白色萎缩1例

白色萎缩是种好发于下肢的局限性皮肤血管病，由Milian在1929年首次描述,并命名为白色萎缩。本病又称Milan白色萎缩、节段性透明性血管炎、有溃疡的网状青斑、青斑性血管炎。现将笔者所见1例报告如下。     

白色萎缩1例

患者男 ,18岁。双小腿、踝部、足跟部反复出现瘀点、瘀斑、水疱、溃疡及象牙白色萎缩斑 ,伴疼痛。皮损组织病理为真皮血管增生、充血 ,血管内有透明血栓形成 ,管壁增厚 ,管腔闭塞 ,血管周围有少数淋巴细胞浸润 ,伴红细胞外渗。本例通过抑制血栓形成 ,改善微循环 ,局部中药蒸发罨包痊愈。     

白色萎缩1例

患者男，32岁，因双踝部反复红肿，溃烂9年于2003年12月12日来我科就诊，患者自1994年5月无明显诱因出现双踝部轻度红肿，左踝内侧有一黄豆大溃疡，伴黄色渗液，自服抗生素后愈合。以后每年5—9月份双踝部均出现类似皮损，冬季溃疡愈合。曾在当地医院就诊，双下肢X线摄片正常，诊断不明。     

白色萎缩1例

患者男,33岁.双侧踝内侧及足弓内侧反复出现红斑、瘀斑、溃疡伴疼痛1年余,于2011年4月10号到我科就诊.患者于1年前无明显诱因双侧踝内侧及足弓内侧出现紫红色的斑疹,数日呈紫红色瘀斑,少数皮损破溃,形成溃疡,伴疼痛,在外院诊断为变应性皮肤血管炎,经治疗(具体用药不详),效果不明显.溃疡愈合缓慢,愈后遗留白色萎缩性瘢痕.愈合前和愈合后原发皮损周围出现新发皮损,反复发作.病程中无发热、关节痛,腹痛及雷诺现象.患者既往体健,否认系统性疾病史,家族成员无类似疾病史.     

白色萎缩1例

患者女,46 岁.因左踝周疼痛性红斑反复发作2 年余,再次发作2 个月余,于2009 年11 月10 日来我院就诊.皮损初发于左踝周,为数枚米粒至绿豆大红色斑疹,散在分布,数日后变为暗紫红色,后逐渐扩大至1 分硬币大片状斑疹,伴轻微触痛.局部自行破溃形成浅溃疡,溃疡缓慢愈合,局部遗留白色萎缩性瘢痕及色素沉着,部分皮损中央遗留不规则黑色结痂,长期不脱落.自觉烧灼感,偶有针刺样疼痛,久站后踝周肿胀,疼痛加重,皮损反复发作.2 个月前左踝周再次出现类似疼痛性红斑,外院诊断为"变应性血管炎",给予口服雷公藤多苷、维生素B6、抗组胺药等治疗,皮损缓解不明显.病程中无发热、关节痛、腹痛,无雷诺现象.家族中无类似疾病患者.     

白色萎缩1例

患者男,38岁.因发作性头痛1年余,说话迟钝伴记忆力下降7个月余,于2008年2月4日入院.患者于2006年11月-2007年6月反复出现头痛、呕吐,可自行缓解.2007年6月出现语速变慢,记忆力下降,反应稍迟钝,持物时双手颤抖,偶有饮水、进食时呛咳,声嘶,视物模糊,症状逐渐加重.     

白色萎缩1例

患者女,42岁。因双踝部出现瘀斑、萎缩性瘢痕4年,于2004年6月来我所就诊。皮损初发于左踝部,为紫红色斑疹,数日后呈暗紫色,少数皮损破溃,呈粟粒大溃疡,伴疼痛,表面有少量黄色分泌物。溃疡缓慢愈合,遗留白色萎缩性瘢痕和色素沉着,愈后不久原部位又有新发皮损,并向四周蔓延,渐累及右踝部。皮损反复发作。体格检查:系统检查未见异常。皮肤科检查:内、外踝部见4处直径5～8cm暗红斑,边缘不规则,其间布有较密集的针头大紫色瘀点,中心有粟粒大溃疡,表面覆血痂、白色鳞屑,散在形状不规则和星状瓷白色萎缩性瘢痕及色素沉着(图1)。皮损组织病理检查:表…     

