萎缩型隆突性皮肤纤维肉瘤二例北大核心CSCD

报道2例萎缩型隆突性皮肤纤维肉瘤。患者分别为16岁和24岁的男性,临床最初均表现为界限清楚的萎缩型红斑,逐渐在红斑基础上发生结节。病程分别为6年和5年。皮损组织病理检查显示表皮萎缩或正常,肿瘤细胞在真皮浅层呈波浪状排列,平行于表皮;肿瘤细胞在真皮下部呈席纹状、车幅状、编织状排列,并可见肿瘤细胞侵犯皮下脂肪,分割脂肪细胞。肿瘤细胞表达波形蛋白、CD34,不表达CD68和S100蛋白。     

Pilomatricoma With Bullous Appearance: A Case Report

Introduction: Pilomatricoma is usually a solitary, deep dermal firm nodule with an overlying normal epidermis. Bullous pilomatricoma is rarely reported; thus, its pathogenesis has not been elucidated.Case presentation: A 19-year-old female presented a firm nodule covered by a bulla on her right upper arm. Histopathological examination showed the tumor was composed of basophilic basal-like cells and shadow cells. No cellular atypia or abnormal mitotic figures were observed. All these clinical cha...     

检测COL1A1-PDGFB融合基因协助诊断萎缩型隆突性皮肤纤维肉瘤

报告1例萎缩型隆突性皮肤纤维肉瘤。患者女,24岁。患者2年前无明显诱因胸部皮肤出现红斑,逐渐变大,中央萎缩。皮肤科检查:胸部一3 cm×4 cm暗紫红色近圆形斑块,表面光滑,中央萎缩,触之质地韧,无凸起包块,无触痛。皮损组织病理检查:表皮轻度乳头瘤样增生,真皮萎缩变薄,真皮及皮下脂肪可见短梭形细胞增生,增生细胞和表皮之间可见肿瘤细胞无浸润带;真皮浅、中层区域肿瘤细胞数目较少,排列稍稀疏,细胞呈波浪状排列,平行于表皮;真皮深部和皮下脂肪层可见密集排列的梭形细胞增生,梭形细胞密集排列呈车幅状、席纹状或束状。免疫组化示肿瘤细胞强阳性表达CD34。采用反转录-聚合酶链式反应(RT-PCR)检测COL1A1-PDGFB融合基因阳性。诊断:萎缩型隆突性皮肤纤维肉瘤。     

浅表肢端纤维黏液瘤一例

A case of superficial acral fibromyxoma is reported.A 43-year-old male patient presented with a 7-year-history progressive mass arising on the Ieft heel.There was no evident discomfort but occasional pain during walking.Clinical examination revealed a plum,solid,well-defined mass sized 1.5 cm×1.5 cm on the left heel.Histologically,there was a proliferation of numerous spindle or stellate cells arranged in a storiform or fascicular pattern embedded in a myxoid stroma with many small blood vessels;no evident atypia or mitosis was observed in these cells.Immunohistochemical staining of lesional tissue was positive for vimentin,fibronectin,CD34 and early membrane antigen,but negative for smooth muscle antibodv,S-100,neurofilaments,desmin or CD99.A diagnosis of superficial acral fibromyxoma Was made.The tumor was removed by expanded excision followed by free skin flap transplantation.No recurrence Was observed dunng a 5-mobth follow up.     

儿童获得性大疱性表皮松解症一例

报道1例儿童获得性大疱性表皮松解症.患者女,12岁,因全身反复水疱、大疱和糜烂面3个月入院.患者弱智,父母非近亲结婚,无家族史.入院时表现全身大片糜烂面,包括手足、膝关节周围和股臀部,同时躯干、四肢正常或水肿性红斑基础上紧张性水疱、大疱.治疗过程中突然发生躯干、四肢大量紧张性水疱.组织病理提示,表皮下水疱,免疫荧光结果见表皮基底膜带IgG和C3线状沉积,盐裂后沉积限于真皮侧.ELISA检测BP180和BP230阴性.采用大剂量糖皮质激素联合四环素口服取得满意疗效.

