Meyerson痣6例临床及病理分析

目的:探讨Meyerson痣临床及组织病理特征。方法:回顾性分析2015年1月至2020年1月第四军医大学西京皮肤医院确诊的6例Meyerson痣患者临床及病理资料。结果:6例患者中,男3例,女3例,年龄7个月至28岁,中位年龄10.5岁。3例皮损位于四肢,3例位于躯干。4例发生于先天性色素痣,2例发生于获得性色素痣。...     

网状红斑黏蛋白病1例

患者男,26岁。面颈部、躯干、四肢皮疹1年。皮损组织病理示轻度角化过度,表皮水肿,真皮浅至中层毛细血管扩张充血,周围有较多单一核细胞浸润。阿新蓝染色见真皮乳头层及网状层大量黏蛋白样物质沉积。诊断:网状红斑黏蛋白病。     

网状红斑黏蛋白病并发蕈样肉芽肿1例

报告网状红斑黏蛋白病并发蕈样肉芽肿1例。患者女。42岁。因躯干、上肢红斑14年,皮损增多,加重5年就诊。6年前行皮损组织病理学检查示真皮乳头层及网状层有大量黏蛋白样物质沉积,诊断为网状红斑黏蛋白病,入院皮肤科检查：头部、面部、颈部、躯干、四肢见片状轻度浸润性红斑,部分融合成网状,腹部红斑上可见紫红色浸润性斑块。皮损组织病理检查：真皮肉淋巴细胞苔藓浸润,部分细胞异形,少量浸润细胞移入表皮,形成Pautrier样微脓种,免疫组化染色结果示：LCA（＋＋＋）,CD45RO（＋＋＋）,诊断。蕈样内芽肿。     

伴毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿

报告2例伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.例1.女,39岁.面部、躯干、四肢出现红斑、丘疹,伴瘙痒3年余就诊.例2.男,52岁.躯干、四肢出现红斑、斑块1年就诊;结合组织病理检查、免疫组化染色及淋巴细胞克隆性基因重排检测.2例患者均诊断为伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.     

Spitz痣25例临床病理分析

对2011-2017年西安交通大学第二附属医院的25例Spitz痣患者的临床及组织病理资料进行回顾性分析。25例患者中男11例,女14例,其中﹤10岁患者16例。皮损位于面部12例,下肢5例,上肢4例,躯干3例,头皮1例。皮损主要表现为扁平隆起或半球形,多有色素增加。84%的Spitz痣具有良性肿瘤的特点,16% Spitz痣结构不对称或境界不清楚。     

毛囊黏蛋白病的临床及组织病理特点

目的:分析14例毛囊黏蛋白病患者的临床和组织病理表现.方法:对14例毛囊黏蛋白病患者临床资料进行回顾性分析,包括临床、组织病理、误诊及治疗情况.结果:该病多见于中青年男性,皮损好发于头部;14例患者均有毛囊周围细胞浸润和黏蛋白沉积(占100％);对羟氯喹的治疗效果均不好;14例中误诊为8例(占57％).12例患者为慢性良性型,2例属于恶性型.结论:该病皮损主要分布头部,基本皮疹为浸润的斑块和结节.皮损组织病理检查显示毛囊及其周围有黏蛋白聚积,阿新蓝染色阳性提示诊断毛囊黏蛋白病的线索.     

肢端持续性丘疹性黏蛋白沉积症

报告1例肢端持续性丘疹性黏蛋白沉积症。患者男,62岁。双前臂、双手背丘疹10年。皮损组织病理检查:真皮浅中层黏蛋白沉积,未见成纤维细胞增生。黏蛋白阿新蓝染色阳性。     

不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿1例国内首报

报告1例不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.患者女,53岁.全身泛发红色毛囊性丘疹伴瘙痒1年.皮肤科检查见躯干、四肢泛发针头大红色毛囊性丘疹,密集分布,互不融合,外观酷似突起的鸡皮疙瘩,触之粗糙.皮损不累及头面部、手掌和足部.皮损组织病理检查:毛囊周围可见以淋巴细胞为主的细胞浸润,部分细胞侵入毛囊上皮和表皮,细胞深染.核周有空晕,异形不明显.免疫组化染色结果证实浸润的淋巴细胞主要是T淋巴细胞.皮损T细胞受体(TcR)基因重排阳性.诊断:不伴有毛囊黏蛋白沉积的向毛囊性蕈样肉芽肿.     

