各型荨麻疹在显微镜下的形态学

为确定各型荨麻疹可能具有的特定的组织病理表现,对108例各型荨麻疹患者的自发或诱发风团及正常对照组的针刺阳性的风团进行了组织学观察及常规免疫组化染色。结果:①在真皮浅层几乎均可见到水肿及淋巴管和毛细血管扩张,而深层少见。在各型荨麻疹中,寒冷性荨麻疹患者的真皮浅层水肿以及内皮细胞肿胀最为明显;②在所有标本中,整个真皮的肥大细胞数目均增加,但浅.层及病期超过10周者更为明显。在深层的肥大细胞常呈现脱颗粒。在     

荨麻疹中肥大细胞电镜观察及其临床意义的探讨

本文用透射电镜观察了44例荨麻疹的皮损和胃粘膜中肥大细胞的超微结构。肥大细胞一般呈卵圆形或不规则形,大小不等约为10～30μm。肥大细胞有伪足样胞质突起,有的突起可长达20μm。胞质内肥大细胞颗粒呈圆形或椭圆形,每个颗粒直径约0.2～0.4μm,另外胞质中可见大小不等的空泡。皮损和胃粘膜中的肥大细胞形态未见明显差异。对荨麻疹中的肥大细胞超微结构改变及其临床意义进行了初步探讨。     

荨麻疹的近代展望

发病机理荨麻疹的组织病理学为:水肿、皮肤微血管扩张和充血、淋巴管扩张及血管周围少数细胞浸润(可含有一些嗜酸粒细胞)。真皮上层受累最为明显,而血管神经性水肿则为真皮较深层之血管受累。皮肤划痕症之风团在电子显微镜下可见表皮及感觉神经末稍和肥大细胞的形态学的改变。 Lewis于一世纪前所观察到的荨麻疹三联反应(红斑、风团和红晕)迄今仍未有何改变,这些现象是由于毛细血管和小静脉扩张、受累血管渗透性增加及轴索反射引起周围小     

持久性发疹性斑状毛细血管扩张症伴色素性荨麻疹1例

目的:报告1例持久性发疹性斑状毛细血管扩张症伴色素性荨麻疹。患者男,48岁。颈、躯干及四肢出现丘疹、斑丘疹4年余。皮肤科检查:颈、前胸及四肢可见红褐色丘疹、斑丘疹,间杂散在毛细血管扩张;背部可见褐色丘疹,摩擦后出现风团(Diarer征阳性)。皮损组织病理:表皮大致正常,真皮浅层毛细血管扩张,有单一核细胞浸润;Giemsa染色:真皮浅层肥大细胞浸润。诊断为持久性发疹性斑状毛细血管扩张症伴色素性荨麻疹。     

匐行性回状红斑样荨麻疹性血管炎1例国内首报

患者男,57岁,全身出现环状红斑,伴瘙痒和疼痛5 d。皮肤科情况：面部、躯干及四肢可见水肿性同心圆形红斑,呈木纹状外观,边缘隆起,未见明显鳞屑,全身浅表淋巴结未触及肿大。皮损组织病理示：白细胞碎裂性血管炎,真皮浅层水肿明显,胶原纤维束间隙增宽;血管内皮肿胀,血管壁纤维蛋白样变性不明显;真皮浅层及血管周围可见以淋巴细胞为主的炎细胞浸润,少许红细胞外渗。根据患者临床表现和组织病理学检查,诊断为匐行性回状红斑样荨麻疹性血管炎。给予降低毛细血管通透性等治疗,皮疹基本消退,仅残留淡褐色色素沉着斑。患者出院4个月后随访皮疹未再复发。     

