织细胞浸润。结论:临床表现及组织病理学检查为诊断本病的依据，可根据组织病理表现选择相应的治疗。医务人员应加强对早期硬化性苔藓的认识，减少误诊率，并根据疾病时期选择相应的治疗。     

与文胸摩擦有关的硬化萎缩性苔藓

报告1例与文胸摩擦有关的硬化萎缩性苔藓。患者女,51岁。肩部及双乳下方硬化萎缩性斑片2个月,无明显自觉症状。皮肤科检查:沿双侧胸罩的压力线分布的瓷白色硬化萎缩性斑疹及斑片。皮损病理检查:表皮轻度萎缩,毛囊角栓;真皮浅层胶原均一化改变,局部血管扩张充血,见少量嗜色素细胞;真皮中部胶原增生,排列致密,血管周围见中等量淋巴单一核细胞及个别浆细胞浸润。予吡美莫司乳膏外用,嘱穿戴宽松文胸。诊断:与文胸摩擦有关的硬化萎缩性苔藓。1个月后复诊可见皮损范围缩小,无新发皮损。     

粘液水肿性苔藓1例附文献复习

粘液水肿性苔藓是一种结缔组织代谢异常而引起的皮肤病,临床罕见。其特征是临床出现无症状的局限性苔藓样丘疹、斑块或全身性硬皮病样改变,病理检查在真皮有成纤维细胞增殖、酸性粘蛋白过度沉积。该病病因未明,临床治疗困难,但预后良好。我们近期成功诊治1例患者,现作报道,并复习文献综述该病的病因、临床表现、诊断与治疗,以供临床诊治参考。     

硬化性苔藓

介绍了硬化性苔藓的发病机理、临床特征和治疗方法。硬化性苔藓与自身免疫、角蛋白异常表达及胶原和结缔组织代谢异常有关。典型病例易诊断,但对不典型或疑有恶变的病例应进行组织病理学检查,及时治疗,严密随访,以防恶变。局部应用强效皮质类固醇,口服阿维A酯、阿维A均可获得满意疗效。     

The pathogenesis of the transepithelial elimination of the collagen bundles in acquired reactive perforating collagenosis

Two cases of acquired reactive perforating collagenosis with poorly controlled diabetes mellitus were studied by histochemistry and by electron microscopy. In excoriated wounds, the necrotic mass on the bottom of the ulcer contained the collagen bundles which were continuous with the collagen bundles in the reticular layer. In the developing stage, the epidermis regenerated between the necrotic mass and the reticular dermis, and the collagen bundles in the reticular dermis were in continuity with those in the necrotic mass through the epithelial tunnels. The collagen in the epidermal channels did not degenerate ultra-structurally. In the mature lesion, collagen bundles being eliminated through the epidermis were surrounded by the fibroblasts at the basal cell layer. Collagen fibers were seen in the cytoplasm of these fibroblasts. From these findings, the mechanisms of the formation of the eruption in acquired reactive perforating collagenosis might be as follows: 1) In the developing stage, the regeneration of epidermis progresses between the necrotic mass and the reticular dermis, and among the collagen bundles. As a result, the collagen bundles remain in the channels of the epidermis. And then, 2) the regenerated epidermis makes the thick horny layer. As a result, the necrotic masses are lifted up and the collagen bundles are pulled up from the dermis through the epidermal channels.     

Mast cell numbers in diffuse scleroderma

Numbers of mast cells were quantitated in the lesions of diffuse scleroderma and morphea. Mast cells increased and then decreased in number in the papillary dermis of diffuse scleroderma. No significant change of mast cell numbers was noted in the reticular dermis. Mast cells increased in the papillary dermis with fine collagen bundles (grade 2 skin of scleroderma) and decreased in the papillary dermis with homogeneous collagen bundles (grade 3 skin of scleroderma). The total number of cells increased in the papillary dermis of grade 1 and 2 skin of scleroderma and decreased in the grade 3 skin of scleroderma. In morphea a reduced number of mast cells was noted in grade 3 lesions. It is suggested that mast cells play an important role in fibrotic process of scleroderma skin.     