色素性神经纤维瘤六例

6例色素性神经纤维瘤中男3例,女3例,年龄8～55岁.6例患者的皮损均表现为褐色斑片或斑块,3例皮损位于左颈部及躯干、四肢,1例皮损位于右腋窝及左上臂,1例皮损位于左前臂伸侧,1例皮损位于腰臀部,皮损2 cm×3 cm至30 cm×40 cm.3例合并Ⅰ型神经纤维瘤病.皮损组织病理主要表现为表皮轻度角化过度,基底层色素增加,真皮和皮下脂肪组织内可见肿瘤细胞呈弥漫性分布,肿瘤组织无包膜,主要由大量的梭形细胞构成.肿瘤组织中散在或簇集状分布黑素细胞,胞质内可见黑素颗粒.免疫组化显示S100(+)和波形蛋白(+).6例患者均未治疗.色素性神经纤维瘤少见,临床和组织病理上,需与Becker痣、先天性黑素细胞痣、色素性隆突性皮肤纤维肉瘤等鉴别.     

线状和漩涡状痣样过度黑素沉着病1例

患儿女,10岁。躯干及四肢色素沉着斑10年。皮肤科情况:躯干及四肢可见沿Blaschko线分布的线状、漩涡状褐色色素沉着斑片,边界清楚,表面无鳞屑和萎缩。皮损组织病理示:表皮轻度角化过度,基底层色素增加,灶性基底层液化变性,真皮浅中层小血管周围少量淋巴细胞、噬黑色素细胞浸润。诊断:线状和漩涡状痣样过度黑素沉着病。     

Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern

Background Interstitial granulomatous dermatitis (IGD) is a rare disease for which a clinical–pathological correlation is essential to establish diagnosis. Objectives To describe the histological and clinical features of patients with IGD seen in our department from 2004 to 2010, and to undertake a literature review and critical analysis of additional cases. Methods Twelve adult patients (nine women and three men; mean age 58·5 years; range 32–73 years) with IGD were enrolled. Lesions consisted of asymptomatic erythematous papules and plaques, symmetrically distributed on the trunk and the proximal limbs. Two patients had skin‐coloured papules. Six patients had articular involvement (arthralgias, spondyloarthritis, rheumatoid arthritis) and three patients had cancer. Results All cases showed a predominant CD68‐positive macrophage infiltrate distributed between collagen bundles of the mid‐ and deep dermis. Macrophages were also surrounding degenerated collagen fibres. A few neutrophils and/or eosinophils were also present. No vasculitis or significant mucin deposition was observed. Of the 62 cases of IGD reported since 1993, 53 fulfilled stringent diagnostic criteria. Erythematous papules and plaques on the trunk and proximal limbs were the dominant manifestation. Approximately 10% of patients had cord‐like lesions. More than 50% of patients with IGD had arthralgia or arthritis, and less commonly other rheumatic disorders. Disease duration is months to years, but long‐term prognosis seems favourable. Conclusions IGD is a distinct entity with a typical histological and clinical pattern. The importance and the nature of the association with extracutaneous diseases remains to be clarified. Patients should be screened for rheumatic and autoimmune diseases.     

白色纤维性丘疹病1例

报告1例白色纤维性丘疹病.患者女,28岁,双上肢及上背部丘疹1年,既往鱼鳞病病史20年.皮肤科检查:双上肢及上背部散在白色丘疹,直径2～3 mm,瓷白色,未有融合倾向.皮损组织病理检查示表皮网篮状角化过度,真皮浅层血管周围少量淋巴组织细胞,真皮中上层胶原纤维束增粗.弹性纤维染色显示真皮浅层弹性纤维减少.胶原纤维及弹性纤...     

CD34+ dermal dendritic cells and mucin deposition in dermatomyositis

Dermal mucinosis is often associated with collagen diseases such as rheumatoid arthritis, lupus erythematosus, and dermatomyositis, in addition to autoimmune thyroiditis. We report eight cases of dermal mucin deposition secondary to typical dermatomyositis with cutaneous lesions known as heliotrope rash and Gottron’s papules. Striking mucin deposition was observed in both the papillary dermis and reticular dermis of all biopsy specimens. Immunohistochemical analysis showed that CD34+ dermal dendritic cells (DDCs) in the perilesional area in combination with vimentin+ cells within the mucinous lesion might be important in giving rise to abnormal deposition of dermal mucin. On the other hand, numbers of factor XIIIa+ DDCs and tryptase+ mast cells were reduced within and surrounding the mucin deposition, as compared with those in the dermis of normal controls. A pathogenic mechanism of dermal mucin deposition is proposed.     

Confetti‐like macular atrophy: A new entity?

