泛发性硬化性萎缩性苔藓1例

患者,女,61岁。躯干泛发斑片状皮疹4年,外阴瓷白色斑块3年。外阴部及前胸部病理检查示:表皮角化过度,基底层液化变性,真皮浅层胶原纤维水肿、均质化,考虑为硬化性萎缩性苔藓。患者既往有甲状腺功能减低及多系统慢性炎症疾病。     

白癜风合并甲亢及胫前黏液性水肿1例

患者男,52岁,双下肢色素脱失斑10年,继发淡红色斑块、结节3个月。皮肤科情况:双胫骨前大片状或点状白斑,散在浸润性淡红色斑块、结节,局部坚实肿胀,加压无凹陷,毛孔粗大呈橘皮样外观,毳毛旺盛粗黑。淡红色斑块组织病理示:胫前黏液性水肿;甲状腺功能检查符合甲亢。诊断:白癜风合并甲亢及胫前黏液性水肿。予甲亢相关治疗后甲亢控制,胫前黏液水肿轻微改善,白癜风无进展。     

泛发型环状肉芽肿合并糖尿病1例

患者女,67岁。四肢、颈部丘疹1年,伴血糖升高。皮疹呈环状扩大,中央消退,周边隆起。皮损组织病理示:真皮内可见胶原纤维、弹力纤维变性,黏蛋白沉积,组织细胞及多核巨细胞呈栅栏状排列。诊断:泛发型环状肉芽肿合并糖尿病。     

22例SCLE临床及实验室观察

本文分析了22例亚急性皮肤型红斑狼疮(SC-LE)的临床及实验资料,并与以往报告的160例SLE及Sontheimer等报告的27例SCLE进行了比较.SCLE较SLE皮疹及光敏发生率高,而肾脏及中枢神经系统受累较少,同时发现SCLE的血液系统受累也较少.SCLE病人中的抗Ro(SSA)和(或)抗La(SSB)抗体阳性率为63.6%我们还发现,在SCLE中,环形红斑型肾脏及中枢神经系统受累更少,低补体血症发生率也低,而抗La(SSB)抗体及类风湿因子阳性率较高,约半数病人可以不用激素治疗,对DDS的治疗反应较好.丘疹鳞屑型则均需要激素治疗.我们认为SCLE是红斑狼疮谱中一个预后较好的亚型:而环形红斑型则为一个预后更好的SCLE亚型.     

14例亚急性皮肤型红斑狼疮临床分析及中医、中西医结合治疗

目的 观察亚急性皮肤型红斑狼疮(SCLE)的临床特征和中药治疗、中西医结合治疗的效果。方法 对SCLE的临床表现和实验室检查进行分析；所有病例予青凌草方治疗，若皮疹多，合用羟氯喹或少量皮质类固醇。3个月后评价治疗效果。结果 SCLE的光敏感发生率高(57．14％)，抗Ro抗体阳性率高(64．29％)，而较少合并内脏损害。经3个月治疗，临床缓解率35．71％，显效率28．57％，有效率21．43％，无效率14．29％。结论 SCLE有鲜明的临床特征；青凌草方对SCLE有较好的治疗效果。     

皮肤脉管炎与长期的硫氧嘧啶治疗

硫氧嘧啶通常是作为他巴唑或甲亢平的替换药治疗甲状腺毒症的。作者报道一例长期应用甲基硫氧嘧啶引起的皮肤脉管炎。一例21岁的白人妇女,主诉咽痛数天后出现呕吐,腹泻及腹部绞痛3天。4年前患者被诊断为甲状腺毒症,治疗开始用甲亢平,但2周后因进行性咽痛而改用甲基硫氧嘧啶以后的4年内一直持续治疗直至本次入院前。入院时患者有紫罗兰色的皮疹分布于鼻、左耳和两侧颊部。右髂窝轻度触痛,但无其它异常体征。最初的化验结果如下:电解质正常;血红蛋白13.9g;白细胞4.0×     

