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。     

浆细胞性龟头炎一例并文献复习

本文报道1例浆细胞性龟头炎患者并回顾相关文献。患者,男,65岁。龟头红斑2年,皮疹表现为龟头两处境界清楚呈花瓣型鲜红色斑片,表面湿润,触诊无压痛,无明显溃疡,无明显浸润感。皮肤组织病理检示：表皮萎缩,部分真表皮分离,真皮浅层大量淋巴细胞、浆细胞浸润。诊断:浆细胞性龟头炎。治疗：外用1%吡美莫司乳膏,3个月后皮疹好转,现正随访中。     

持久性隆起性红斑伴再生障碍性贫血一例并文献复习

持久性隆起性红斑(erythema elevatum diutinum, EED)是一种罕见的皮肤白细胞破碎性血管炎,其病因不明确,认为与感染、血液系统疾病、自身免疫性疾病感染的循环免疫复合物在血管壁沉积有关,因此除单纯的皮肤表现外,既往报道39.4%的EED伴发HIV、病毒性肝炎、链球菌感染、副蛋白血症、骨髓增生异常综合征、系统性红斑狼疮和类风湿性关节炎等疾病。现报道我院诊治的持久性隆起性红斑伴再生障碍性贫血一例,并通过文献检索,分析伴发其他系统性疾病的EED的临床特征,初步探讨EED与血液系统疾病及恶性肿瘤的关联性。     

硬化性黏液水肿合并嗜酸性粒细胞增多症一例并文献复习

报告1例硬化性黏液水肿合并嗜酸性粒细胞增多症并对相关文献进行复习。患者，男，37岁。关节疼痛14个月，皮疹10个月。皮疹表现为全身泛发、粟粒大小、蜡样坚实丘疹。皮肤组织病理提示真皮成纤维细胞增生、黏蛋白沉积。实验室检查提示IgGλ型单克隆免疫球蛋白血症、外周血嗜酸粒细胞计数显著升高。经静脉输注人免疫球蛋白（IVIG）联合激素、沙利度胺等治疗，患者皮疹及关节症状显著缓解。     

胫前黏液性水肿一例

<正>临床资料患者,男,36岁。主因双下肢散在肿物1年余、加重半年,于2010年12月3日就诊。1年前患者双胫前出现散在黄豆大小斑丘疹,无自觉症状,未予特殊处理,近半年来皮损逐渐增大,数目增多,无疼痛、瘙痒。患者既往有甲亢病史3年,于当地治疗,现血清T3、T4水平均正常。体格检查:双侧凸眼征(+),甲状腺未触及,心、肺检查未见明显异常。皮肤科检查:双胫前散在边界清楚的圆形或卵圆形坚实红色结节或斑块,其中右胫前两处斑块较大,近端斑块约5cm×6 cm、远端约10 cm×8 cm,隆起、凹凸不平,表面有蜡样光泽,界限清楚,质硬,无压痛,皮肤张     

淋巴水肿性角皮症一例

患者女,47岁,因是部丘疹,双膝以下水肿3个月来我科就诊,就诊前3个月无明显诱因在右侧是背部,足趾背部出现散在的针尖大小的丘疹,1周后左脚也出现类似皮疹,后逐渐增多融合成斑块,高出皮面,表面呈乳头瘤样增生,随之发现双膝以下水肿,局部无自觉症状,未经任何治疗,既往有小腿丹毒的病史,近5年来每年发作1次,静脉滴注青霉素后可治愈,否认高血压,糖尿病,肝炎,结核等疾病史,否认疫水,疫区接触史。     

黏液水肿性苔藓一例

患者男,58岁,因四肢出现丘疹2年就诊。患者2年前无明显诱因双手背出现数个粟粒大的丘疹,无自觉症状,逐渐增多并发展到四肢。患者既往体健,家族中无类似患者。     

原发皮肤ALK阴性间变性大细胞淋巴瘤1例并文献复习

报告1例原发皮肤间变性大细胞淋巴瘤。患者男,42岁。左小腿红斑5年余,发现左小腿结节10月。皮损组织病理检查:真皮浅层及深层可见淋巴细胞团块状及片状浸润,细胞核大、异型,核分裂象多见。免疫组化示CD4、CD7、CD56、CD45RA、CD45RO、LCA、CD30、VIM阳性,CD8、CD20、Ki67、TDT、P53、ALK阴性,kappa、lamda散在个别(+),CD68散在(+)。诊断为原发皮肤ALK阴性间变性大细胞淋巴瘤。     

多重自身免疫综合征一例并文献复习

多重自身免疫综合征（multiple autoimmune syndrome, MAS）是指同一患者至少同时出现3种自身免疫性疾病,本文报道1例。患者,男, 67岁。患有IgA天疱疮、白癜风、斑秃、重症肌无力、胸腺瘤（已切除）、糖尿病,符合MAS诊断。给予长期口服泼尼松,IgA天疱疮病情得到控制。     

