头面部血管肉瘤1例

患者男,76岁。头面部红斑2月余。皮肤组织病理示:真皮内可见形状不规则裂隙状血管腔,部分血管内皮细胞有异型性。免疫组化:CD31(+)、CD34(+)、von Willebrand因子(+)、CollagenIV血管壁(+),Ki-67阳性率8%。临床及组织病理确诊为血管肉瘤。     

头面部恶性血管内皮细胞瘤1例

报告1例老年人头面部恶性血管内皮细胞瘤。患者女,63岁。头面部无症状性红斑、丘疹3个月,伴紫红色斑块、结节11d。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如芏鞋钉状,瘤细胞核深染,核分裂像偶见。诊断为恶性血管内皮细胞瘤。行右颈外动脉化疗加栓塞手术治疗1次后,皮损明显好转。     

9例老年人头面部血管肉瘤临床病理分析

目的：探讨老年人头面部血管肉瘤的临床和病理特征。方法：分析9例患者的临床特点，观察其常规组织病理变化及CD31、CD34、Ⅷ相关因子（FⅧ）免疫组化标记特点。结果：该病发病年龄平均71．4岁，男：女为2：1，临床早期常在头面部出现淤斑样皮损，后期发生浸润性暗红斑，伴结节及溃疡，易破溃出血。组织病理检查示真皮广泛血管增生及浸润，组织形态变异较大，常见细胞异形。CD31、CD34、FⅧ均可标记内皮细胞，在管腔明显区域表达强，实体瘤区域表达弱或不表达。结论：老年人头面部出现淤斑样皮损时，应及时做组织病理检查，早期明确诊断。CD31、CD34、FⅧ免疫组化标记有助于明确其来源。     

并发肺转移的头面部血管肉瘤

报告1例并发肺转移的头面部血管肉瘤.患者男,81岁.头面部出现多发性紫红色丘疹、结节和斑块10个月.皮损组织病理学检查:真皮梭形细胞增生,形成不规则管腔.免疫组化染色示CD31、Ⅷ因子、CD34阳性.患者近3个月出现胸闷,胸部X线及CT检查见肺内有多发性结节影.提示右肺内和左胸膜下肿瘤转移可能,最后诊断为头面部血管肉瘤并发肺转移.     

反应性血管内皮细胞增生症

报告1例反应性血管内皮细胞增生症.患者女,53岁.反复发热2年,面部出现多发性水肿性红斑2个月.超声心动图检查示主动脉瓣畸形伴赘生物形成.皮损组织病理检查示真皮乳头层和网状层有许多增生和扩张的毛细血管,内皮细胞显著增生,管腔闭塞,可见纤维蛋白性血栓.内皮细胞均无不典型性,无核分裂相.免疫组化染色结果示CD34和波形蛋白阳性,而血管内皮生长因子、CD31、FⅧ、CD45RO、CD20均阴性.     

恶性血管内皮细胞瘤1例

患者男性，66岁，头面部斑块，结节，溃疡伴疼痛8个月，查体头顶部至前额眶上有约8cm*12cm大小紫红色浸润性斑块，结节，中央多处溃疡，表面有结痂，边缘有类脓疱，血疱样结节，组织病理示广泛血管增生，多数为不规则血管腔，管腔内有增生的异常内皮细胞，诊断：恶性血管内皮细胞瘤。     

老年人头面部血管肉瘤1例

报告1例老年人头面部血管肉瘤。患者男,70岁。头顶部出现紫红色斑块,伴破溃4个月。检查见头顶及前额部可见 一紫黑色斑块,约2.0 cm×4.5 cm×0.5 cm大,表面有黄豆大的结节,溃破伴有黑色结痂。组织病理检查示,真皮及皮下组织内 多数不规则的血管腔、裂隙及增生的内皮细胞团块,细胞核大,有明显异形。免疫组化染色示FVⅢ(++),CD31(+++)。     

头面部血管肉瘤1例

血管肉瘤又称恶性血管内皮细胞瘤,是一种少见的来源于血管内皮细胞或淋巴管内皮细胞的恶性肿瘤。我科近来诊治1例老年人头面部血管肉瘤,现报告如下。     

血管淋巴样增生伴嗜酸性粒细胞增多症2例并文献复习

报告2例血管淋巴样增生伴嗜酸性粒细胞增多症。患者分别为28岁男性和26岁女性,病程分别为5年和1年,临床表现为主要分布在头面部的紫红色丘疹、结节。组织病理检查均有真皮层血管的增生,增生的血管内皮细胞呈特征性的上皮样,可内衬血管腔,突入血管腔内生长,血管内皮细胞胞浆丰富酸染,1例还可见特征性的空泡。血管周围大量淋巴细胞、嗜酸性粒细胞及组织细胞浸润。根据临床及皮肤组织病理,诊断为血管淋巴样增生伴嗜酸性粒细胞增多症。     