副肿瘤性亚急性皮肤型红斑狼疮:合并肺部肿瘤的一例报告

报告1例合并肺部肿瘤的副肿瘤性亚急性皮肤型红斑狼疮(SCLE)。患者皮损符合副肿瘤性皮病的特征,即:皮损出现于肿瘤形成之后;皮损和肿瘤具有相同的临床病程,对恶性肿瘤的治疗可导致皮疹的消退。据文献记载此前仅有5例报道。其中乳癌2例、胃癌、肺癌和子宫癌各1例。     

以皮肤表现为首发症状的Wegener肉芽肿1例

患者男,51岁。右下肢暗紫红色皮疹半年余。患者近2个月反复出现口腔溃疡,尿常规检查结果示:潜血(+)。皮损组织病理示:表皮大致正常,真皮全层及皮下脂肪间隔可见以血管为中心的肉芽肿性改变,中央为变性坏死的血管,周围可见大量组织细胞、浆细胞及少量淋巴细胞浸润,并可见多核巨细胞。结合临床、尿常规及组织病理检查诊断为:Wegener肉芽肿。     

皮肌炎和疱疹样皮炎

本文报告一例67岁、女性患者,在20年内先后发生原发性甲状腺机能减退同时伴有皮肌炎、疱疹样皮炎和干燥综合征.1960年开始患甲状腺机能减退.1974年当停用甲状腺药物2周后,实验室指标包括T4、TSH、抗甲球蛋白抗体等均证实此诊断.替代疗法2月后,又恢复到正常.同年发生坐位时站起困难.根据检查发现近端肌无力,此时肘部出现红斑性丘疱疹伴瘙痒.肌肉活检示炎症和变性改变及肌病型肌电图.CPK600u(正常<175u).诊断为多发性肌炎.每日服强的松40mg,肌无力明显好转,肘部皮疹消退,数周内CPK也恢复正常.但肌无力症状尚存在.以后强的松减为每日15mg.     

皮肌炎合并纵隔及皮下气肿1例

患者女,43岁,周身无力1个月余,面部、颈部皮疹伴发热20d。查体:右侧锁骨上窝及右颈部有握雪感,胸部CT示:纵隔气肿。诊断:皮肌炎合并纵隔及皮下气肿。     

姐妹同患硬斑病合并桥本甲状腺炎二例

报告姐妹俩同患硬斑病合并桥本甲状腺炎。例1 女,64岁,颈前、躯干和双胫前皮疹5年,会阴部皮疹伴瘙痒4年,既往桥本甲状腺炎病史9年。体检：甲状腺Ⅰ度肿大,质韧,无突眼、胫前黏液水肿等表现。颈部、前胸淡红色斑片,后背部硬化萎缩,会阴部瓷白色斑块。皮损组织病理提示：（前胸）硬斑病,（外阴）硬化萎缩性苔藓。例2 女,例1的妹妹,55岁,左乳下及腹部皮肤逐渐硬化萎缩4年,既往桥本甲状腺炎病史3年。体检：甲状腺Ⅰ度肿大,质韧,无突眼、胫前黏液水肿等表现。左乳下、腹部和后背中央皮肤色素减退,硬化萎缩。皮损组织病理提示：硬斑病。根据临床特点、组织病理、过碘酸-雪夫染色和甲状腺功能检查,2例均诊断为硬斑病合并桥本甲状腺炎。     

表皮松解性角化过度鱼鳞病一例

5岁女性患儿,全身皮肤潮红5年,出现干燥及角化增厚4年。皮损组织病理示:表皮显著角化过度,颗粒层细胞内见不规则的透明角质颗粒呈空泡样变性改变,棘层不规则增厚,真皮浅层血管周围少量炎性细胞浸润。基因突变检测示KRT10位点突变,基因编码区478号碱基由T变为A。诊断:表皮松解性角化过度鱼鳞病。给予局部外用0.1%维A酸乳膏每日2次及皮肤保湿剂治疗,40 d复诊时皮损明显好转,全身皮肤基本正常。     

An unusual case of genodermatosis with familial gastrointestinal polyposis,angiomatous malformation and ascites

Gardner's syndrome is a familial adenomatous polyposis syndrome with extraintestinal manifestations, characterized by the coexistence of intestinal polyposis with an early risk of malignant degeneration and extraintestinal manifestations mainly involving the skin, eye, bone and thyroid. We describe an unusual case of intestinal adenomatous polyposis, retinal hypertrophy, fibromas of the skin, bone and thyroid tumors accompanied by congenital arteriovenous malformations with lethal complications.     

