同时具有特应性皮炎样及肥厚性扁平苔藓样皮损的慢性移植物抗宿主病

报告1例同时具有特应性皮炎(AD)样及肥厚性扁平苔藓样皮损的慢性移植物抗宿主病(cGVHD)。患者男,22岁。因全身扁平丘疹伴皮肤干燥、瘙痒4个月就诊。2014年4月患者因急性淋巴细胞白血病行父供子人类白细胞抗原(HLA)7/10相合异基因造血干细胞移植术。皮肤科检查:全身皮肤干燥,毛发稀疏,面部色素沉着,躯干及四肢皮肤毛周隆起,散在紫红色肥厚性丘疹,口腔黏膜损害,甲板纵嵴。皮损组织病理检查:(背部结节)表皮角化亢进,角质层可见少量炎性细胞浸润,颗粒层及棘层不规则增厚,部分区域表皮突杵状下延,部分区域基底层液化变性;真皮浅层带状淋巴细胞浸润,可见噬黑素细胞,真皮小血管增生,内皮细胞肿胀。直接免疫荧光(-)。诊断:同时具有AD样及肥厚性扁平苔藓样皮损的cGVHD。     

毛囊角化病1例

患者男,38岁。面部及四肢、躯干密集毛囊性丘疹20余年。皮损表现为密集针头及米粒大小褐色毛囊性丘疹,表面油腻性污痂,双侧腹股沟疣状增生。皮损组织病理示:角化过度伴灶状角化不全及角栓形成,棘层增生肥厚,呈疣状增生,表皮上部棘刺松解,可见圆体和谷粒。真皮浅层有少量淋巴细胞为主的炎症浸润。诊断:毛囊角化病。     

有表皮下水疱形成的急性泛发性扁平苔藓1例

患者男,49岁。全身起紫红色丘疹和丘疱疹3月余,伴瘙痒。皮肤科情况:躯干及四肢可见泛发性紫红色扁平丘疹及丘疱疹,粟粒至黄豆大小,以腰背部及双下肢受累为著,部分融合成斑块,上附少量白色鳞屑,不易刮除,可见Wickham纹。腰部皮损组织病理示:表皮角化过度,颗粒层局灶性增厚,棘层肥厚,基底细胞严重液化变性,表皮下水疱,真皮浅层单一核细胞带状浸润。诊断:急性泛发性扁平苔藓。     

慢性苔藓样角化病1例

患者男,31岁。舌部溃疡11年,四肢及腰腹部紫红色鳞屑性丘疹5年。皮损组织病理示:表皮角化过度,灶性角化不全,可见个别坏死角质形成细胞,少量淋巴细胞游入表皮,基底细胞液化变性,可见较多胶样小体,真皮浅层可见中等量淋巴细胞呈带状浸润。诊断:慢性苔藓样角化病。     

慢性家族性良性天疱疮误诊为慢性湿疹1例

患者女,62岁。躯干、四肢皮肤皱褶处红斑、丘疹、斑块伴瘙痒20余年。先后在多家医院均按慢性湿疹治疗,无明显疗效。皮损组织病理示:表皮灶状角化不全,棘层增生肥厚,可见灶状角化不良细胞和轻度的棘刺松解现象。真皮血管周围灶状炎症细胞浸润。诊断:慢性家族性良性天疱疮。     

面部痤疮样Darier病1例及家系调查

患者,女,22岁,未婚。面部丘疹、粉刺伴痒18年。有Darier病家族史。皮肤科检查:面额部、鼻部、鼻周、口周可见类似痤疮的针头大小肤色粉刺及米粒大小浅红色毛囊性坚实丘疹,表面油腻。大致对称分布。躯干、四肢、口腔黏膜及指趾甲未受累。病理检查:表皮角化过度,表皮上部见"圆体"和"谷粒";棘层肥厚,可见棘层松解,基底层上形成裂隙,真皮浅层淋巴细胞浸润。诊断:Darier病。     

