以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状的间变性大细胞淋巴瘤1例

患儿女,7岁,以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状,半年后左下肢出现皮下肿物确诊为间变性大细胞淋巴瘤。患儿首发临床表现为全身皮肤水疱、溃疡、结痂伴发热、腹股沟淋巴结肿大。皮损组织病理:表皮可见角化不良细胞,界面改变,表皮少量淋巴细胞浸润,未见异型细胞;淋巴结病理:淋巴结大片坏死,其间血管壁坏死,周围绕以多数淋巴样细胞;肿物病理:大量淋巴样细胞弥漫分布。免疫组织化学:CD30(+),ALK胞浆(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+)。诊断:间变性大细胞淋巴瘤。     

原发皮肤间变性大T细胞淋巴瘤1例

患者男,59岁,发现左大腿皮肤肿物7个月就诊,肿物活检送病理检查,镜下病理学表现为:瘤细胞呈圆形或类圆形,胞质丰富,胞核大小不一,核仁明显,核分裂象可见,免疫组化结果:CD3(+),CD30(弥漫+),LCA(+),Ki-67(约60%+),CD5(灶+),Bcl-2(+/-),CD45RO(+),Vimentin(+),CD56,CD20,CD23,CD4,CD8,CD79a,CD38,CD15,CD68,Cyclin D1,SMA,S-100,CK和TIA-1均(-),其中,LCA(+)提示淋巴造血系统肿瘤;CD20,CD23和CD79a(-)除外B细胞来源肿瘤;CD3和CD45RO(+)提示T细胞来源肿瘤;CD30(弥漫+)提示间变性大T细胞淋巴瘤。结合免疫组化结果,诊断为原发皮肤间变性大T细胞淋巴瘤。     

皮肤Merkel细胞癌伴鳞状细胞癌1例

患者女,76岁。左面颊部肿物1年,明显增大1个月。皮损特点为紫红色球形肿物。皮损组织病理示:表皮见团块状鳞状上皮中-重度异型增生,突破基底膜;真皮全层见形态一致的肿瘤细胞浸润,巢状或片状分布,圆形或卵圆形,胞质少、嗜碱性,核大、散点状分布染色质,核仁明显,可见多数核分裂相。免疫组化:CK20(部分+),P63及CKH(鳞状分化的细胞+),Syn(+),CgA(+),CD56(+),LCA(-),CK7(-),S100(-)。诊断:皮肤Merkel细胞癌伴鳞状细胞癌。     

原发性皮肤弥漫大B细胞淋巴瘤1例

患者男,58岁。右颈肩部肿物7个月,加重3周。皮肤科情况:右颈肩部见20cm×20cm大小肿块,质地中等度硬,未触及疼痛。皮损组织病理示:真皮中部至皮下脂肪组织内见密集淋巴样细胞浸润,细胞体积较大,胞浆丰富,核圆形、卵圆形或不规则形,核膜厚,染色质粗颗粒状,核仁明显,核分裂可见。免疫组化显示:大淋巴样细胞CD20弥漫(+),CD79a,BCL6,MUM1(+),CD3,CD4,CD8散在(+),CD56,CD30,CD10,BCL-2(-),Ki67(+)约80%。诊断原发性皮肤弥漫大B细胞淋巴瘤。经8个疗程CHOPE方案化疗及1个月放疗,皮肤肿块完全消退。随访5年未见复发。     

黑素瘤1例

患者女,51岁。右足底近跟部丘疹、溃疡、肿物2年。皮损组织病理示:表皮溃烂,真皮内弥漫分布核大、异型的黑素瘤细胞,瘤细胞内、间质均可见色素颗粒。免疫组化染色示:S-100(+),HMB-45(+),CK(+),Vim(-)。诊断:黑素瘤。     

种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

累及中枢神经系统的皮肤CD30~+间变性大细胞淋巴瘤1例

患者男,75岁,反复皮肤肿块11年。1个月前第3次复发伴语言、记忆和生活自理能力减退。头颅MRI示:左额叶,右顶叶多发占位,颅内淋巴瘤浸润。左大腿肿块组织病理示:真皮内大量异型细胞,体积较大,形态不规则,核扭曲,核分裂相可见;免疫组化:CD3(+),UCHL-1(+),CD30(+),ALK(-),MIB-1>90%。诊断:皮肤CD30+间变性大细胞淋巴瘤。中枢神经系统(central nerve system,CNS)累及可以是皮肤间变性大细胞淋巴瘤发生皮外累及的唯一部位,患者的意识改变是进行CNS筛查的重要提示。     

