成人线状IgA大疱性皮病2例

报道2例成人线状IgA大疱性皮病。患者1,女,57岁。躯干、四肢红斑、水疱2个月,伴痒。皮肤科检查:躯干、四肢水肿性红斑,呈环形,部分边缘可见黄豆粒大水疱,疱液清,尼氏征阴性。患者2,女,43岁。躯干、四肢散发性水疱20天,伴痒。皮肤科检查:躯干、四肢绿豆至黄豆大水疱,疱液清,尼氏征阴性。两例患者组织病理检查均表现为:表皮下水疱,真皮乳头嗜中性粒细胞小脓肿;直接免疫荧光示:表皮基底膜带IgA线状沉积。     

成人线状IgA大疱性皮病1例

患者女,43岁。面颈部及躯干反复出现红斑和水疱7月。皮肤科情况:面颈部及躯干泛发水肿性红斑、丘疹和张力性小水疱,呈环状排列。皮损组织病理示:表皮下水疱,疱内及真皮可见大量嗜酸性粒细胞浸润,真皮层有少许中性粒细胞等炎性细胞浸润。直接免疫荧光见基底膜带IgA呈线状沉积。诊断:成人线状IgA大疱性皮病。予氨苯砜治疗2周,起效快,疗效好。     

多形红斑型玫瑰糠疹

患者男,52岁。躯干、四肢红斑鳞屑1个月。皮肤科检查:躯干、四肢环形或靶形水肿性红斑,部分皮损中央见银白色鳞屑,其游离缘向皮损中心,呈领圈状。躯干部皮损长轴与皮纹一致,呈圣诞树样排列。掌、跖、皮肤黏膜未累及。病理示表皮局灶性角化过度及角化不全,棘细胞内及细胞间轻度水肿,真皮乳头水肿,真皮浅层及小血管周见淋巴、单核样细胞浸润。诊断:多形红斑型玫瑰糠疹。     

妊娠性类天疱疮一例

27岁女性患者,躯干、四肢红斑伴痒4个月余,加重并出现水疱1 d。患者4个多月前(妊娠20~+周)四肢出现红斑、风团伴剧烈瘙痒,后逐渐扩散至躯干。1 d前(妊娠39~+5周)患者自然分娩后红斑加重,四肢出现张力性水疱、大疱,伴痛痒。体格检查:四肢、躯干大片红斑、丘疹,双上肢及股内侧红斑上见张力性水疱、大疱。红斑处组织病理示:表皮下裂隙性疱,真皮浅层血管扩张,血管周围炎性细胞浸润,有较多嗜酸粒细胞。直接免疫荧光示:C3、IgG在基膜带呈线状沉积。诊断:妊娠疱疹。未予治疗,病情逐渐好转,分娩后第20天全身水疱消退,未见新发皮损。     

疱疹样天疱疮1例

患者男,25岁。头皮红斑脱屑1年伴躯干四肢红斑水疱、瘙痒1月。体检:头皮、躯干及四肢伸侧呈环形或半环形排列的紧张小水疱,尼氏征阴性。皮损组织病理示:表皮内水疱及嗜酸性海绵形成,无棘层松解细胞,真皮混合炎症细胞浸润。直接免疫荧光示C3棘细胞间沉积。诊断:疱疹样天疱疮。     

大疱性扁平苔藓

报告1例大疱性扁平苔藓。患者男,51岁。躯干及四肢红斑、丘疹及水疱2个月余。皮肤科检查:躯干、四肢散在较多紫红色扁平丘疹、水疱,呈圆形、椭圆形或多角形,大小不等,界清,部分皮损上覆少许鳞屑。皮损组织病理检查:表皮角化过度,表皮下水疱,疱周基底细胞液化变性,真皮上部较多淋巴细胞呈带状浸润。诊断:大疱性扁平苔藓。     

大疱性蕈样肉芽肿

报告1例大疱性蕈样肉芽肿.患者男,40岁.因躯干、四肢红斑伴瘙痒6年,出现斑块半年、水疱1个月就诊.皮肤科检查:面部、躯干及四肢泛发红斑和斑块,部分斑块浸润明显.右下腹斑块上可见0.5 cm×2 ca的水疱.皮损组织病理检查:真皮浅层明显水肿,部分区域可见表皮下疱形成,真皮浅层可见异形明显的单一核细胞呈带状浸润.部分单一核细胞侵入表皮,形成Pautrier微脓肿.免疫组化染色结果示LCA( )、CD4( )、CD45RO( ).结合临床、免疫组化和组织病理检查结果,诊断为大疱性蕈样肉芽肿.     