Abstract：

A case of epidermolysis bullosa acquisita (EBA) in childhood is reported. A 12-year-old girl was hospitalized for a 3-month history of recurrent blisters, bullae and erosions on the trunk and limbs. The girl had mental retardation but no family history of similar disorders. The marriage between her parents was not consanguineous. Physical examination on admission revealed large erosions with moderate oozing on the hands and feet, around the knees and on the buttock and thighs. There were scattered tense blisters and bullae arising in normal skin or edematous erythema on the trunk and limbs. During the treatment course the patient suddenly developed a number of tense blisters over the whole integument on the trunk and limbs. Skin biopsy showed subepidermal bullae with moderate perivascular infiltration of neutrophils and eosinophils. Direct immunofluorescence (DIF) revealed linear IgG and C3 deposition along the basal membrane zone, which was on the dermal side of salt-split skin on indirect immunofluorescence (IIF). ELISA detected no serum antiBP180 or -BP230 antibodies in the patient. A diagnosis of EBA was made. The patient was successfully controlled by intravenous steroids combined with oral tetracycline.     

非肿瘤期和肿瘤期蕈样肉芽肿皮损中CXCR3和CD1a的表达

目的 探讨不同分期蕈样肉芽肿皮损中表皮内CXC趋化因子受体3(CXCR3)和CD1a的表达情况.方法 免疫组化法分别检测16例正常人对照、16例非肿瘤期和8例肿瘤期蕈样肉芽肿患者皮损表皮内CXCR3和CD1a的表达情况.结果 当蕈样肉芽肿由斑片/斑块期进展至肿瘤期,表皮内淋巴细胞的CXCR3阳性率由38.9%降至17.5%,两者差异有统计学意义.CD1a在正常对照、非肿瘤期蕈样肉芽肿、肿瘤期蕈样肉芽肿表皮内每高倍视野分别含朗格汉斯细胞7.3、10.6、6.7个.非肿瘤期蕈样肉芽肿表皮内朗格汉斯细胞数量明显多于肿瘤期与正常人对照,肿瘤期与正常人对照差异无统计学意义.结论 蕈样肉芽肿中亲表皮现象与Th1相关趋化因子受体CXCR3及表皮内朗格汉斯细胞数量有关.

Abstract：

Objective To investigate the expressions of CXC chemokine receptor 3 (CXCR3) and CD1a in skin lesions of different stages of mycosis fungoides (MF). Methods The expression and distribution profiles of CD1a and CXCR3 were detected by immunohistochemistry in the epidermis of skin samples from 16 normal human controls, 16 patients with non-tumor (patch/plaque) stage MF and 8 patients with tumor stage MF. Results With the progression of MF from patch/plaque stage to tumor stage, the positivity rate of CXCR3 in intraepithelial neoplastic cells in lesions dropped from 38.9% to 17.5% (P ＜ 0.05). The average number of intraepithelial Langerhans cells (LCs) per high power field (HPF) in skin samples was 10.6 in patch/plaque stage MF, significantly higher than that in normal control (7.3) and tumor stage MF (6.7), while no significant difference was found between normal control and tumor stage MF specimens. Conclusion The epidermotropism in MF may be associated with the expression of T-helper (Th) 1-associated chemokine receptor (CXCR3) and quantity of intraepithelial LCs.     

儿童微静脉血管瘤一例

报告1例微静脉性血管瘤.患者女,8岁.因右食指褐黑色丘疹8年就诊.皮损组织病理检查:真皮中可见增生的薄壁小静脉,真皮胶原未见明显异常,胶原间隙可见散在血管内皮细胞,内皮细胞胞体饱满,无异形性.免疫组化染色显示:血管内皮细胞CD31、CD34及血管周围的细胞平滑肌动蛋白均阳性,而Ki-67阴性.符合微静脉性血管瘤的诊断.给予局麻下完全切除,目前随访无复发.该病主要需与早期的Kaposi肉瘤鉴别.