白色萎缩1例

1 临床资料患者女,19岁.因双小腿反复起红色斑疹、溃烂,伴局部疼痛3年,于201 1年6月3日入院治疗.3年前无明显原因双小腿起绿豆大小的红色斑疹,局部疼痛,3周后皮疹增多,新起小水疱,局部坏死,呈虫咬样溃烂,疼痛加重,在本地拟诊血管炎予丹参、泼尼松等药物治疗,病情好转;6个月后双小腿皮疹复发,溃烂加重,愈后留白色疤痕及色素斑.1年前红斑渐波及双踝及双足背部,行走活动受限,在我院门诊对症治疗无效而入院.既往体健,无吸烟史,否认有家族遗传病史.体格检查一般情况好,系统检查未见异常.皮肤科检查:皮疹对称分布于双小腿伸侧、双踝及双足背部,可见淡红色斑疹、瘀斑、白色瘢疤及色素沉着斑,间有形状各异,大小不一的虫蚀状溃疡,表面渗出少许脓性分泌物,周边红肿,明显压痛,见图1.     

白色萎缩1例

报告白色萎缩１例。女性，２６岁，双下肢反复起皮疹溃疡１３年。检查：双下肢象牙白色萎缩性白斑，周围有毛细血管扩张，紫癜。病理示真皮浅层血管壁纤维蛋白样物质沉积及血管周围淋巴细胞浸润，直接免疫荧光示真皮浅层、小血管壁ＩｇＧ、ＩｇＭ、Ｃ３沉积。用低分子右旋糖酐及复方丹参静滴后皮损消退，随访６周无复发。对此病的病因、分类及治疗进行了讨论。     

白色萎缩l例

患者女性,28岁,临床表现为小腿下段早期紫癜性损害伴疼痛,以后变成溃疡,溃疡愈合后,留下白色萎缩性瘢痕,有青斑样色素沉着。组织学检查示真皮血管节段性,透明变性,内皮细胞肿胀及血栓形成,血管周围不等量淋巴细胞浸润。     

Atrophie blanche: a review from the perspective of a 31 patient cohort

ABSTRACT: This article combines review of the distinctive healing pattern most commonly known as atrophie blanche with some previously unpublished observations from perhaps the largest patient cohort reported. A pragmatic approach to this painful, chronic, ulcerative and scarring disorder of the lower limbs is taken. Emphasis is placed on examination of the patient once the clinical pattern is diagnosed. Treatment is also from a very practical approach, with literature references given for the various strategies.     

角层下脓疱性皮病合并结缔组织病二例

报道2例角层下脓疱性皮病，皮损均表现为红斑基础上的水疱、脓疱，组织病理学特征为角层下脓疱，直接免疫荧光阴性；脓疱疱液细菌培养阴性。患者1，女，53岁，合并类风湿关节炎。口服阿维A，糖皮质激素和羟氯喹治疗。患者2，女，69岁，合并干燥综合症及自身免疫性肝炎，予糖皮质激素及米诺环素治疗。2例患者均好转。     

Nailfold digital capillaroscopy in 447 patients with connective tissue disease and Raynaud''s disease

The presence of megacapillaries and a decreased capillary density are the hallmarks of the scleroderma capillary pattern, which can be detected by nailfold capillarmicroscopy. One hundred and eighty‐six patients with Raynaud's phenomenon, 65 cases with undifferentiated connective tissue disease (UCTD), 47 patients with systemic lupus erythematosus (SLE), 26 patients with dermato/polymyositis, 14 with rheumatoid arthritis, seven cases with primary Sjögren's syndrome and 102 patients with systemic sclerosis (SSc) were investigated. Of the 16 patients with diffuse cutaneous SSc and the 86 limited cutaneous SSc cases, 14 (87.5%) and 53 (61.6%) showed the scleroderma capillary pattern, respectively. Nine of the 65 (13.8%) cases with UCTD and 24 of the 186 (12.9%) cases with Raynaud's phenomenon also exhibited the same pattern. Four of the 47 (8.5%) with SLE and seven of the 26 (26.9%) with dermato/polymyositis, and no patients with rheumatoid arthritis or Sjögren's syndrome, exhibited the scleroderma capillary pattern. The conclusion is that the scleroderma capillary pattern is often present in SSc and dermato/polymyositis. Furthermore, patients with Raynaud's phenomenon and UCTD may also occasionally exhibit this pattern. Therefore, capillarmicroscopy seems to be a useful tool for the early selection of those patients who are potential candidates for developing scleroderma spectrum disorders.     