以泛发性丘疹为表现的成人肥大细胞增生病

报告成人肥大细胞增生病1例。患者男,74岁。全身泛发粟粒至绿豆大红色丘疹伴轻度瘙痒4年,皮损自行消退后形成褐色色素沉着斑点的同时不断出现新皮损,Darier征阴性,部分皮损呈紫癜样外观。组织病理学显示真皮浅层中等量肥大细胞弥漫浸润,伴部分细胞异形,诊断为肥大细胞增生病。由于其临床表现特殊,皮损与临床所见的皮肤肥大细胞增生病(包括色素性荨麻疹、弥漫型皮肤肥大细胞增生病、持久性发疹性斑状毛细血管扩张等)不同,导致长期不能确诊。该患者在随访过程中出现系统受累。     

持久性发疹性斑状毛细血管扩张症1例

患者男,40岁。面颈、双上肢、胸背部红色斑丘疹10+年。皮损组织病理示:表皮变薄,表皮突变平,基底层呈灶性及节段性液化变性,真皮浅层毛细血管扩张,其周围可见稀疏淋巴细胞及体积稍大、胞浆丰富红染的疑似肥大细胞浸润。甲苯胺蓝染色疑似肥大细胞胞质内紫红色颗粒。免疫组织化学标记:疑似肥大细胞CD117(+)。诊断:持久性发疹性斑状毛细血管扩张症。鉴于患者病变只累及皮肤,建议暂时不治疗,需长期随访。     

带状分布的色素性荨麻疹

报告1例带状分布的色素性荨麻疹.患者女,17岁.双侧腰腹部各见一条呈带状分布的色素沉着斑,用手摩擦后局部出现红色风团.皮损组织病理检查:表皮基底层黑素增加,甲苯胺蓝染色示真皮内毛囊及汗腺周围小片状肥大细胞浸润,胞质内含有嗜异染颗粒.     

荨麻疹的鸟瞰

荨麻疹为临床上常见病。是各种刺激所致的以风团为特征的皮肤反应。人们一生中发生过荨麻疹或昆克氏水肿者约为15—20％左右。荨麻疹可分为急性与慢性两大类。急性者、以青年男女多见,病因较容易发现,有异位性素质者发病率较高;而慢性者,以中年妇女为多,3/4的病例找不着病因。荨麻疹的病理学主要表现为真皮上中部分水肿,淋巴管与小血管扩张与充血,管周为嗜伊红细胞和其他炎性细胞所浸润。风团内的胶元束及纤维可被水肿所分离,如水肿扩展至皮下组织内,即表现为昆克氏水肿。     

新生儿弥漫性皮肤肥大细胞增生症1例

患儿男,10个月。全身皮疹伴瘙痒10月。检查见全身多发苔藓化丘疹,皮肤明显增厚,并见散在血疱及水疱。皮损组织病理示:真皮血管周围可见肥大细胞浸润,胞核圆形或卵圆形,Giemsa染色阳性。诊断:新生儿弥漫性皮肤肥大细胞增生症。     

Quantitation of tryptase- and chymase-containing mast cells in cutaneous lichen planus.

The distribution and density of tryptase- and chymase-positive mast cells in lesional and non-lesional cutaneous lichen planus (LP) was analysed. For this, enzyme-histochemical staining techniques and morphometrical measurements were applied. In non-lesional LP skin, chymase-positive cells (TC mast cells) showed a distribution similar to that found in both non-lesional psoriatic skin and in normal skin. Tryptase-positive cells (reflecting both T and TC mast cells), however, were increased in number in the upper dermis of non-lesional LP skin. In lesional LP skin, there were fewer chymase-positive cells in the upper dermis, whereas there were more tryptase-positive cells. In the upper dermis, no differences in the number of tryptase containing cells were detected between lesional and nonlesional LP skin. In lesions of LP and psoriasis, tryptase-positive mast cells are increased but differ in their distribution in the papillary dermis. In psoriatic lesions, tryptase-positive cells are frequently observed in epidermal contact, a feature very rarely seen in LP lesions. The present results suggest that the increased numbers of T mast cells in the upper dermis of nonlesional LP skin may be involved in initiating the LP lesion. It seems unlikely that mast cells could be responsible for the epidermal basal cell damage, though T mast cells do participate in the general inflammatory reaction.     