Familial cutaneous collagenoma: a clinicopathologic study of two new cases

Two siblings with familial cutaneous collagenoma syndrome had the essential clinical features of multiple skin-colored nodules on the trunk and upper arms. On light microscopy, histopathologic findings included excessive accumulation of dense, coarse collagen in the dermis. Elastic tissue stains demonstrated a proportionately diminished number of abnormal elastic fibers intermingled with the collagen bundles. A predominance of densely packed collagen bundles of normal morphology with a marked decrease in abnormal elastic tissue were the major ultrastructural features. The diagnosis was therefore confirmed to be connective tissue nevi of the collagen type. The differential diagnosis of connective tissue nevi disorders is delineated.     

Solitary sclerotic fibroma

A 63-year-old Chinese man with a solitary sclerotic fibroma on the abdomen is described. Skin biopsy showed a well-demarcated dermal nodule composed of hypocellular, eosinophilic collagen bundles separated by prominent clefts. Retrospective electron microscopic study revealed wide collagen bundles containing tightly packed collagen fibrils, only 50 nm in diameter. There were no signs of Cowden's disease. To our knowledge, this is the first report of electron microscopic study of sclerotic fibroma. A brief review of the literature is included.     

A morphologic study of a mild form of ovine dermatosparaxis

A mild form of dermatosparaxis has recently been identified in sheep from several properties in central Victoria. Examination of the skin of affected animals by both transmission and scanning electron microscopy has shown that the structure of the majority of the collagen fibrils is irregular and the distorted fibrils do not pack into tight and well-ordered fiber bundles. Examination of the skin by light microscopy has shown that the fiber bundles are not compact and interwoven, and that there is a tendency for the collagen to form layered sheets in the reticular dermis. These studies also show that there is no buildup in the elastic tissue component, but that there is an increased population of fibroblasts in the affected animals.     

Nephrogenic fibrosing dermopathy.

This report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease. The affected patients were initially identified among recipients of renal transplants at a single institution, but later cases at other centers were identified, and included patients receiving renal dialysis for a variety of different kidney diseases. The cutaneous changes consisted largely of indurated plaques and papules on the extremities and trunk. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in early lesions, to a florid proliferation of fibroblasts and dendritic cells in fully developed cases. Thick collagen bundles with surrounding clefts are a prominent finding, and a variable increase in dermal mucin and elastic fibers was usually evident with special stains. CD-34 positive dermal dendrocytes were floridly abundant, with dendritic processes aligned with elastic fibers and around collagen bundles in a dense network. Factor XIIIa and CD-68 positive mono-and multinucleated cells are also present in increased numbers. Electron microscopy highlighted increased elastic fibers closely apposed to dendritic cell processes. The entire dermis was commonly involved, with increased spindle cells, collagen, mucin, and elastic fibers extending through the subcutis along the septa of fatty lobules. In some instances, the process resembled a sarcoma on histopathologic examination. The recent emergence of this condition and the apparent clustering of cases in specific dialysis centers initially suggested a possible infectious and/or toxic agent. To date, however, no such agent has been identified. We propose the term "nephrogenic fibrosing dermopathy (NFD)" until a specific cause can be identified.     

Generalized morphea with blisters. A case report

A patient suffering from generalized morphea developed blisters in the morpheic plaques on her buttocks. The plaques had an increased concentration of serum aminoterminal propertice of type III procollagen, an echo response and thickened skin on ultrasound scanning, and compact bundles of collagen fibrils with bimodal distribution of the diameters. The blisters appeared as an echo-free band in the subepidermal zone by ultrasound scanning. Electron microscopy revealed blisters in the upper papillary dermis, surrounded by degraded collagen fibrils.     