Abstract：

A case of microvenular hemangioma is reported.A 8-year-old girl presented with a 8-year history of a brown-black papule on the right forefinger.Histological examination showed numerous irregular,thin-walled,branching venules in the dermis with no abnormalities of collagen.In the space of collagen were plump endothelial cells without atypia.Immunohistochemical analysis of the vascular structures showed positive staining for CD31,CD34,smooth muscle actin (SMA),but negative staining for Ki-67.These findings are consistent with microvenular hemangioma,which should be mainly differentiated from Kaposi's sarcoma at early stage.The tumor was completely resected under local anesthesia,and no recurrence had been observed until the time of this writing.     

额部隆突性皮肤纤维肉瘤一例

A 56-year-old male was admitted to the hospital for a mass on the left forehead for more than one year. Twenty years prior to the presentation, the patient developed a broad bean-sized nodule on the left forehead, thereafter, an in situ resection was performed. One year prior to the presentation, the lesion recurred and gradually enlarged with no evident symptom. Physical examination showed a pink, painless and indurated mass measuring 3 cm × 2 cm × 1 cm on the left forehead. The lesion appeared as a hemispheroid-shaped elevation, had a smooth and tense surface, and was adherent to skin but not to deep fascia. There was an obvious depression around the mass. Histopathological examination revealed an evident proliferation of spindle cells with a braid-like distribution in the mid- and lower dermis. Mild atypia was observed in these cells.Immunohistochemistry revealed that the tumor cells were positive for CD34 and vimentin, but negative for CK, S-100 and epithelial membrane antigen. A diagnosis of dermatofibrosarcoma protuberans was made. The lesion was resected followed by a free full thickness skin graft. After operation, the flap survived well, and a satisfactory outcome was achieved.     

Malignant Cutaneous Adnexal Tumor in Posterior Occipital Region of a Child: A Case Report

Introduction: The most common cutaneous adnexal tumors in children were follicular, especially pilomatricoma, and a few were predominant glandular/ductal differentiation, malignant forms are occasionally encountered.Case presentation: A 10-year-old male child was presented with a nodular in posterior occipital for half a year with no symptoms. Histopathology showed there was acanthosis in epidermis, partially with local ulceration and crusting; and in the dermis, there was irregular scattered or...     

萎缩性隆突性皮肤纤维肉瘤5例临床及组织病理分析

目的:探讨萎缩性隆突性皮肤纤维肉瘤(DFSP)的临床及组织病理特征。方法:对西京皮肤医院确诊的5例萎缩性DFSP进行临床及组织病理学分析。结果:萎缩性DFSP主要表现为缓慢生长的界限清楚的萎缩性斑块,部分皮损可伴有皮下结节或外生性结节。临床上萎缩性DFSP容易误诊,其中4例误诊为硬斑病。萎缩性DFSP组织病理表现为真皮明显变薄,真皮内肿瘤细胞不形成典型的席纹状排列,而是平行于表皮,肿瘤细胞可以不浸润脂肪组织。免疫组化显示肿瘤细胞CD34阳性,CD68、S-100蛋白阴性。结论:相对于经典型DFSP而言,萎缩型DFSP的皮损不呈经典的结节性改变,而是表现为萎缩斑,容易误诊。组织病理表现为真皮萎缩,肿瘤细胞不呈席纹状排列,容易误诊为瘢痕或其他纤维细胞肿瘤。     

上皮样肉瘤1例

患者男,35岁。左上肢结节伴疼痛4个月。皮损组织病理示：表皮无明显变化,真皮内瘤细胞呈栅栏状排列,其中央可见坏死的黏蛋白沉积。瘤细胞呈梭形,少数有明显异型,未见上皮样细胞。免疫组化染色示：肿瘤细胞表达上皮膜抗原（EMA）、细胞角蛋白（CK）、细胞角蛋白18（CK18）、波形蛋白（Vimetin）和CD34,不表达CD68和结蛋白。     