恶性血管内皮细胞瘤1例

恶性血管内皮细胞瘤 (ＭａｌｉｇｎａｎｔＡｎ ｇｉｏｅｎｄｏｔｈｅｌｉｏｍａ)又名血管肉瘤 (Ａｎｇｉｏｓａｒｃｏｍａ ,ＡＳ) ,是起源于内皮细胞或其前身细胞 ,为血管内皮或淋巴管内皮细胞的一种恶性肿瘤。本病在不同性别、年龄均可发生。可见于全身脏器内。发生于皮肤的通常见于 2 0岁以下的青年或较大的儿童 ,也见于 60岁以上的老年人[1] 。 3 0～5 0岁的头面部患者少见 ,国内尚未见报道。我们在 2 0 0 0年 6月收治了 1例头面部恶性血管内皮细胞瘤患者 ,现报告如下。临床资料　患者 ,女 ,42岁 ,农民 ,因头面部斑块、萎缩、坏死伴疼痛 …     

Mediation of alopecia areata by cooperation between CD4+ and CD8+ T lymphocytes: transfer to human scalp explants on Prkdc(scid) mice

OBJECTIVE: To determine the role of CD4+ and CD8+ T lymphocytes in the pathogenesis of alopecia areata. DESIGN: Relapse of alopecia areata was induced in autologous human scalp grafts on Prkdc(scid) mice by injection of activated T lymphocytes derived from lesional skin. CD4+ and CD8+ T cells were separated by magnetic beads before injection. SETTING: University-based dermatology practice. PARTICIPANTS: Eleven patients with either alopecia totalis or severe alopecia areata. MAIN OUTCOME MEASURES: Hair regrowth, hair loss, and immunohistochemical findings of scalp explants. INTERVENTION: Transfer of scalp T cells to autologous lesional scalp explants on Prkdc(scid) mice. RESULTS: Injection of unseparated T cells and mixed CD4+ plus CD8+ T cells resulted in significant hair loss (P<.01) in 5 of 5 experiments. However, injection of purified CD4+ or CD8+ T cells alone did not result in reproducible hair loss. CD4+ and CD8+ T cells induced follicular expression of intercellular adhesion molecule 1 (CD54), HLA-DR, and HLA-A, HLA-B, and HLA-C after injection into scalp grafts. CONCLUSIONS: CD4+ and CD8+ T cells have a role in the pathogenesis of alopecia areata. It is hypothesized that CD8+ T cells act as the effector cells, with CD4+ T cell help. It is now necessary to look for HLA-A, HLA-B, and HLA-C associations with alopecia areata. Therapeutic manipulations that interfere with CD8+ activity should be examined.     

CD44 expression in alopecia areata and androgenetic alopecia

CD44 is a widely distributed cell surface protein thought to be involved in multiple steps of normal immune cell function, including T-cell activation, and in cellular adhesion where it mediates cell attachment to hyaluronate. In normal skin, CD44 is found by immunohistochemical means to be primarily in eccrine coil cells. In this study, we have looked at the expression of CD44 in normal scalp and in two different hair disorders, androgenetic alopecia and alopecia areata. In normal scalp and androgenetic alopecia, CD44 was found in its normal distribution in eccrine coil cells. In scalp of 30 patients with alopecia areata, there was no expression of this glycoprotein. Patients were also assessed pre and post treatment for their alopecia areata, and even though they had no significant hair regrowth, 2 patients regained expression of CD44, indicating a variable expression of this protein in the alopecia areata disease process. The absence of CD44 expression in alopecia areata-affected scalp may give further information regarding the pathogenesis of this disease.     

Expression of vascular endothelial growth factor, apoptosis inhibitors (survivin and p16) and CCL27 in alopecia areata before and after diphencyprone treatment: an immunohistochemical study