Verrucous form of chilblain lupus erythematosus

A 45-year-old woman had symmetrical livid plaques with yellowish hyperkeratoses for 5 years, which progressed on to the fingers and toes and on the soles of the feet. Two years later creamy, whitish areas and maceration appeared on the buccal mucosa and the lips. A skin biopsy revealed massive collagen hyaline degeneration in the perivascular area, hyperkeratosis and hypergranulosis, small lymphocyte infiltrates with several melanophages and extravasates of erythrocytes in the upper corium in perivascular areas and hydropic degeneration of basal keratinocytes. The findings using direct immunofluorescence were compatible with lupus erythematosus (LE). Laboratory investigation showed a slight leucopenia and thrombopenia, a slightly elevated erythrocyte sedimentation rate, hypocomplementaemia C3 and C4, a high titre of rheumatoid factor and antinuclear antibodies positivity of extractable nuclear antigen. The results reflected probably the development of a systemic form of the disease. The patient was successfully managed by methylprednisolone and hydroxychloroquine. After 1 year of therapy, a new skin biopsy revealed a substantial reduction of hyperkeratosis and hyaline degeneration of collagen tissue in the perivascular areas. The combination of the extensive hyperkeratosis and hyalinization thus seems to be features of the long-lasting, untreated lesions in chilblain LE.     

Ultrastructure of the microvasculature in skin affected by systemic scleroderma

The microvasculature of the upper dermis in cases of systemic scleroderma was examined with an electron microscope. Microvessels in clinically uninvolved skin areas showed nearly normal structure. On the other hand, in moderately involved skin, enlargement of the vascular lumen, endothelial swelling, increase in the pinocytotic vesicle and microvilli and reduplication of the basal lamina were observed. Along with these, Ruthenium red-positive granules, microfibrils and fine collagen fibrils were perivasculary increased. Markedly involved skin revealed a degenerative change in the endothelial cells with interendothelial gap; in the perivascular region the number of microfibrils and Ruthenium red-positive granules decreased, and were replaced by increased collagen fibrils.     

Ultrastructural aspects of primary anetoderma

Anetoderma is a rare cutaneous disorder characterized by focal loss of dermal elastic tissue due to unknown mechanisms. Primary anetoderma develops on clinical normal skin, without any preceding dermatosis and it can be associated with autoimmune conditions. Secondary anetoderma develops on the same area of a previous disorder, such as infectious, neoplastic or inflammatory diseases. A 37‐year‐old female patient noticed for 4 years circumscribed, normochromic, asymptomatic herniated plaques on the trunk and upper limbs. Family history was negative. Only a positive antinuclear factor (ANF) test, with titer of 1:160 and nuclear homogeneous pattern was found. Light microscopy with Weigert staining showed a lessening of elastic fibers with fragmentation; the oxytalanic fibers were also affected or absent. Transmission electron microscopy showed fragmentation and granular degeneration of elastic fibers. With greater magnification, fragments similar to those seen with optical microscopy were identified. The collagen fibers did not present any alteration. The examination of the dermis with scanning electron microscopy also identified fragmentation and significant fissures of the elastic tissue, granular degeneration was also observed. With greater magnification fragmented elastic fibers were seen.     

深在性红斑狼疮1例

患者女,37岁。右上臂斑块1年。皮肤科情况:右上臂前侧和后侧各见约1.5×2.5cm,1.5×4.0cm大小的淡红色浸润性斑块,表皮萎缩,质地较硬,活动度差,无压痛。皮损组织病理示:角化过度,表皮萎缩,基底细胞空泡化变性,真皮及皮下脂肪组织小血管扩张充血,小血管周围伴大量灶状淋巴细胞浸润,皮下脂肪小叶内大量淋巴细胞浸润。直接免疫荧光(LBT):表皮基底膜带C3,IgM呈线状沉积。诊断:深在性红斑狼疮。硫酸羟氯喹0.2g口服,2次/d,白芍总苷0.6g口服,3次/d,卤米松软膏、多磺酸黏多糖软膏外用。3周后皮损变软,面积变小。     