淋巴瘤样丘疹病1例

患者女,60岁。躯干、四肢起红色水肿性丘疹2个月。组织病理检查示:真皮浅层、中层和血管周围致密淋巴细胞浸润,细胞大小不一,核形态不规则,染色深,部分细胞核呈脑回状。免疫组化染色结果:CD30(+)。诊断:淋巴瘤样丘疹病。     

累及皮下组织的Sweet综合征

报告1例Sweet综合征.患者女,28岁.发热6d,躯干及四肢结节3d.皮肤科检查:躯干、四肢及臀部多发直径1～3 cm的红色水肿性结节,压痛明显.皮损组织病理检查:真皮上部毛细血管内皮细胞明显肿胀,血管周围大量淋巴细胞及中性粒细胞浸润,真皮深部及邻近皮下脂肪中性粒细胞呈团块状浸润,可见明显核碎裂.诊断:Sweet综合...     

HPV疫苗引起的多形红斑1例国内首报

患者女,32岁,全身出现红斑、斑丘疹、水疱伴瘙痒10 d。皮肤科情况:躯干、四肢泛发粟粒至蚕豆大孤立或融合隆起性红斑、斑丘疹,部分红斑中央可见水疱,疱壁紧张,尼氏征(-),呈典型靶形损害特点。皮损组织病理示:表皮可见浆痂,表皮内水疱形成,表皮中可见坏死的角质形成细胞,上皮脚延长,基底层液化不明显,真皮乳头水肿,真皮浅深层血管及附属器周围均可见淋巴细胞浸润,伴散在嗜酸性粒细胞浸润。诊断:多形红斑。     

巨大浅表脂肪瘤样痣1例

患者女,22岁。出生后发即发现右侧胸部丘疹、结节等软性皮损,皮疹呈进行性增多,扩大至腋下。查体右侧胸部及腋下可见多个皮色或黄色的米粒至甲板大小扁平或半球形丘疹、结节、皮赘,触之柔软,部分皮疹融合成斑块。皮损组织病理示:真皮中上部胶原束间见脂肪细胞团块,呈片状或叶状分布,脂肪细胞形态成熟,发育正常。弹力纤维染色可见弹力纤维减少或短小、破碎。组织病理学诊断为浅表脂肪瘤样痣。腋下皮损行局部切除,疗效满意。     

毛囊漏斗部肿瘤

报告1例毛囊漏斗部肿瘤。患儿女，11岁。因左颞部斑块、结节11年就诊。患儿出生后左颢部即有一粉红色斑块．3个月前在斑块表面出现一小结节。皮损组织病理检查示真皮乳头层见呈水平盘状生长的肿瘤，与表皮多部位相连。肿瘤细胞染色偏淡，周围细胞呈栅栏样排列。诊断：毛囊漏斗部肿瘤。     

黏液性皮肤纤维瘤

报告1例黏液性皮肤纤维瘤.患者女,43岁.右前臂丘疹2周余.皮肤科检查:右前臂一直径约0.8 cm结节.组织病理检查示表皮不规则增生,真皮内可见大量梭形细胞增生,局部胶原排列疏松,阿新蓝染色阳性.免疫组化染色结果示波形蛋白(vimentin)阳性,CD31、CD34、平滑肌肌动蛋白(SMA)和S-100蛋白阴性.诊断为黏液性皮肤纤维瘤.     

Trichotemnomania: obsessive-compulsive habit of cutting or shaving the hair

A 28-year-old woman presented with a completely hairless scalp. The disorder had started 1 year ago, and at the same time she had developed dysphonia. During the past year, her hair disease had been diagnosed as alopecia areata totalis by many specialists, including several dermatologists. A close inspection of her scalp, however, revealed that no alopecia was present, because all infundibula were filled with a hair shaft that, on microscopic examination, showed cleanly cut surfaces. A scalp biopsy specimen showed completely normal structures. The pubic area was found to be covered with hair stubs of the same length. Therefore, a diagnosis of trichotemnomania was made. This term is derived from Greek thrix (hair), temnein (to cut), and mania (madness). After a stressful life event, the patient had developed both psychogenic dysphonia and the compulsive habit to remove the hair of her scalp, eyebrows, and axillary and pubic areas by shaving. Trichotemnomania is a distinct obsessive-compulsive disorder that should not be confused with trichotillomania. The condition should be taken into account when a supposed alopecia areata looks somewhat unusual.     

