泛发型皮肤肥大细胞增生病一例

肥大细胞增生病是一种少见的侵犯内皮网状系统的肥大细胞增生性疾病。临床上分为良性皮肤型肥大细胞增生病、系统性肥大细胞增生病、无皮疹或内脏型肥大细胞增生病及恶性肥大细胞增生病。任何一种类型的肥大细胞增生病都不多见。我们碰到一例良性皮肤型肥大细胞增生病中的成人泛发性肥大细胞增生病,现报道如下。患者男,２６岁。躯干、四肢泛发红斑、丘疹２６年。患者出生４个月始,于躯干部出现暗红色的斑疹、斑丘疹,略感瘙痒。此后皮疹逐年增多,４年左右斑疹、斑丘疹遍布躯干、四肢,伴瘙痒加重,皮疹颜色变暗,经多方治疗无效来本院就…     

儿童皮肤性肥大细胞增生病12例临床与组织病理分析

目的归纳总结儿童皮肤性肥大细胞增生病的临床及组织病理特点。方法回顾性分析2012年1月—2014年12月苏州大学附属儿童医院皮肤科诊治的12例儿童皮肤性肥大细胞增生病患儿的临床资料和组织病理学检查结果。结果皮损多表现为躯干、近端四肢圆形或类圆形的斑丘疹,部分可见单发结节,面部基本不受累,掌跖受累少见,Darier征部分阳性。组织病理学检查示:真皮浅层可见肥大细胞不同程度浸润,甲苯胺蓝染色阳性。均无皮肤以外的症状体征。结论根据典型的皮损特点及病理表现可诊断儿童皮肤性肥大细胞增生病。     

曲尼司特治疗泛发性肥大细胞增生病1例

报告曲尼司特治愈泛发性肥大细胞增生病1例,患儿男,6岁,面部,躯干,四肢广泛分布许多绿豆至黄豆大的斑丘疹和丘疹,部分为小结节或增生性瘢痕样皮损,明显高出皮面,呈圆形,椭圆形或不规则形,大多呈棕红色或棕褐色,组织病理检查示表皮角化过度伴角化不全,真皮浅,中层及附属器周围见致密的肥大细胞和少许嗜酸性粒细胞浸润,诊断为泛发性肥大细胞增生病,单用曲尼司特口服至第110天时,除留有少许点状色素沉着外,面部,躯干,四肢的皮损全部清退,服药期间未见不良反应,停药后随访6个月无复发。     

大疱性肥大细胞增生病1例

患儿男,4个月。头皮、躯干皮肤水疱、大疱3天。系统检查无异常。皮损组织病理:表皮大致正常,表皮下水疱形成。Giemsa染色示真皮内胞质有大量紫红色异染颗粒的肥大细胞。结合临床表现诊断:大疱性肥大细胞增生病。     

间擦部位肥大细胞增生病

报告1例间擦部位肥大细胞增生病.患者男,16岁.双腋下及腹股沟起多数灰褐色丘疹10年,缓慢发展,无自觉症状.皮肤科检查:双腋下及腹股沟散在较多粟粒大灰褐色扁平丘疹,Darier征阴性.皮损组织病理示真皮乳头部致密梭形肥大细胞浸润,并经甲苯胺蓝染色及免疫组织化学染色证实.诊断为间擦部位肥大细胞增生病.该病国内尚未见报道,国外至今报道5例.     

结节型皮肤肥大细胞增生症2例

报告2例结节型皮肤肥大细胞增生症患儿。2例患儿均为出生后不久发病,表现为躯干和四肢散发的结节性皮损,部分皮损表面可出现水疱。皮肤组织病理表现为真皮浅层或中层弥漫肥大细胞浸润,Giemsa染色可见肥大细胞内紫红色异染颗粒。结合病史、临床表现和组织病理学改变诊断为结节型皮肤肥大细胞增生症。     