胸腺瘤相关移植物抗宿主样病皮肤表现一例北大核心CSCD

患者男,37岁,因全身皮肤红斑丘疹痒1月余就诊,皮损持续不退.既往有胸腺瘤术后病史,病理分期为B3型4期.5个月前出现重症肌无力,患者有长期腹泻病史.体检：口腔黏膜可见白膜,面部及躯干四肢可见大小不等的鳞屑性红斑,刮之可见层层鳞屑,薄膜和血露现象阴性,手掌和足部可见鳞屑性红斑角化过度.组织病理：表皮银屑病样增生,角化不全,可见多个角化不良细胞,部分周围围绕淋巴细胞,血管周围少量淋巴细胞浸润.免疫组化：CD3阳性、CD4真皮阳性、CD8表皮阳性、CD20和CD68阴性、CD45RO阳性、CD30阴性.诊断：胸腺瘤相关移植物抗宿主样病,重症肌无力,黏膜念珠菌病.给予泼尼松及他克莫司治疗后病情逐渐缓解.     

播散型匐行性穿通性弹性纤维病1例

报告1例播散型匐行性穿通性弹性纤维病。患者男,17岁。全身出现成片丘疹伴轻度瘙痒8个月。皮肤科检查示面颈部、躯干、四肢泛发对称的米粒大红色角化性丘疹,排列成线状、环状、半环状或匐行状。组织病理检查显示：表皮角化过度,棘层肥厚,部分表皮穿通,形成管道,真皮上部弹性纤维增多呈嗜碱性变性,其周围可见混合性炎性细胞及嗜碱性碎片组成的异物肉芽肿。     

慢性苔藓样角化病

报告1例慢性苔藓样角化病。患者女,20岁。面部、躯干及四肢紫红色丘疹和斑块约20年。皮肤科检查:面部、躯干及四肢可见紫红色丘疹和斑块,皮损相互融合呈网状及线状,部分为毛囊性丘疹,皮损表面覆少量灰白色鳞屑,伴轻度瘙痒。Auspitz征阴性。未见口腔溃疡、眼损害及甲损害等。皮损组织病理检查:表皮角化过度伴角化不全,棘层不规则肥厚,基底层色素增加,灶状基底层变性,可见胶样小体;真皮浅层可见由淋巴细胞及组织细胞为主的带状浸润。诊断:慢性苔藓样角化病。予口服阿维A治疗6个月后皮损变平。     

扁平苔藓样慢性移植物抗宿主病1例

正器官移植可发生移植物抗宿主反应,常累及皮肤。慢性移植物抗宿主反应的皮损距离移植时间较长,容易造成漏诊或误诊。笔者报告1例扁平苔藓样表现的慢性移植物抗宿主病。1病历摘要患者女,54岁。因面部、躯干暗红色斑丘疹伴瘙痒1个月就诊。患者于2015年11月底出现面部暗红色丘疹及斑块,伴瘙痒,夜间尤剧。后皮疹逐渐增多,波及     

A case of acute cutaneous graft-versus-host disease mimicking psoriasis vulgaris

Graft-versus-host disease (GVHD) is a frequent complication occurring after allogenic hematopoietic stem cell transplantation and is divided into acute and chronic type. Cutaneous involvement is the most frequent manifestation of acute GVHD, with maculopapular exanthema and perifollicular papular lesions. We describe the first case to develop acute cutaneous GVHD mimicking psoriasis vulgaris shortly after allogenic peripheral blood stem cell transplantation. The patient's rash resembled psoriasis vulgaris and showed histologic features of both psoriasis and acute GVHD. Despite various immunosuppressant therapies, the skin lesion was drug-resistant. Therefore, we administered psoralen-UVA (PUVA) therapy and achieved the desired therapeutic effect. As far as we know, this is the first case of psoriasiform skin eruption as a manifestation of acute GVHD.     