BACKGROUND: Alopecia areata (AA) is a relatively common inflammatory form of nonscarring hair loss of unknown pathogenesis, but possibly of autoimmune origin. Topical immunotherapy, using a potent contact allergen such as diphencyprone (DPC), is currently considered the most effective mode of treatment. However, the way in which DPC operates on hair follicles in AA still remains to be elucidated. Vascular endothelial growth factor (VEGF), essential for angiogenesis and vascular permeability, may be responsible for maintaining proper vasculature around hair follicles, and several studies provide evidence that apoptosis is a central element in the regulation of hair follicle and vascular regression. The cutaneous lymphocyte-associated antigen (CLA) and the skin-associated chemokine CCL27 highlight an important role for epithelial cells in controlling homeostatic lymphocyte trafficking. OBJECTIVES: To determine the expression pattern of VEGF, factor (F)VIII, survivin, p16, CD4, CD8, CLA and CCL27 in alopecic skin before and after treatment with DPC. Methods Immunohistochemical staining methods were applied to skin biopsy specimens obtained from alopecic areas of 14 patients before and after DPC treatment and from five healthy subjects. Sections were incubated with monoclonal antibodies against VEGF, FVIII, survivin, p16, CCL27, CLA, CD4 and CD8, and their immunohistochemical expression was evaluated by light microscopy. RESULTS: The intensity of VEGF staining in alopecic human hair follicles was significantly lower than in healthy scalp tissue. FVIII immunostaining showed a significantly reduced development of the microvasculature in AA in comparison with healthy scalp tissue. After DPC therapy, cells of alopecic hair follicles showed a significant increase of VEGF immunopositivity, and the number of capillary vessels expressing FVIII was markedly increased in comparison with untreated scalp tissue. The increase in microvessels was associated with strong survivin expression in endothelial cells after treatment. All alopecic specimens showed expression of p16 in the hair follicle outer root sheath (ORS), with a significant increase after therapy. After treatment we observed a significantly decreased number of CD4+ cells and an increase of CD8+ cells (CD4/CD8 ratio 0.85) in alopecic skin compared with untreated scalp tissue (CD4/CD8 ratio 3.45). Most of the T lymphocytes found in inflammatory skin lesions expressed CLA antigen and after therapy we observed a significantly higher CLA positivity in hair follicles (50% or more) in comparison with untreated alopecic scalp tissue. Alopecic patients showed a CCL27 immunopositivity significantly lower than in normal scalp tissue. After DPC therapy the labelling intensity for CCL27 showed a significant increase both in the ORS and in the inner root sheath; similarly, in the basal interfollicular keratinocytes we observed a moderate increase in CCL27 expression. CONCLUSIONS: Topical immunotherapy exerts an important role in angiogenesis, upregulating VEGF in human hair follicle keratinocytes and upregulating survivin to preserve endothelial cell viability. Moreover, it considerably alters the peribulbar CD4/CD8 ratio, restoring a condition close to normal scalp skin. Our study could contribute to explaining some aspects of AA pathogenesis that are still unknown and aid understanding of how DPC could act in this complex disease.     

Perivascular localization of dermal stem cells in human scalp

In mammalian skin, the existence of stem cells in the dermis is still poorly understood. Previous studies have indicated that mesenchymal stem cells (MSCs) are situated as pericytes in various mammalian tissues. We speculated that the human adult dermis also contains MSC-like cells positive for CD34 at perivascular sites similar to adipose tissue. At first, stromal cells from adult scalp skin tissues showed colony-forming ability and differentiated into mesenchymal lineages (osteogenic, chondrogenic and adipogenic). Three-dimensional analysis of scalp skin with a confocal microscope clearly demonstrated that perivascular cells were positive for not only NG2, but also CD34, immunoreactivity. Perivascular CD34-positive cells were abundant around follicular portions. Furthermore, CD34-positive cell fractions collected with magnetic cell sorting were capable of differentiating into mesenchymal lineages. This study suggests that dermal perivascular sites act as a niche of MSCs in human scalp skin, which are easily accessible and useful in regenerative medicine.     

Biomarkers of alopecia areata disease activity and response to corticosteroid treatment

Alopecia areata (AA) is a common inflammatory disease targeting the anagen‐stage hair follicle. Different cytokines have been implicated in the disease profile, but their pathogenic role is not yet fully determined. We studied biopsies of pretreatment lesional and non‐lesional (NL) scalp and post‐treatment (intra‐lesional steroid injection) lesional scalp of 6 patchy patients with AA using immunohistochemistry and gene expression analysis. Immunohistochemistry showed increases in CD3⁺, CD8⁺ T cells, CD11c⁺ dendritic cells and CD1a⁺ Langerhans cells within and around hair follicles of pretreatment lesional scalp, which decreased upon treatment. qRT‐PCR showed in pretreatment lesional scalp (compared to NL) significant increases (P < 0.05) in expression of inflammatory markers (IL‐2, IL‐2RA, JAK3, IL‐15), Th1 (CXCL10 and CXCL9), Th2 (IL‐13, CCL17 and CCL18), IL‐12/IL‐23p40 and IL‐32. Among these, we observed significant downregulation with treatment in IL‐12/IL‐23p40, CCL18 and IL‐32. We also observed significant downregulation of several hair keratins in lesional scalp, with significant upregulation of KRT35, KRT75 and KRT86 in post‐treatment lesional scalp. This study shows concurrent activation of Th1 and Th2 immune axes as well as IL‐23 and IL‐32 cytokine pathways in lesional AA scalp and defined a series of response biomarkers to corticosteroid injection. Clinical trials with selective antagonists coupled with cytokine‐pathway biomarkers will be necessary to further dissect pathogenic immunity.     