阴茎尖锐湿疣治疗后发生红斑增生病1例及文献回顾

目的:报告1例阴茎尖锐湿疣治疗后发生红斑增生病,同时复习文献,回顾本病的病因、诊断及治疗。方法:报告该患者的病历特点。患者男,44岁,龟头、冠状沟起红斑伴疼痛3年余。组织病理示黏膜上皮增生肥厚及异形细胞增生。查阅既往关于红斑增生病的文献,总结本病的病因及诊疗特点。结果:根据皮损表现及组织病理学检查最后诊断为红斑增生病。行阴茎部分切除术治疗,目前在随访中。结论:红斑增生病属癌前病变,早期诊断和积极治疗具有重要的意义。     

肥大细胞和肥大细胞病

肥大细胞病(肥大细胞增生症)的特征是在不同组织中有肥大细胞增生和聚集,因释放的介质所产生的局部和系统作用不一而临床上有不同的症状和体征。皮肤肥大细胞增生症最常见,而系统性肥大细胞增生症则少见。从病史和皮肤活检即可诊断皮肤肥大细胞增生症。本病预后好,治疗上以对症处理为主,必要时可选用皮质类固醇,α-2b干扰素,手术切除等。     

肥大细胞和肥大细胞病

肥大细胞病（肥大细胞增生症）的特征是在不同组织中有肥大细胞增生和聚集，因释放的介质所产生的局部和系统作用不一而临床上的膛现的症状和体征，皮肤肥大细胞增生症最常见，而系统性肥大细胞增生症则少见，从病史和皮肤活检即可诊断皮肤肥大细胞增生症，本病预后好，治疗上以对症处理为主，必要时可选用皮质类固醇ａ－２ｂ干扰素，手术切除等。     

银屑病瘙痒皮损中肥大细胞的数量与分布研究

目的探讨肥大细胞在银屑病瘙痒症状发生中的作用。方法应用免疫组化技术对30例患者(无瘙痒12例,瘙痒18例)皮损区、非皮损区以及2例正常人皮肤组织中肥大细胞数量进行检测。结果银屑病伴有瘙痒症状的患者皮损中肥大细胞数量明显增多(Z=-2.732,P<0.05)。结论皮损中肥大细胞的数量变化与银屑病的瘙痒症状具有相关性。     

Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report

Severe generalized nodular and bullous mastocytosis of the xanthelasmoid type is described in a 7-month-old boy. Reddish to yellowish-brown xanthelasmoid papules and nodules first developed in the inguinal region a few weeks after birth and then progressively spread to cover nearly the entire body surface. There was severe pruritus and recurrent episodes of blistering. The diagnosis of cutaneous mastocytosis of the xanthelasmoid type with subepidermal bullae was confirmed by skin biopsies showing solid and deeply penetrating infiltrates of metachromatic mast cells under light and electron microscopy. Systemic involvement of other organs, however, was excluded by bone scintigraphy, abdominal ultrasound, bone marrow aspiration and echocardiography. The extensive skin involvement was reflected in highly elevated urinary levels of histamine (263.4 microg L(-1)) and its metabolite N-methylimidazole acetic acid (20.8 mg L(-1)). The patient was systematically well and received only symptomatic treatment. Over a period of 1 year, the condition gradually improved, and the skin lesions began to flatten and regress.     

Adult Onset of Xanthelasmoid Mastocytosis: Report of a Rare Entity

Xanthelasmoid or pseudoxanthomatous mastocytosis is an extremely rare variant of diffuse cutaneous mastocytosis. Herein, we describe an adult male with cutaneous mastocytosis showing multiple widespread yellowish ovoid papules like eruptive xanthoma. A 60-year-old male visited our outpatient clinic with a 1-year history of generalized yellowish, ovoid, and skin color papular eruption located on the trunk, groin, extremities, with the modest pruritus. Vital signs were stable, and Darier''s sign was negative. No other subjective and objective signs were detected during the examination. No abnormality was detected in his diagnostic laboratory tests. Skin biopsy was taken, and histopathologic examination revealed proliferation of mast cells with ovoid and spindle nuclei with distinct cytoplasm borders around the capillaries, which was compatible with mastocytosis. Antihistamine was prescribed for pruritus control which was successful, but eruptions were persistent, and even 1-year phototherapy was not useful.     

Erythrodermic mastocytosis.