Case of remitting seronegative symmetrical synovitis with pitting edema (RS3PE syndrome) showing dermatomyositis-like eruption

A 73-year-old woman developed linear erythema at the sites of scratching-induced scars on the bilateral thighs 2 weeks before the initial consultation. Subsequently, edematous erythema developed in the upper eyelids, dorsum of the nose and the face, and pitting edema in the dorsum of the bilateral hands and feet. The C-reactive protein (CRP) level was 8.2 mg/dL and erythrocyte sedimentation rate (ESR) 121 mm/h. The antinuclear antibody titer was 1:160, and rheumatoid factor (RF) and anti-Jo-1 antibody were negative. X-ray examination of the bilateral hands showed neither narrowing of the joint spaces nor bone erosion. Ga scintigraphy showed synovitis of the bilateral wrists. A diagnosis of remitting seronegative symmetrical synovitis with pitting edema syndrome (RS3PE) was made. The erythema disappeared after diclofenac sodium administration. However, because the joint swelling and pitting edema did not improve, p.o. administration of prednisolone (20 mg/day) was initiated. The CRP and ESR levels normalized 2 months after the initiation of administration, and pitting edema disappeared after 3 months. We report this case because linear erythema like that observed in dermatomyositis has not been described as eruptions associated with RS3PE.     

Severe necrotic and haemorrhagic edema of the upper limbs revealing remitting seronegative symmetrical synovitis with pitting edema

BACKGROUND: RS3PE (Remitting Seronegative Symmetrical Synovitis with Pitting Edema) syndrome is characterized by bilateral and symmetrical tenosynovitis of the distal extremities. It occurs with acute onset in older patients aged over 50 years. This heterogeneous entity can be isolated or can reveal various rheumatic diseases and neoplastic conditions. We report a case of RS3PE syndrome associated with unusually severe cutaneous necrotic and haemorrhagic lesions, and revealing malignant monoclonal IgM proliferation. CASE REPORT: A 62-year-old man was admitted for acute and symmetrical synovitis of both hands and forearms associated with fever and increased acute phase reactants. Severe necrotic and haemorrhagic edema developed simultaneously. Laboratory tests ruled out infectious disease and collagen vascular disorder. Clinical symptoms responded promptly to corticosteroids to reveal severe erosive arthropathy of both wrists. The monoclonal IgM proliferation discovered during the acute phase evolved into a malignant medullary plasmocytosis. After adequate treatment of this proliferation, no haematological, cutaneous or articular relapse occurred during the two-year follow-up period. DISCUSSION: This type of cutaneous symptoms has never been reported during the course of RS3PE syndrome. It may be supposed that the severity of the initial clinical picture was linked in this patient to the paraneoplastic nature of this.     

Annular epidermolytic ichthyosis: a case report and literature review

Annular epidermolytic ichthyosis is a rare subtype of epidermolytic ichthyosis that is characterized by erythematous, polycyclic, and migratory scaly plaques accompanied by palmoplantar keratoderma. This report presents the case of an 8-year-old girl who developed migratory, erythematous, scaly plaques associated with palmoplantar keratoderma. The initial hypothesis was erythrokeratodermia variabilis et progressiva; however, the finding of epidermolytic hyperkeratosis in histopathological examination led to the diagnosis of annular epidermolytic ichthyosis.     

皮肤母细胞性NK细胞淋巴瘤一例

患者男,20岁,因右大腿起皮疹5个月,泛发全身2个月余就诊.体检:躯干、四肢散在分布的蚕豆至鸽蛋大小的暗红色结节,表面无破溃,质韧,无压痛.全身浅表淋巴结未扪及.背部皮损组织病理及免疫组化染色证实为母细胞性NK细胞淋巴瘤.经依托泊苷+环磷酰胺+长春新碱+地塞米松+甲氨蝶呤方案化疗后缓解.     

甲母质瘤一例并文献复习

报道一例甲母质瘤并对相关文献进行复习。患者，男，54岁。右手食指指甲增厚、变黄10年。组织病理检查：甲板显著增厚，甲母质细胞V形增生，底端呈条索状伸向真皮，上端呈乳头瘤样伸向甲板，上覆嗜酸性角质增生带，部分乳头瘤顶端见渗出结痂。真皮浅层梭形细胞增生，排列杂乱，间有散在肥大细胞，深部纤维及纤维细胞增生，排列与表皮平行。免疫组化染色：梭形细胞CD34阳性，S-100阴性，FⅩIIIa阴性，CD68阴性，CK7阴性。诊断：甲母质瘤。手术切除肿瘤后随访10个月无复发。     

对称性肢端角化病一例

患者,男,16岁。双手角化性斑片3年、复发2个月。本患者与既往报道的SAK病例不同,初期仅表现为手掌及指侧缘淡黄色角化性斑片,随着病情进展,皮损表现为黑褐色角化性斑片,皮损浸水后均发白、起皱,伴轻微紧绷感,离开水后恢复原状。治疗1个月后随访,皮损基本消退。