Alopecia areata. Autoreactive T cells are variably enriched in scalp lesions relative to peripheral blood.

BACKGROUND AND DESIGN--Alopecia areata is a condition characterized by hair loss in association with perifollicular infiltration of T cells and antigen-presenting cells. Autoreactive T cells are postulated to amplify this abnormality by interacting with DR+ follicular epithelium. These cells may recognize either autologous major histocompatibility complex class II antigen or an autoantigen restricted by major histocompatibility complex class II. Limiting dilution analysis was used to determine the frequency of autoreactive lymphocytes in scalp biopsy specimens and peripheral blood from seven adult patients with alopecia areata. Autoreactive T cells are defined for this study as those that proliferate in response to autologous irradiated peripheral blood mononuclear cells. RESULTS--Autoreactive lymphocytes were enriched in scalp biopsy specimens relative to peripheral blood in five of seven patients. This enrichment was statistically significant in four of five patients. Five autoreactive T-cell clones derived from lesional scalp were characterized. Four of these clones were CD3+CD4+CD8- and one clone was CD3+CD4-CD8+. CONCLUSIONS--Enrichment of autoreactive cells in lesions of alopecia areata supports a role for these cells in the pathogenesis of this condition. Enrichment of autoreactive lymphocytes is also found in allergic contact dermatitis. Thus, these autoreactive lymphocytes may have a general role in inflammation.     

Primary cutaneous CD30 (Ki-1)-positive non-anaplastic B-cell lymphoma

BACKGROUND: The CD30 (Ki-1)-positive lymphoproliferative disorders show a non-epidermotropic infiltrate of large-sized pleomorphic T cells, being extremely rare those that develop from B cells. CLINICAL CASE: A 51-year-old white man presented with a 8x10 cm lobulated scalp tumor that had developed during the previous 8 months. Histopathology demonstrated a proliferation of medium and large-sized non-anaplasic lymphocytes. The tumor cells (CD45+) expressed B phenotype (CD20+, CD3) and were CD30 (Ki-1) positive. The extension study revealed no extracutaneous involvement and the lesion resolved following chemotherapy. Two years later, after six cycles of chemotherapy that followed the CHOP protocol the patient remained asymptomatic. CONCLUSIONS: We report a patient with a diffuse lymphoma of medium and large-sized B phenotype cells on the scalp that showed the unusual characteristic of being both non-anaplastic and CD30 (Ki-1) positive.     

Chronic lymphocytic leukaemia skin infiltrates affecting prominent parts of the face and the scalp

Chronic lymphocytic leukaemia (CLL) infiltrating the skin is uncommon and can present in different forms. We report a case of CLL infiltrating the prominent parts of the face and the scalp. A 63-year-old male with a 10-year history of CLL presented with plum-coloured swelling of the skin of the ears, eyebrows, tip of the nose and the scalp. Histopathology showed dense sheets of lymphoid infiltrate of the dermis which stained positive with B-cell markers CD20 and CD5 in keeping with the infiltrate of CLL.     

头面部血管肉瘤1例

患者女，67岁，头皮，前额起紫红色斑块半年，组织病理学检查符合血管肉瘤改变，免疫组织化染色结果示CD34，第Ⅷ因子（F8RA），波形蛋白（vimentin）等均阳性，证实为血管肉瘤。     

Alopecia areata is a T-lymphocyte mediated autoimmune disease: lesional human T-lymphocytes transfer alopecia areata to human skin grafts on SCID mice

Much evidence suggests that alopecia areata is a tissue restricted autoimmune disease. Alopecia areata responds to immunosuppressive agents, and is associated with other tissue restricted autoimmune diseases, including autoimmune thyroiditis and vitiligo. Furthermore, hair regrows when involved scalp is transplanted to nude mice. This study was undertaken to determine whether alopecia areata is mediated by T lymphocytes. Involved scalp from alopecia areata patients was grafted onto SCID mice. Additional biopsies from lesional scalp of the same patients were used to isolate T lymphocytes. These T lymphocytes were cultured with hair follicle homogenate, as well as autologous antigen presenting cells. The T lymphocytes were then injected into autologous scalp grafts on the SCID mice, which had regrown hair. Injection of scalp T lymphocytes resulted in hair loss. Hair loss was associated with the histologic and immunochemical changes of alopecia areata, including perifollicular infiltrates of T cells, along with HLA-DR and ICAM-1 expression by the follicular epithelium. Scalp T lymphocytes that had not been cultured with hair follicle homogenate did not have this effect. Preliminary data suggests hair loss requires a collaboration between CD8+ and CD4+T cells. These studies have demonstrated that alopecia areata can be induced by the transfer of T cells that recognize a hair follicle autoantigen.