具有白癜风样表现的慢性移植物抗宿主病1例

患者男,21岁。口腔溃疡、全身发疹伴眉毛、睫毛变白2周。6个月前患者因"急性淋巴细胞白血病"行异体造血干细胞移植。查体示:躯干部弥漫性分布暗红斑,伴脱屑和苔藓样改变,口腔、舌黏膜白斑、糜烂出血,双侧眉毛和眼睫毛部分呈白色。诊断:慢性移植物抗宿主病。系国内首例局限于眉毛和睫毛的具有白癜风样表现的慢性移植物抗宿主病。     

Pemphigus vulgaris with psoriasis vulgaris successfully treated with methotrexate and low‐dose methylprednisolone

Psoriasis and pemphigus are clinically well‐characterized chronic, inflammatory skin diseases. Many case reports have described the coexistence of psoriasis and bullous pemphigoid. However, the present report is about a rare case of pemphigus vulgaris in a patient with psoriasis vulgaris. We had a 68‐year‐old male psoriatic patient who developed blisters lesions and erosions on the trunk and extremities. The histopathology of a blister lesion showed the intraepidermal blisters that contained serous fluid and inflammatory cells. Both of desmoglein core protein 1 antibody and desmoglein core protein 3 antibody were detected. Diagnoses of pemphigus vulgaris and psoriasis vulgaris were made. The patient was treated with methotrexate (12.5 mg/week) and methylprednisone (16 mg/day) after his admission. Two weeks after admission, the patient's lesions gradually subsided. This case reminds us that the therapeutic effect of pemphigus vulgaris may be related to the incidence of psoriasis.     

Lichenoid sarcoidosis: a case with clinical and histopathological lichenoid features

Sarcoidosis is a chronic multisystemic granulomatous disease of unknown etiology, characterized by the formation of noncaseating granulomas in the involved organs. Cutaneous involvement is about 25% with different clinical expressions, the lichenoid pattern being one of the rarest types of cutaneous sarcoidosis. Lichenoid sarcoidosis clinically manifests with multiple scale papules involving extensive skin areas, especially the trunk, limbs, and face mimicking a lichen planus. Although diverse histologic patterns have been previously related, a lichenoid granulomatous infiltrate involving the dermo-epidermal junction has never been reported in lichenoid sarcoidosis. We report a case of a 43-year-old woman presenting with skin-colored pruritic papules, slightly scaling in trunk, extremities, and ears. These symptoms condition continued to expand and worsen for several years. The patient was otherwise in good health with no lymphadenopathies. Histopathologic examination of a skin biopsy showed an upper dermal granulomatous infiltrate of epithelioid cells, without necrosis, distributed in a lichenoid pattern with many cytoid bodies. We consider this may be the first case presenting a characteristic microscopic granulomatous lichen-like pattern in the setting of a clinically lichenoid type of sarcoidosis.     

小儿寻常型银屑病伴发皮肌炎1例

患儿男，9岁，头皮、凹肢、躯干部鳞屑性斑块4年。病理报告符合寻常型银屑病，面部水肿性红斑伴肌痛、肌无力2年。肌酶指标升高，肌电图示广泛肌源性损害神经电图示运动神经传导速度在正常范围内。诊断为寻常型银屑病伴发皮肌炎。     

Acute follicular graft-vs-host reaction. A distinct clinicopathologic presentation

Human graft-vs-host disease (GVHD) is a life-threatening complication that may occur following allogeneic bone marrow transplantation. In acute GVHD, skin involvement is frequent, and the skin is often the initial organ involved. The rash typically is a blanchable, erythematous macular eruption. We present the first report of follicular cutaneous GVHD. Three patients developed follicular papules simulating bacterial or fungal folliculitis as a major clinical expression of cutaneous involvement in acute GVHD following allogeneic bone marrow transplantation. In each case, histopathologic examination demonstrated features of acute graft-vs-host reaction involving hair follicles. This suggests that follicular epithelium may be an early target in acute GVHD.     

Chronic Graft-versus-Host Disease with Follicular Involvement

The clinical and histopathological findings in a 25-year-old Japanese male patient who suffered from chronic graft-versus-host-disease (GVHD) with follicular involvement are described. The patient had been diagnosed as aplastic anemia and underwent an allogeneic bone marrow transplant (BMT). In the eighth month thereafter, pruritic follicular red papules developed over his trunk and extremities. A biopsy specimen revealed histological exocytosis of lymphocytes into the hair follicles and basal-cell vacuolization of the follicular epithelium. Analysis of T-lymphocyte subpopulations in the dermis revealed a predominance of CD4 positive cells. To our knowledge, several cases of acute follicular GVHD have been reported (1, 2); however, the occurrence of chronic GVHD with follicular involvement (chronic follicular GVHD) has not been clearly documented.     