弥漫性大B细胞淋巴瘤继发皮肤受累一例

患者,女,70岁。左胸部皮肤多发性暗红色结节2个月。皮损组织病理示表皮萎缩,基底细胞空泡化,真皮浅皮层可见弥漫性大淋巴细胞浸润,部分细胞成巢,伴不等量核尘。免疫组化：CD20(+),CD19(+),CD79a(+),MUM-1(+),CD5散在+,Bcl-2(+),BCL-6弱+,C-MYC+约10%,KI-67LI约80%。诊断为弥漫大B细胞淋巴瘤,IV期,NCCN-IPI 4分,继发皮肤受累。予以6个R-CHOP(R-美罗华,C-环磷酰胺,H-吡柔比星,O-长春新碱,P-泼尼松)方案化疗,病情好转。随访半年未见复发。     

隆突性皮肤纤维肉瘤一例

患者女,27岁。右侧背部出现红斑、结节5年。组织病理检查示:表皮轻度增厚,表皮突延长,基底层色素增多,真皮中下部及皮下组织大量梭形细胞团块,核大,深染,胞质丰富,核浆比例增大,呈漩涡状排列,侵袭性生长。免疫组化结果示:CD34(+)、Ki67 0~10%(+)。诊断:隆突性皮肤纤维肉瘤。     

Atrophic dermatofibrosarcoma protuberans with diffuse eosinophilic infiltrate

Atrophic dermatofibrosarcoma protuberans (atrophic DFSP) is a variant of dermatofibrosarcoma protuberans (DFSP), and is clinically characterized by depressed lesions. We report a patient with a typical atrophic DFSP lesion with marked eosinophilic infiltration. The patient was a 55-year-old woman with a dark-red, depressed lesion in the epigastric region. Histopathological examination of the lesion showed proliferation of fibroblast-like cells in a storiform pattern in the dermis and subcutaneous tissue. Immunohistochemical staining of tumor cells was positive for CD34. The lesion was histopathologically typical of DFSP, but no elevated lesion was clinically observed. Thus, a diagnosis of atrophic DFSP was made. Moreover, this tumor tissue exhibited marked eosinophilic infiltration. To our knowledge, they are no reports of eosinophilic infiltration in DFSP tissue. Therefore, this seems to be an extremely rare case of DFSP.     

未定类细胞组织细胞增生症一例并文献复习

报道1例未定类细胞组织细胞增生症并对既往文献进行复习。患者,女,75岁。全身丘疹、结节50天。组织病理检查:表皮萎缩变平,真皮中上层组织细胞、淋巴细胞为主间有少许泡沫细胞和多核巨细胞浸润。免疫组化:组织样细胞CD43、CD68阳性,大部分细胞CD1a阳性,部分细胞S100阳性,部分细胞CD163阳性,Ki67约30%阳性,Langerin阴性。诊断:未定类细胞组织细胞增生症。患者自行服用中草药治疗,具体不详,8个月后电话随访,皮损较前变小。     

Childhood onset dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, slow growing, locally aggressive dermal tumor of disputed histogenesis with a tendency for local recurrence. A 16-year-old female presented with hyperpigmented macules, papules, plaques, erythematous nodules and atrophic, indurated lesions ranging from a few millimeters to a few centimeters. Biopsy revealed thinned out epidermis and a diffuse dermal infiltration by fascicles of spindle cells arranged in storiform pattern. The pseudoseptae formed by the tumor cells interdigitating between the subcutaneous adipocytes resulted in a honeycomb pattern. Histochemical findings are helpful, but the chronicity, size, indolent nature, recurrence after surgery and histopathology are more important to arrive at a diagnosis. Wide and deep surgical excision is the treatment of choice.     