白色萎缩7例报告与文献复习

目的：提高对白色萎缩的认识,探讨其诊断依据和有效的治疗方法。方法：对2002年以来我院住院的7例本病患者的临床资料进行回顾性分析。结果：本病发病年龄以中年以上女性居多,皮损表现为双小腿、踝部、足部散在淡红色和紫红色斑疹、斑片,逐渐破溃、渗液、溃疡、结痂、萎缩形成象牙白色瘢痕,夏重冬轻。实验室检查无特异性;组织病理改变为真皮浅层小血管数量增多,血管内皮细胞显著增生,管腔狭窄,少数血管壁有纤维蛋白样物质沉积及玻璃样变、透明血栓形成,血管周围有少量淋巴细胞和组织细胞浸润。结论：本病主要根据临床特点和组织病理诊断。目前,以大剂量双嘧达莫、小剂量阿司匹林联合治疗为首选;其他还可选用纤维蛋白溶解药、抑制血小板聚集药,以及中药生地、山药;重症患者采用血浆置换术可获得近期良好的疗效。     

Undifferentiated connective tissue disease and its cutaneous manifestations

Undifferentiated connective tissue disease (UCTD, also named UCT syndrome, latent lupus or incomplete lupus) is regarded as an autoimmune disorder in which signs and symptoms are widely variable and evocative for connectivitis but not sufficiently evolved to fulfil any of the accepted classification criteria for the defined connective tissue diseases. In this paper we describe the case of a 47-year-old woman affected by UCTD according to the preliminary classification criteria supplied by Mosca et al. in 1999.     

Glucocorticoids in autoimmune connective tissue diseases

ABSTRACT: Glucocorticoids (GCs) are occasionally required for the cutaneous manifestations of autoimmune connective tissue diseases. In general, good therapeutic alternatives with fewer side effects than GCs are available, including anti-inflammatory agents such as antimalarials or dapsone, or immunosuppressives such as azathioprine or methotrexate, and these can serve as GC-sparing agents or can substitute for the use of GCs. When GC use cannot be avoided, it is important to implement a number of recommendations and improvements in dosing and prevention of side effects in order to optimize care. These include using appropriate and adequate doses of GCs initially, appropriate tapering regimens, and proper monitoring, prophylaxis, and treatment for infections, osteoporosis, avascular necrosis, hyperglycemia, hypertension, hyperlipidemia, and glaucoma.     

Cutaneous mucinosis in mixed connective tissue disease

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin orglycosaminoglycan in the skin and its annexes. It is described in some connectivetissue diseases but never in association with mixed connective tissue disease. Thisreport concerns two cases of cutaneous mucinosis in patients with mixed connectivetissue disease in remission; one patient presented the papular form, and the otherreticular erythematous mucinosis. These are the first cases of mucinosis described inmixed connective tissue disease. Both cases had skin lesions with no other clinicalor laboratorial manifestations, with clinical response to azathioprine in one, and toan association of chloroquine and prednisone in the other.     

Subcutaneous calcification of the lower legs in a patient with mixed connective tissue disease

A 56-year-old woman who had been treated for mixed connective tissue disease (MCTD) noticed a skin ulcer on the lower leg. There was no history of trauma. X-rays of the lower legs showed extensive calcification in the soft tissue. Biopsied tissue from the ulcer showed marked calcium deposition with necrosis. Laboratory findings revealed normal serum calcium and phosphate levels and normal parathyroid function. On the basis of these findings, we diagnosed skin ulcer due to subcutaneous dystrophic calcification associated with MCTD. The ulcer was gradually reduced in size and epithelialized by treatment with local debridement and antibiotics.     

未分化结缔组织病的研究进展

未分化结缔组织病是临床实际存在的一种疾病状态,目前发病机制尚不清楚。未分化结缔组织病的临床特征为具有自身免疫病的症状和体征,只有一种自身抗体阳性,不符合任何结缔组织病的诊断标准。演变是未分化结缔组织病疾病过程中最为突出的特点,30％可演变为结缔组织病,70％维持未分化结缔组织病的状态。经未分化结缔组织病演变而来的结缔组织病,病情相对轻微,重要器官损害较少。疾病特点和演变结果提示,未分化结缔组织病是自身免疫性疾病疾病谱中重要一员。