Anetoderma, is characterized by herniated atrophic macules clinically and by decreased to absent dermal elastic fibers histopathologically. Atrophoderma is characterized by depressed, atrophic, pigmented patches clinically and by thickened and homogenized dermal collagen bundles with absence of elastolysis histopathologically. Atrophoderma elastolyticum discretum describes lesions that are reminiscent of atrophoderma clinically but they are compatible with anetoderma histopathologically. A 34-year-old female patient presented with diffuse, hypopigmented, atrophic, shiny macules on the upper limbs and upper trunk. Histopathological examination revealed an atrophic epidermis with disorganized, hyalinized and coarse collagen bundles in the middle and lower dermis. Elastic fiber loss and fragmentation were detected in the upper dermis. The other patient was a 42-year-old female patient. She applied with diffuse, hypopigmented, shiny, atrophic macules on the upper limbs and upper trunk. Histopathological examination revealed findings that were similar to those of the first case but there was near complete loss of elastic fibers throughout the whole thickness of dermis. Our cases did not show depressed or herniated atrophic macules clinically but the macules were at the same level with the surrounding healthy skin. Histopathological findings in these cases showed the histopathological features of both atrophoderma and anetoderma. These two cases are interesting because they may represent a clinicopathological entity which has not been described before.     

Nevoid follicular mucinosis: a new type of hair follicle nevus

Follicular mucinosis represents a term for a histopathologic reaction pattern in follicular epithelium. It is a characteristic of alopecia mucinosa. However, it may also occur in a variety of unrelated conditions. Epidermal nevi are considered to be hamartomatous disorders and they can show a predominant component of non‐organoid (keratinocytes) and/or organoid nevi. All the cases of epidermal nevi described with mucin deposits until now are reported as mucinous nevus or mucinous eccrine nevus; in the first type of disorder, diffuse mucin deposition is only seen in the papillary dermis, and in the second type, the mucin is found around the proliferation of eccrine structures. We believe this is the first reported case of epidermal nevus along Blaschko's lines exhibiting typical microscopic findings of mucinosis exclusively distributed inside the follicular epithelia.     

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.     

毛囊黏蛋白病

报告1例毛囊黏蛋白病。患者男,33岁。全身泛发红斑伴瘙痒13年余,加重近半年,并出现眉毛脱落。皮损组织病理检查示：真皮毛囊周围炎性细胞浸润。阿新蓝染色阳性,毛囊内可见黏蛋白沉积。诊断为毛囊黏蛋白病。     

Cutaneous lupus mucinosis: a review of our cases and the possible pathogenesis

Cutaneous lupus mucinosis (CLM) is a rare variant of lupus erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology of CLM mainly revealed abundant mucin deposits among splayed collagen bundles throughout the dermis. However, some CLM lesions showed discoid lupus erythematosus-like epidermal and dermal changes and/or lupus profundus. Vasculitis was also revealed in the CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to its two important features, the male preponderance and the association with SLE. Vasculopathy may also be involved in the development of CLM.     

Reed痣26例临床病理特征分析

【摘要】 目的 分析总结Reed痣的临床病理特点。方法 分析2015年1月至2019年6月于第四军医大学西京皮肤医院确诊的26例Reed痣患者的临床病理特征。结果 男15例,女11例,平均发病年龄17.35岁,中位发病年龄13.85岁。发病部位：下肢12例,面部6例,上肢5例,躯干3例。皮疹颜色均呈黑色,7例为斑疹,19例为扁平丘疹,22例皮疹形态类圆形,4例皮疹形态不规则。皮疹直径2 ~ 10 mm,23例直径 ≤ 5 mm。组织病理：15例为交界痣,11例为混合痣,26例黑素细胞形态均呈梭形,细胞色素明显,4例痣细胞巢与周围表皮融合,22例与周围表皮形成明显的裂隙,均未见明显细胞异型性及有丝分裂象,6例可见Kamino小体。皮损均予手术切除,随访1 ~ 5年无复发。结论 Reed痣皮疹形态可不规则,组织病理可出现呈Paget样分布的大小不一梭形细胞,需与黑素瘤鉴别。     

恶性萎缩性丘疹病1例

患者男,23岁。躯干、四肢反复出现丘疹2年,丘疹中央可坏死,愈后遗留瓷白色萎缩性瘢痕,周围绕以水肿性红晕,无任何自觉症状。皮损组织病理示:角化过度,棘层萎缩变薄,皮突消失,真皮浅层见带状的胶原坏死区,其下少许黏蛋白沉积,未见明显的血管病变。诊断:恶性萎缩性丘疹病。