银屑病合并急性髓细胞白血病一例的治疗观察

目的 报告继发急性髓细胞白血病(M4EO型)的慢性斑块状银屑病1例临床研究及骨髓移植治疗.方法采用临床资料收集,组织病理检查,骨髓和外周血涂片检查,利用流式细胞仪进行细胞免疫分型,用骨髓细胞体外培养做染色体检查及G显带分析.结果患者女,33岁,有寻常性银屑病史20余年,反复出现红斑鳞屑,皮损以斑块为主,有家族史,用多种方法治疗(以中药为主).近来不明原因肌肉酸痛,牙龈出血,发热伴胸骨叩痛.骨髓检查发现异常幼稚单核细胞及早幼粒细胞,并见含有粗大嗜碱颗粒的嗜酸粒细胞,确诊为急性髓细胞白血病M4EO型,经骨髓细胞免疫分型检查符合诊断.骨髓细胞体外培养做染色体检查,+G-显带发现inv(16)的克隆异常和+8克隆异常,染色体核型为46,XX,inv(16)/47,XX,inv(16),+8(2/22).经异基因骨髓移植治疗,银屑病皮损完全消退,急性髓性白血病症状缓解,骨髓异常幼稚细胞减少,病情得到有效控制.结论为国内首例寻常性银屑病(斑块状)继发急性髓细胞白血病M4EO型的临床研究及骨髓移植治疗     

Immunohistochemistry of cutaneous graft-versus-host disease after allogeneic bone marrow transplantation

Graft-versus-host disease (GVHD) is an immunologically mediated disease occurring most frequently after allogeneic bone marrow transplantation. The aim of this study was to evaluate the contribution of immunohistochemistry in the diagnosis of cutaneous GVHD. Patients transplanted for either leukemia or beta-thalassemia were included in the study. Skin lesions of acute and chronic GVHD were examined both by direct immunofluorescence to detect immunoglobulin deposits and by an avidin-biotin-peroxidase complex technique to evaluate the inflammatory cell infiltrate. Epidermal and dermal fluorescent bodies (IgG and IgM) were frequently found in both acute and chronic GVHD. Most of the infiltrating cells were CD3+ T lymphocytes, with CD8+ cells representing the major cell population invading the epidermis both in acute GVHD and in chronic lichenoid GVHD. A small proportion of the dermal cells were CD14+ macrophages; no B cells were detected. HLA-DR, but not HLA-DQ antigens, were variably expressed by keratinocytes in all cases of acute GVHD and in chronic lichenoid GVHD. KL-1, a monoclonal antikeratin antibody specific for the 56.5 KD acidic polypeptide usually present in suprabasal keratinocytes, stained all epidermal layers, including the basal layer. Langerhans cells were dramatically reduced in number in the epidermis of both acute and chronic lichenoid GVHD. It is concluded that immunohistologic analysis may be supportive in the diagnosis of acute and early chronic lichenoid cutaneous GVHD.     

Risk factors and clinical characteristics of acute and chronic cutaneous graft-versus-host disease in pediatric patients undergoing hematopoietic stem cell transplantation

Acute and chronic cutaneous graft-versus-host disease (GVHD) are common complications following hematopoietic stem cell transplantation (HSCT) in pediatric patients. In this retrospective study, we explored the risk factors and clinical characteristics of acute and chronic cutaneous GVHD in a case series of children undergoing HSCT at a tertiary referral hospital. We found that 36% of acute cutaneous GVHD was severe and these patients were more likely to have an unrelated donor, and that children with acute cutaneous GVHD who progressed to chronic cutaneous GVHD had a higher proportion of malignant diseases, total body irradiation, and bronchiolitis obliterans compared to those who did not progress to chronic cutaneous GVHD. Our study highlights the importance of identifying and monitoring these high-risk patients to improve the clinical management and outcomes of cutaneous GVHD in pediatric HSCT recipients.