ELECTRON MICROSCOPY OF THE SKIN IN ERYTHROPOIETIC PROTOPORPHYRIA

SUMMARY. —An electron microscopical examination has been made of skin from 4 patients with erythropoietic protoporphyria. Both exposed areas and skin usually covered by clothing were studied.
In exposed skin from the knuckle and dorsum of the hand the main abnormality is associated with small blood vessels in the upper part of the dermis. The amorphous material seen on light microscopy is found to consist of a multilayered partially fragmented basement membrane, and finely fibrillar material of moderate electron density which permeates and surrounds the vessel walls. The presence of this substance in the vessel walls and its abrupt junction with the normal dermal connective tissue supports the idea that it is derived from constituents of the blood or from the vessels themselves rather than by degeneration of dermal connective tissue.
Nothing abnormal is seen in covered skin from the back.     

Degenerative alterations of dermal collagen fiber bundles in photodamaged human skin and UV-irradiated hairless mouse skin: possible effect on decreasing skin mechanical properties and appearance of wrinkles

Dermal collagen fiber bundles (DCFB) are the major constructional element in the dermis. Although degenerative alterations of DCFB have been reported in chronologically aged skin, changes in photodamaged skin have not been fully investigated. We report ultrastructural alterations of DCFB, and their relation to skin elasticity using photodamaged human skin and UV-irradiated hairless mouse skin. The degree to which DCFB were intact and closely packed was evaluated and scored blindly. Exposed skin (outer forearm) exhibited marked ultrastructural degeneration. In UV-irradiated hairless mouse skin, the intact ultrastructural appearance of DCFB was gradually lost with increasing UV dosage; however, marked alterations in DCFB ultrastructure were absent in either human inner upper arm (unexposed) skin or nonirradiated age-matched control mouse skin. Skin mechanical properties were measured using a Cutometer SEM 474 suction extensometer, recording Ue* immediate deformation, Uv* viscous deformation, Uf* final deformation, and Ur* immediate contraction, all normalized for skin thickness. Uf*, Ue*, Uv*, and Ur/Uf were significantly decreased in exposed compared with unexposed skin. Significant positive correlations between degenerative alterations of DCFB and the decrease in Uf*, Ue*, and Uv* were seen. Changes of "% area of wrinkles" in UV-irradiated mouse skin was significantly correlated with degenerative changes of DCFB. Based on these results, we confirm observations made by others that chronic photodamage may have more severe effects on degeneration of DCFB than that of chronologic aging alone. Furthermore, degeneration of DCFB as detected ultrastructurally may, by its effect on skin elasticity, result in an increase in the appearance of wrinkles.     

皮肌炎合并胸椎管内副神经节瘤一例

患者男,20岁,双上肢肌肉疼痛、四肢肌无力1月余。体检：面颊、双上眼睑、胸颈及双手背弥漫性红斑,双上肢近端肌力Ⅳ级,远端肌力Ⅴ级,双下肢近端肌力Ⅲ级,远端肌力Ⅴ级。实验室检查：肌酸激酶2103 U/L,肌酸激酶同工酶MB （CK-MB） 83 U/L,乳酸脱氢酶489 U/L,均高出正常范围。肌电图示肌源性病损,神经传导速度正常。腓肠肌活检示肌纤维肥大为主,部分肌纤维肿胀,横纹模糊或消失,肌纤维间淋巴样细胞浸润。上胸部皮损组织病理检查：基底细胞液化变性,基底细胞层可见胶样小体,真皮内血管周围淋巴样细胞浸润。结合患者临床表现及检查,诊断为皮肌炎。治疗：甲泼尼龙80 mg/d静脉滴注及对症治疗4周后,患者双上肢肌力好转,肌酸激酶、CK-MB、乳酸脱氢酶均恢复正常。但患者双下肢肌力下降逐渐加重,并出现感觉障碍,MRI平扫加增强示胸椎11 ~ 12水平椎管内占位,外科手术切除直径约3 cm类球形包膜完整肿瘤,组织病理及免疫组化检查诊断为椎管内副神经节瘤。该患者诊断皮肌炎合并胸椎管内副神经节瘤。