头皮局限性神经纤维瘤伴脱发一例

患者,男,22岁。脱发1年余,头皮棕褐色肿物6个月。病理可见毛囊微小化,真皮下部大量细长梭形瘤细胞,波浪状排列。S-100多克隆(+),SOX-10(+),MelanA(+),Vimentin-10(+)。诊断为头皮局限性神经纤维瘤。后行手术治疗,随访中。     

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。     

沿耳洞蔓延的基底细胞癌

报告1例基底细胞癌。患者女，58岁。耳后肿物30年，加重伴结节溃疡5个月。皮肤科检查：右侧耳后可见7 cm×2 cm暗红色斑片，边界较清，色素不均，部分破溃结痂，肿物沿耳洞蔓延至右耳垂前方，可见1 cm×1 cm紫红色结节，质硬，中央破溃，周边绕以珍珠状隆起的边缘。皮损组织病理检查：肿瘤细胞与表皮相连，真皮内可见由基底样细胞组成的嗜碱性的肿瘤团块，外周绕以肿瘤细胞，呈栅栏样排列，可见收缩间隙。免疫组化：p63(+),CK5/6(+),p53(40%～50%+)，增殖核抗原（Ki-67）(50%～60%+)。诊断：基底细胞癌。     

The nonrandom distribution of facial hemangiomas

OBJECTIVE: To map sites of occurrence of facial infantile hemangiomas and correlate these with pattern of tumor growth, clinical complications, and proximity to structural and developmental landmarks. DESIGN: A retrospective medical record review of 205 patients diagnosed with facial infantile hemangioma. SETTING: Arkansas Children's Hospital, Little Rock, a 250-bed teaching hospital affiliated with the University of Arkansas for Medical Sciences. PATIENTS: Based on their clinical photographs, 232 of the hemangiomas were mapped on a facial schematic. Each lesion was encoded with a number reflective of its location, and this number was shared by other lesions occurring at the same site. Frequencies of complicating ulceration and airway obstruction were determined by medical record review. RESULTS: Two patterns of tumor growth were evident among the hemangiomas analyzed: focal (177 lesions [76.3%]) and diffuse (55 lesions [23.7%]). The focal hemangiomas mapped to 22 sites of occurrence, all near lines of mesenchymal or mesenchymal-ectodermal embryonic fusion. The 55 diffuse hemangiomas showed a segmental tissue distribution and thus were designated as frontonasal (15 lesions [27%]), maxillary (19 lesions [35%]), or mandibular (21 lesions [38%]). Ulceration was 3 times more common in patients with diffuse hemangiomas (21 [51%] of 41) than in patients with focal hemangiomas (28 [17%] of 164). Airway obstruction was characteristic of diffuse mandibular hemangiomas. CONCLUSIONS: Facial infantile hemangiomas occurred in 2 distinct patterns of tissue involvement: a focal type with a tumorlike appearance and a less common diffuse type with a plaquelike appearance. The diffuse lesions were more likely to be complicated by ulceration or airway obstruction and showed a strikingly segmental distribution pattern. Focal hemangiomas, in contrast, showed a predilection for regions of embryological fusion.     

大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤

报告1例大疱性类天疱疮并发原发性皮肤弥漫性大B细胞淋巴瘤.患者男,80岁.全身水肿性红斑伴水疱、大疱,诊断为大疱性类天疱疮.在发病7个月后,右胫前出现多个暗紫红色结节,逐渐增多、增大,并扩展至右股内侧和背部.皮损组织病理检查示真皮全层及皮下脂肪小叶弥漫致密的淋巴细胞浸润,可见多数淋巴细胞体积增大,形态不规则,核大深染,可见核分裂相.免疫组化染色:CD20( ),bcl-2( ),bcl-6( ).诊断:原发性皮肤弥漫性大B细胞淋巴瘤.在肿瘤出现后1个月,患者大疱性类天疱疮的病情出现反复,通过CHOP方案治疗,病情明显好转.     