进行性对称性红斑角化症1家系报道

患者女,26岁。出生后6个月发病,双手、足对称性红斑角化25年。体检:一般情况良好。双侧掌跖及腕关节屈侧对称性分布边界清楚的角化性红斑,边缘覆以少许脱屑,压之不褪色,指/趾甲均未受累。实验室检查未见异常。手背侧缘皮损组织病理示:表皮明显角化过度,伴轻度角化不全,颗粒层及棘层肥厚,表皮增生至同一水平线,真皮小血管轻度扩张充血,管周少量淋巴细胞浸润。诊断:进行性对称性红斑角化症。     

A case of prurigo and lichenified plaques successfully treated with proton pump inhibitor.

A case of prurigo and lichenified plaques successfully treated with proton pump inhibitor is presented. She presented with pruritic eruptions, which showed marked lichenification and prurigo nodules, on her trunk and extremities. She had been treated with steroid ointment and H1-histamine receptor antagonist without success. Laboratory examinations revealed increased eosiophils and elevated lactate dehydrogenase. The skin biopsy specimen showed moderate acanthosis with spongiosis and lymphocytic and eosinophilic infiltration into the upper dermis. Because of vomiting and epigastralgia, endoscopical examination was performed, and an ulcer was found at the angle of her ventricle. A biopsy specimen disclosed a benign gastric mucosa with moderate inflammation within the lamina propria, and organisms consistent with Helicobacter pylori. Treatment for gastric ulcer with proton pump inhibitor (omeprazole) and aluminium hydroxide gel improved her eruptions and her pruritus resolved. She was discharged with complete cure of her eruption and ventricular ulcer. Our case indicates that gastric lesions induced by Helicobacter pylori infection may play an important role in dermatological diseases. Proton pump inhibitors including omeprazole are one of the choices for the treatment of some dermatological diseases including prurigo and lichenified plaques.     

皮损中有瓷白色斑的无色素痣

报告1例皮损中有瓷白色斑的无色素痣。患者女，24岁。出生时右侧颈肩部有色素减退白斑，近半年白斑中出现瓷白色斑，并逐渐增多，无自觉症状。皮肤科检查：右侧颈肩部带状分布片状不规则色素脱失斑，其间密集分布针尖至米粒大瓷白色色素脱失斑。白斑处组织病理检查：表皮角化过度。散在毛囊角栓，部分棘层轻度肥厚，基底层透明细胞增多，呈群集性分布，少量黑素颗粒。真皮浅层炎性细胞浸润。     

Linear and whorled nevoid hypermelanosis complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris

We report a 17-year-old girl who presented with linear and whorled melanosis following Blaschko lines mainly on her trunk. Ichthyosiform lesions and linear scaling erythemas were observed respectively on her lower limbs and the dorsa of her hands, left knee, ankle and foot. No abnormality was found in systemic examination and blood tests. A biopsy specimen of pigmentation of her back showed there was increased pigmentation within the basal keratinocytes, with focal incontinentia pigmenti. Notable lymphangiectasis could also be seen in the mid part of dermis, which had never previously been described. The biopsy of scaling erythemas of her hand showed dominant hyperkeratosis with focal parakeratosis, acanthosis and papillomatous hyperplasia in the epidermis. The diagnosis of linear and whorled nevoid hypermelanosis (LWNH) complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris was made in accordance with clinical and pathological manifestations. LWNH ought to be differentiated from incontinentia pigmenti and hypomelanosis of Ito.     

LIPH基因突变致常染色体隐性遗传羊毛状发1例

患者女,5岁,自幼头发生长缓慢,卷曲,稀疏。皮肤科情况:头发弥漫性稀疏、细软,致密卷曲缠绕。拉发试验阴性。眉毛及其他体毛正常。毛发镜检查示:头发细软,卷曲呈波浪状,散在黑点征。外显子组测序发现患者家系存在3号染色体LIPH基因突变,患者为c.742C>A (p.His248Asn)纯合突变,其父母为携带者。最终诊断为常染色体隐性遗传羊毛状发。     

Case of Rapidly Progressing Angiosarcoma after Total Hip Arthroplasty

The occurrence of malignant tumor in proximity to an arthroplasty prosthesis has been a matter of debate since it was first reported in 1978. Upon considering the number of orthopedic implants used, the occurrence of malignancy is rare. Especially in case of angiosarcoma, only a few cases have been reported worldwide. In this case, we report an extremely rare case of angiosarcoma arising at the site of a revision total hip arthroplasty. A 69-year-old female had received total hip replacement on her left hip due to osteoarthritis 8 months ago. Four months later, she complained pain on her operated area, X-ray showed loosening of implanted cup on her left hip. Thereafter, erythematous and purpuric papules and nodules were developed and spread around on her left hip. Through the skin biopsy she was diagnosed with angiosarcoma, and then she died of a sharp deterioration. Herein, we report a rare case of angiosarcoma occurred after total hip replacement with a review of the literature.