Electron microscopical study of psoriasis pustulosa

Summary Five cases of psoriasis pustulosa were examined by electron microscopy. The main features within the dermis were dilated capillaries filled with polymorphonuclear leukocytes and red cells. Neutrophils passed out of the vessels through the gaps and fenestrations and migrated towards the epidermis throughout the distinctly edematous corium. Lymphatic vessels could be found. Histiocytes containing Langerhans granules were observed in the dermis and epidermis. The morphological changes of the epidermis depented on the place examined and were most evident near fully developed pustules in the upper layers. The presence of previously described morphological findings could be confirmed. Perinuclear cytolysis and other changes in the keratinocytes, however, seem to be secondary, resulting from edema and accumulation of neutrophils within the epidermis. Immunological phenomenona may be responsible for the latter event.Supported by the Deutsche Forschungsgemeinschaft. The technical assistance of Mrs. I. Pfab is gratefully acknowledged     

Cutaneous mast cells are altered in normal healthy volunteers sitting in front of ordinary TVs/PCs – results from open-field provocation experiments

BACKGROUND: Considerable controversy has surrounded the question of possible biological responses to electromagnetic fields (EMFs) generated from visual display terminals (VDTs), such as personal computers (PCs) and ordinary television sets (TVs). The cellular and molecular mechanisms for such potential harmful health hazards have not yet been understood, although clues from the literature include mast cells and histamine. The aim of this study was therefore to investigate possible biological mast cell responses to TV/PC screens. METHODS: Using the indirect immunofluorescence technique, we studied the presence of histamine-containing mast cells in the dermis of healthy volunteers. Cutaneous biopsies taken before and after exposure to ordinary TV/PC screens for 2 or 4 h were investigated in 13 healthy subjects. RESULTS: Our present in vivo study indicates that normal cutaneous mast cells could be altered by exposure from ordinary TV/PC screens. To our great surprise, we found the number of mast cells in the papillary and reticular dermis to increase, to varying degrees, in 5 out the 13 subjects after such an exposure. A migration of mast cells towards the uppermost dermis appeared as the most important event. Thus, the normally upper "empty zone" of the dermis disappeared, and instead, a higher density of mast cells were found in this zone. These cells also seemed to have a tendency to increase in number towards the epidermal-dermal junctional zone and some of them lost their granular content and the cytoplasm shrunk (=degranulation). These findings could only be seen in the exposed skin. Two of the 13 cases instead showed a decrease in mast cell number, but the shift in mast cells towards the upper dermis was still visible. Twenty-four h after the provocation, the cellular number and location were normalized in all subjects. CONCLUSIONS: By definition, normal healthy volunteers are assumed not to react to a TV/PC screen provocation. To our great surprise, this proved not to be true. The present results might lay a foundation to understand the underlying cause of so-called "screen dermatitis" with special reference to mast cells. However, blind or double-blind experiments using patients ought to be further investigated in order to find out the exact cause for the observed changes. Such causes include the effects of surrounding airborne chemicals, stress factors, etc.     

Erythema gyratum atrophicans transiens neonatale.

An unknown dermatosis was observed in an infant girl. During the newborn period, she had a generalized, patchy, erythematous eruption of about 60 round erythematous patches on the trunk, thighs, head, labial mucosa, and palate. After a few weeks, the lesions became whitish, atrophic, and depressed, and they were surrounded by an erythematous and slightly infiltrated border, which was occasionally lobulated. Histologically, the lesions demonstrated a thin epidermis overlying an edematous dermis. Around the border of the lesions the collagen bundles were infiltrated with mononuclear cells. Granular deposits of IgG, C'3, and C'4 were found at the dermoepidermal junction and around the superficial capillaries. The lesions healed completely and spontaneously within a year.     