牛痘样水疱病样皮肤NK/T细胞淋巴瘤一例

患儿男，11岁，面部、手臂出现红斑、水疱4年，四肢肿块、溃疡4个月。逐渐感觉乏力，有不规则间歇发热。体检：体温37.7 ℃。全身淋巴结无明显肿大，口腔，颜面和鼻部皮肤片样轻度浸润的粉红色斑片，四肢见大小不等的暗红色浸润性结节和肿块，结节直径2.0 ~ 18 cm不等，部分表面有坏死和黑色结痂，较大的结节表面形成溃疡，坏死性溃疡深达肌肉并有白色脓性分泌物。皮损边界清楚，质地较硬，有压痛，部分结节周围见到卫星样小结节。取自大腿的皮损病理检查发现，表皮真皮全层至皮下可见大量形态不一、中等大小的肿瘤细胞浸润，肿瘤细胞胞质清晰，核大，扭曲状，染色质丰富，细胞呈巢或散在，可见围管样分布；真皮和皮下见碎核和反应性组织细胞浸润，肿瘤细胞浸入毛囊和外泌汗腺。免疫组化：胞质CD3在核周区可见阳性染色，CD56、CD45RO和TIA-1强阳性（+++），部分肿瘤细胞CD30、CD8和Ki 67阳性（++）。EB病毒编码小核RNA（EBER）阳性，TCR-γ 基因重排阳性。最后诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤，这是1例罕见的原发于皮肤长达51个月的进展缓慢的病例。     

恶性血管内皮瘤合并疱疹样天疱疮一例

报告1例恶性血管内皮瘤合并疱疹样天疱疮。患者男,71岁,因额面部结节破溃伴涨痛九月,全身红斑、水疱伴痒20天就诊。组织病理改变示肿瘤位于真皮浅中层, 瘤组织内有大量增生的肿瘤性血管, 管腔大小不一, 血管内皮细胞增生, 部分血管内皮瘤细胞异型性。瘤细胞核深染, 核分裂像偶见,免疫组化结果示CD34、CD31等阳性,诊断为恶性血管内皮细胞瘤。水疱病理示见表皮内水疱,疱内有嗜酸细胞、中性粒细胞浸润。直接免疫荧光(DIF)显示：IgG在表皮全层,C3于表皮中下层的细胞间阳性表达,诊断为疱疹样天疱疮。两病合并国内外未见类似报告。给予患者强的松30mg/d口服,皮损1周后消退,遂转入整形外科,切除额面部肿瘤,采用任意游离皮瓣转移至创面,术后3个月,未有新发皮疹。     

A case of primary cutaneous CD30+ T-cell lymphoproliferative disorder with features of granulomatous slack skin disease

We present a patient with primary CD30+ cutaneous T-cell lymphoma whose histological and clinical features overlapped with those of granulomatous slack skin disease (GSSD). A 26-year-old woman had infiltrative erythema on the abdominal wall and an incurable ulcerative lesion on the left knee. Her skin progressively became atrophic and pendulous, showing a hyperpigmented appearance over almost the whole body. Histopathologically, a dense lymphoid cell infiltrate accompanying numerous macrophages and multinucleated giant cells (MGC) extended into the subcutaneous tissue. Most lymphoid cells were small and positive for T-cell markers. Some relatively large atypical cells were scattered in the lesion, most of which (60%) were positive for CD30. T-cell receptor-beta gene rearrangement was confirmed in the abdominal lesion. MGC infiltrated more dominantly into a deeeper layer of the skin with the elastic fibres there almost completely disappearing. Immunoreactivity for CD30 of MGC was negative and overexpression of elastolytic metalloproteinases was observed. The association between primary cutaneous CD30+ lym- phoproliferative disorders and GSSD has not previously been reported. Overexpression of elastolytic metalloproteinases in MGC contributes to the disappearance of the elastic fibres and enhances the severity of the clinical course.     

以皮肤单核细胞肉瘤为首发表现的急性单核细胞白血病的特征研究

患儿4个月始发病,以额头、四肢皮肤出现多发皮色结节为特征,一般情况好,未触及肝、脾、淋巴结肿大、睾丸无明显异常、牙龈无异常增生。皮损组织病理：表皮大致正常,真皮及皮下弥漫浸润的异形细胞,体积中等偏大,胞质少,核椭圆形,不规则形,染色质细,个别细胞可见核沟及核仁。免疫组化：S-100蛋白阳性,CD68阳性;CD1阴性,Ki-67阳性率40%;CD21阴性,CD4部分细胞弱阳性。髓过氧化物酶（MPO） 阴性,CD56阳性,CD123阳性,CD163阳性。骨髓片见原、幼单核细胞占0.245。诊断：以皮肤单核细胞肉瘤为首发的急性单核细胞白血病。