HAIR DIAMETER IN FEMALE BALDNESS

Summary.— Twenty scalp hairs were plucked from each of 131 Caucasoid women, and measurements of diameter, at 1 mm intervals, starting just above the root, were made for each hair. There was little variation in diameter along normal anagen hairs in any of the subjects. The mean anagen hair diameter in 58 women with diffuse hair thinning, of whom 44 were without detectable endocrine abnormality and 14 were clinically hypothyroid, significantly decreased with age. This appeared to be an accentuation of a normal trend, although this could not be statistically established in the control group of 73 women. At any age the mean thickness of hair in the subjects with diffuse thinning was less than in normal subjects of the same age. Normal subjects showed a symmetrical distribution of hair diameter, with a marked peak at 0.08 mm. In contrast, subjects with diffuse alopecia without detectable endocrine abnormality showed a wide spread of diameters, with 2 equal peaks at 0.04 mm and 0.06 mm The hypothyroid group showed a similar distribution, except that there was a larger proportion of hairs of the smaller diameters. The results suggest that, in the development of diffuse thinning, not all follicles behave identically, and that there may be more than one type of follicle in the human scalp.     

Papillon-Lefèvre综合征1例

患者男,12岁。双手、双足、双肘和双膝部皮肤弥漫性角化10年。皮肤科情况:双手、双足、双肘和双膝部皮肤红斑角化,伴有鳞屑。自幼牙龈红肿化脓,恒牙萌出时牙周组织出现破坏,牙龈红肿,牙排列不齐。左手背部皮损组织病理示:表皮银屑病样增生,真皮浅层水肿,少许慢性炎细胞浸润。诊断:Papil-lon-Lefèvre综合征。     

Brawny cutaneous induration and granulomatous panniculitis in large cell non-Hodgkin''s (T suppressor/cytotoxic cell) lymphoma

A 56-year-old lady presented with striking peau d'orange-like, woody, diffuse infiltration of the skin of the upper, inner aspects of both arms and thighs. Initial biopsy specimens showed a granulomatous panniculitis with non-specific lymphohistiocytic infiltration. Clinical deterioration occurred with development of ascites and peripheral oedema; a single axillary node became palpable. Cytology of ascitic fluid, and histology and immunopathology of the axillary node, and of a further skin biopsy, now revealed diagnostic changes of a diffuse large cell lymphocytic lymphoma of T suppressor/cytotoxic type. Granulomatous panniculitis resulting in diffuse cutaneous infiltration may rarely be a presenting sign of malignant lymphoma.     

Measurement of Skin Elastic Properties with a New Suction Device (II): Systemic Sclerosis

We previously reported the skin elastic properties of normal subjects as measured by a newly developed suction device (6). In the present report, we examined the skin elastic properties of 62 patients with systemic sclerosis (SSc), clinically diagnosed as limited or diffuse type. We measured the skin elastic properties on the forearm and chest and investigated correlations with disease type and clinical skin score. Patients with the diffuse type SSc showed significantly lower values than those with the limited type SSc or normal subjects in distention and retraction ability at the chest and forearm. This suction device is clinically useful for the objective evaluation of skin elastic properties in diffuse systemic sclerosis.     

Angiokeratoma of Fordyce as a cause of red scrotum

Two men presented with asymptomatic diffuse redness of the scrotum. Examination showed angiokeratoma of Fordyce (AF), an associated finding. In our practice, AF and diffuse scrotal redness co-occur in 50% of patients. Providing reassurance to the patient is appropriate if this clinical link is detected.     

泛发性脓疱型银屑病12例临床分析

对12例泛发性脓疱型银屑病作临床分析，发现其中多数患者因药物诱发，皮质类固醇激素、全反式维甲酸及免疫抑制剂联合治疗取得满意疗效。     

Systemic mastocytosis--case report and literature review

A 74-year-old Chinese male has had diffuse asymptomatic thickening, wrinkling, and mild hyperpigmentation of the skin with prominent skin markings and a leathery appearance over the neck, abdomen, flanks, and flexural area for forty years. Darter's sign was positive. Skin biopsy showed a dense band-like infiltration of mast cells into the upper dermis. Mast cell infiltration into the bone marrow and liver was also proved histologically. Electron microscopic studies of the skin and liver specimens were performed. Related aspects of diffuse and systemic mastocytosis are discussed. To our knowledge, this is the first reported Chinese case of diffuse and systemic mastocytosis in English literature. A review of the literature on systemic mastocytosis is also included.