Poikiloderma-like lesions on the neck in atopic dermatitis: a histopathological study

Reticulate pigmentation with or without skin atrophy, depigmentation and telangiectasia is frequently encountered on the neck of severe cases of adult type atopic dermatitis. These skin changes were graded clinically into 3 stages. Based on histological features, hyperplasia of the sebaceous gland, dilated tortuous capillaries, and mild degeneration of elastic fibers were noted in stage I lesions. Lesions of both stages II and III contained increased melanin in the basal cell layer with incontinence of pigment, remarkable destruction and degeneration of elastic fibers, proliferated and dilated capillaries, and deposition of mucinous substances. The numbers of mast cells in papillary dermis were significantly increased in late stage I and stage II lesions. Poikiloderma-like lesions on the neck could be attributable to chronic inflammation and delay of wound healing process, possibly caused by long-standing topical corticosteroid therapy.     

Alterations in mast cells showing tryptase and chymase activity in epithelializating and chronic wounds

Mast cells can be found in contact with epidermis in certain circumstances; especially in chronic inflammatory skin diseases and chronic ulcers, but the significance of this association is obscure. In this study, the association of mast cells with wound healing was studied by counting mast cells in the wound edges at different stages after wounding the donor site skin for pinch-grafting. Chronic venous leg ulcers were biopsed for comparison. Tryptase- and chymase-positive mast cells were stained enzyme-histochemically for active proteinases. Both the number of tryptase-positive, i.e. total mast cells, and chymase-positive mast cells decreased during wound healing, but only the change in chymase-positive mast cells was statistically significant (P< or =0.03) the maximal decrease being 63% on day 7. No mast cells could be found in the vicinity of epithelialization margin. In venous leg ulcers, significantly more mast cells were present in the perilesional skin near the epithelium margin than in the wound bed (P=0.03), and mast cells were also seen in close contact with the basement membrane. Immunoreactivity for IL-4 and TNF-alpha in mast cells was studied to see if either of these molecules was associated with wound healing. In normally healing wounds, only a minority of mast cells were immunoreactive for these cytokines and no change in positive mast cell numbers could be seen during wound healing. In chronic wounds, IL-4 was absent in mast cells, and TNF-alpha positive mast cells were present only in perilesional skin and in small numbers. These results show that mast cells especially chymase-positive - decrease in number and can not be found in the epithelialization zone in normal wound healing, whereas tryptase-positive mast cells are associated with delayed wound healing and epithelialization in chronic wounds. Thus it seems, that mast cells attempt to control hyperproliferation of epidermis in chronic wounds.     

Psoriasis-like cutaneous inflammation in mice lacking interleukin-1 receptor antagonist

Interleukin-1 receptor antagonist-deficient (Il1rn-/-) BALB/c mice developed inflammation localized to the skin of the ear pinna in 64% of the cases examined. Histopathologically, the disease had many features resembling human psoriasis, suggesting that it might be a useful disease model. The epidermis became thickened and hypertrophic, and expressed the immature keratin, K6, throughout. The stratum corneum showed parakeratotsis. Large epidermal projections formed into a grossly thickened dermis and both tissues were infiltrated by leukocytes. Neutrophil-rich microabscesses formed beneath the stratum corneum. Dendritic cells and activated T cells of both helper classes were identified in both the dermis and epidermis, while a high density of macrophages was seen in the dermis, where mast cells were also prominent. Dense patterns of apparently activated small dermal vessels were seen in the diseased dermis. Cutaneous inflammation, along with arterial inflammation and arthritis, is the third site-specific, inflammatory disease to be found to affect Il1rn-/- BALB/c mice. None of the diseases affected Il1rn-/- C57BL/6. In F2 hybrids of Il1rn-/- BALB/c and C57BL/6, cutaneous inflammation was absent, aortic inflammation was common, and arthritis was rare, indicating that the sets of background modifier genes that cause susceptibility to each disease are not fully overlapping.     