Erythrodermic mastocytosis is a rare variant of diffuse cutaneous mastocytosis in which the skin becomes red, thickened, and lichenified and has a doughy consistency with multiple small papules on its surface, giving a leathery appearance to the skin. In this report, I describe a curious case of erythrodermic mastocytosis that appears to be due to vasodilation rather than to mast cell infiltration of the skin. In my opinion, this case might be an example of generalized telangiectasia macularis eruptiva perstans. Results of all laboratory tests failed to demonstrate systemic mast cell involvement; therapy with a combination of H1 and H2 antihistamines plus disodium cromoglycate controlled the symptoms.     

Cutaneous marginal zone B-cell lymphoma treated with rituximab

Marginal zone B-cell lymphoma (MZL) is probably the most frequent of the primary cutaneous B-cell lymphomas, which are entities with indolent behavior. Clinically, it appears in middle-aged patients as papules, nodules or erythematous plaques, solitary or multiple, on the trunk and proximal part of the limbs. The prognosis is excellent despite frequent cutaneous recurrences. We present the case of a 40-year-old male who, after having several recurrences of MZL over a ten-year period, was treated with rituximab for multiple skin lesions. The patient showed full remission after four weeks of treatment, and developed cytokine-release syndrome after the first infusion of the drug.     

儿童泛发性肥大细胞增生症1例

患儿女,11个月。全身反复皮疹伴瘙痒9个月。查体见全身多发淡褐色及淡黄色苔藓样黄豆至蚕豆大小的圆形及半圆形丘疹、结节,界清,表面明显苔藓化及色素沉着,Darier征阳性。皮损组织病理:表皮角化过度,真皮层内可见肥大细胞浸润,细胞呈梭形、卵圆形,胞浆呈淡伊红色。诊断:儿童泛发性肥大细胞增生症。     

Bullous Mastocytosis: Diffuse Cutaneous Mastocytosis with Extensive Blisters Mimicking Scalded Skin Syndrome or Erythema Multiforme

Bullous mastocytosis (diffuse cutaneous mastocytosis) is a rare form of mast cell disease that begins during the first month of life and causes extensive blisters that mimic scalded skin syndrome or bullous erythema multiforme. Discrete pigmented macules, papules, and nodules are absent and the characteristic leathery induration of skin may not develop until 6 months of age. Skin biopsy shows a subepidermal blister with mast cells at the base. The most serious complications are gastrointestinal hemorrhage and shock. The symptoms of bullous mastocytosis may be modified by a number of new therapeutic agents.     

Diagnosis of mastocytosis subsets using a morphometric point counting technique

Mastocytosis, a disease that varies in its clinical presentation, is usually documented by histologic examination of lesional skin. However, no universally accepted histopathologic criteria exist for establishing the diagnosis of this disease. We have combined the method of morphometric point counting with the mast cell-specific stain, conjugated avidin, to accurately quantify mast cells in cutaneous tissue sections of mastocytosis. Using this histologic approach, we found that macules, papules, and nodules of mast cell disease had from ninefold to nearly a 160-fold greater mast cell content than was observed in normal skin and in several other cutaneous disorders. This technique also permitted the objective histologic stratification of mastocytosis skin lesions according to their mast cell density. Morphometric point counting in conjunction with conjugated avidin offers a simple and accurate method for establishing the diagnosis of mastocytosis.     

Urticating Hashimoto-Pritzker Langerhans cell histiocytosis

Red-brown papules developing during the neonatal period may present a diagnostic dilemma. The Darier sign has been a reliable feature in the diagnosis of mastocytosis. However, the cutaneous infiltrate of Hashimoto-Pritzker Langerhans cell histiocytosis (LCH) may contain a large number of mast cells, leading to confusion both clinically and histologically. We report an infant who developed red-brown papules of Hashimoto-Pritzker LCH during the neonatal period and presented with a positive Darier sign and acute urticaria.     

Keratosis lichenoides chronica. Bath PUVA therapy

We describe the case of a 51-year-old male patient with characteristic lesions of keratosis lichenoides chronica confined to the back of his hands and feet. The lichenoid papules, linear hyperkeratotic ridges and erythematosquamous plaques appeared first in early childhood and recurred after a short episode of spontaneous remission. They didn't respond to various topical treatment modalities over the years. After a local PUVA therapy all lesions disappeared with no recurrence for over two years now. Our case report indicates a new promising indication for bath-PUVA-therapy.