Extra nuchal-type fibroma associated with elastosis, traumatic neuroma, a rare APC gene missense mutation, and a very rare MUTYH gene polymorphism: a case report and review of the literature*

We report a case of an extra nuchal-type fibroma in a 51-year-old male suspected to have attenuated familial adenomatous polyposis (Gardner's syndrome), who presented with a longstanding buttock mass excised due to enlargement and pain. Histopathologically, lobules of haphazard, hypocellular, hyalinized collagen bundles replaced the dermis and subcutis and entrapped nerve bundles, mimicking Morton neuroma. Ramifying nerve twigs found around larger nerve fascicles showed the co-existence of traumatic neuroma. Elastic tissue stain revealed elastosis characterized by large, arborizing fibers lying between and within the hyalinized collagen bundles. Modified Masson's trichrome stain showed light blue staining of collagen bundles producing the hyalinized nodules with irregular, light red staining of collagen bundles at their periphery and within tumor collagen. Compression and/or degeneration of collagen and secondary elastosis with later entrapment by tumor collagen could explain this microscopic phenotype. By immunohistochemistry, tumor spindle cells expressed nuclear β-catenin and cyclin D1, mostly within regions of fibrosis implicating activation of the adenomatous polyposis coli (APC)-Wnt pathway. Genetic analysis showed a missense mutation in APC gene (c.7504G>A, p.G2502S in exon 15) and a functional homozygous polymorphism in the MUTYH gene (c.36+325G>C, (IVS1+5G/C)). Nuchal-type fibroma has been associated with Gardner's syndrome and trauma. In this patient, genetic predisposition coupled with repetitive, localized trauma and collagen degeneration may have provided the stimulus for the development of extra nuchal-type fibroma.     

Severe disruption and disorganization of dermal collagen fibrils in early striae gravidarum

Stretch marks affect 50‐90% of pregnant women and can cause emotional distress. Stretch marks initially appear reddish, and mature over months to years, becoming permanent white streaks with a depressed, scar‐like appearance. As little is known about how stretch marks develop, this study, from the University of Michigan in the US, aimed to examine the molecular changes occurring in early stretch marks, with the reasoning that findings may explain the appearance of mature stretch marks. For the study, women in their second or third trimester of pregnancy provided skin samples of recently developed, reddish stretch marks on the abdomen; additionally, some women provided skin samples after delivery. The skin samples were analyzed using laboratory techniques to examine the appearance, organization and production of collagen, the main protein that provides strength and support to the skin. The authors found that collagen appeared abnormal in early stretch marks. In particular, collagen bundles, which are normally densely packed, appeared markedly separated, as if “pulled apart” by extreme skin stretching. The skin attempts to repair these separated, damaged collagen bundles by making more collagen. However, this reparative process is inadequate, and produces abnormally thin, disorganized collagen strands that fail to form normal bundles. These changes persisted in stretch marks after delivery, and likely explain why mature stretch marks appear depressed and lacking in structural support. The study's findings suggest that methods of preserving the normal organization and structure of collagen may prevent stretch marks. Once stretch marks have formed, treatments that stimulate the production of collagen that is properly organized as densely packed bundles may improve the appearance of stretch marks. Many treatments available for improving stretch marks are not effective, likely because they do not stimulate normal collagen rebuilding. As such, the authors’ findings not only provide insight into how stretch marks develop at the molecular level, but also provide a therapeutic strategy for improving stretch mark appearance.     

Increased expression of lysyl oxidase in skin with scleroderma

Summary Lysyl oxidase initiates cross-linkage of collagen and elastin by catalysing the formation of a lysine-derived aldehyde. In order to study cross-linking in scleroderma, we used monoclonal antibodies to lysyl oxidase to determine the localization of this enzyme in systemic and localized scleroderma, and compared the distributions obtained with that in normal skin. Using an indirect immunofluorescent antibody method and an avidin-biotinylated enzyme complex method. 11 cases of diffuse type of systemic scleroderma and seven cases of localized scleroderma were studied. In the oedematous stage of systemic scleroderma, intracellular and extracellular lysyl oxidase were remarkably increased in the dermis, particularly in groups around blood vessels. In the sclerotic stage of systemic scleroderma, lysyl oxidase was detected intracellularly in fibroblasts and extracellularly among collagen bundles between the lower dermis and the subcutaneous fat tissue. In localized scleroderma, a marked increase in lysyl oxidase was observed in mononudear cells and libroblasts near blood vessels in the lower dermis and in the subcutaneous fat tissue, in addition to the extracellular deposits between collagen bundles. The increase in lysyl oxidase in localized scleroderma was much more common than in the oedematous stage of systemic scleroderma. These findings indicated that intracellular and extracellular expression of lysyl oxidase expression was greater in sclerodermatous skin than in normal skin.