Histamine-containing mast cells and their relationship to NGFr-immunoreactive nerves in prurigo nodularis: a reappraisal

The mast cell, which is a histamine-containing cell, has been found to have far more functions in skin inflammation than hitherto understood. To investigate the appearance of mast cells in prurigo nodularis, histamine immunohistochemistry in combination with nerve growth factor receptor (NGFr) double-staining as well as electron microscopic studies were performed. The results revealed that the histamine-containing cell number was increased in the lesional dermis. The mast cell size was also increased and the shape had become more dendritic. They tended to contact the epidermis and even infiltrated into it. In the histamine and NGFr double-staining, both an increased histamine-containing mast cell number and an increased number of NGFr-immuno-reactive nerve fiber profiles were revealed in the upper dermis of the prurigo nodularis lesional skin. Mast cells were seen in close vicinity to NGFr-positive nerves and sometimes even seemingly to contact single nerve fibers. At the ultrastructural level, it is obvious that the mast cell bodies become larger, having more abundant cytoplasm and organelles (e.g. mitochondria), but comparatively fewer characteristic granules. Mast cells were often observed to sprout long dendrites, with or without granules. The cells were also frequently seen to contact other cell types, and a mast cell infiltration into the epidermis was also found. The statistical results of mast cell numbers showed a significant increase in prurigo nodularis lesional skin compared to the normal controls. The present results further indicate that mast cells, together with cutaneous nerve fibers, are actively involved in the pathogenesis of the disease.     

Fucosidosis type 3 with angiokeratoma corporis diffusum

Disseminated angiokeratoma were seen in a 14-year-old Japanese boy with an inherited deficiency for alpha-L-fucosidase. He had gargoylian features, psychomotor retardation, small stature, vertebral changes, pale nail beds, recurrent infections and hypohidrosis in addition to angiokeratoma. In the areas of angiokeratoma, we found thin-walled dilated capillaries in the upper dermis. Several nondistended small blood vessels and eccrine sweat glands of secretory coils and ducts appeared narrowed by swollen epithelial cells with a finely vacuolated cytoplasm. In the pale nail bed, we found thickened dermis without an increased number of blood vessels. Electron microscopic studies showed endothelial cells of dermal capillaries and eccrine sweat gland epithelium were extensively enlarged: their cytoplasm was occupied by numerous electronlucent, membrane-limited vacuoles containing scant, finely granular or electronlucent material. In the myoepithelial cells of sweat gland and in the cytoplasm of Schwann cells, lamellar membrane-bound inclusions were seen.     

Quantitative enzyme-histochemical analysis of tryptase- and chymase-containing mast cells in psoriatic skin

Summary Tryptase-containing mast cells have recently been found to be increased in the upper dermis of psoriatic lesions. In the present study, the distribution of chymaseand tryptase-containing mast cells was morphometrically analysed at different dermal levels of lesional and non-lesional psoriatic skin (12 patients) as well as normal human skin. Mast cell tryptase was identified enzyme-histochemically, using Z-Gly-Pro-Arg-MNA as the substrate. For demonstrating mast cell chymase, a simple and specific enzyme-histochemical staining method was developed, using Suc-Val-Pro-Phe-MNA as the substrate. All mast cells positive for chymase were also positive for tryptase and Giemsa stain. Although the number of tryptase-positive mast cells was slightly increased throughout the dermis of lesional psoriatic skin, this increase was most pronounced in the upper dermis immediately beneath, and in close contact with, the epidermis. In contrast, the number of chymase-positive mast cells was clearly decreased in the upper dermis of psoriatic lesions, but not in the deeper dermis, as compared with non-lesional psoriatic skin. In addition, all chymase-positive mast cells observed in the upper dermis were very weakly stained when compared with those in the deeper dermis. No differences were found between non-lesional psoriatic skin and normal skin in which the number of mast cells containing chymase was 72–73% of the number containing tryptase. The present results suggest that T mast cells particularly, containing tryptase but no chymase, proliferate in psoriatic lesions, and that the increase in tryptase activity and the decrease in chymase activitiy in the upper dermis may lead to an imbalance in the biochemical regulatory systems.