嗜酸性粒细胞增多综合征并发肺栓塞

报告1例嗜酸性粒细胞增多综合征并发肺栓塞。患者男,35岁。以四肢反复皮疹伴瘙痒为主要表现,多次检查外周血嗜酸性粒细胞计数≥1.5×10~9/L,皮损组织病理及骨髓穿刺结果均显示嗜酸性粒细胞增多,诊断为嗜酸性粒细胞增多综合征。患者病程中在无常见高危因素情况下并发肺栓塞,考虑肺栓塞是患者嗜酸性粒细胞增多综合征的并发症之一。     

嗜酸性蜂窝织炎

报告1例嗜酸性蜂窝织炎(Wells综合征).患者女,48岁.躯干反复出现水肿性红斑、斑块伴瘙痒6个月余.组织病理显示:真皮内大量嗜酸性粒细胞浸润,并町见胶原变性,形成火焰现象.诊断为嗜酸性蜂窝织炎.     

嗜酸性粒细胞增多综合征1例

患者女,53岁。反复咳嗽、咳痰1年,全身起疹伴瘙痒2个月入院。入院检查外周血和骨髓嗜酸性粒细胞明显增多,组织病理改变符合嗜酸性粒细胞改变。诊断:嗜酸性粒细胞增多综合征。给予糖皮质激素、抗感染和支持对症等治疗14d后出院。现随访中。     

嗜酸性粒细胞增多性蜂窝织炎

嗜酸性粒细胞增多性蜂窝组织炎,或嗜酸性粒细胞增多性复发性肉芽肿皮炎,又称wells综合征,是一种罕见的以反复出现皮肤肉芽肿和外周血嗜酸性粒细胞增多为特征的皮肤病,国外1971年由wells最先报道而得名[1],国内类似病例较少.现报告1例临床及实验室资料完整的成人患者.     

嗜酸性蜂窝织炎1例

嗜酸性蜂窝织炎(eosinophilic cellulitis, EC)又称Wells综合征(Wells syndrome),为一种复发性肉芽肿性皮炎,伴有嗜酸性粒细胞浸润.本病临床较少见,现将笔者诊治的1例报告如下.     

嗜酸性粒细胞增多综合征1例并文献复习

正嗜酸性粒细胞增多综合征(Hypereosinophilic Syndrome,HES)是以血及骨髓嗜酸性粒细胞增多,组织中嗜酸性粒细胞浸润为特征的一类异质性疾病谱[1]。多累及皮肤、心血管、呼吸、消化及血液系统,并出现相关症状及体征。嗜酸性粒细胞增多性皮炎和慢性嗜酸性粒细胞性白血病分别为该疾病谱的良性端及恶性端,嗜酸性粒细胞增多性皮炎(Hypereosinophilic Dermatitis,HED),是一种仅侵犯皮肤而无系统性损害的亚型,皮疹多型、泛发伴剧烈瘙痒,预后尚可,该定义由Nir及Westfried     

抗心磷脂抗体阳性的播散性嗜酸性细胞胶原病

播散性嗜酸性细胞胶原病（disseminated eosinophilic collagen disease）是一种少见的全身性疾病，属于嗜酸性细胞增多综合征的一种类型，国内少见报道。我科最近收治1例，现报告如下。     

嗜酸性粒细胞增多综合征1例

报告1例嗜酸性粒细胞增多综合征。患者男，57岁。四肢反复出现丘疹、血疱伴间断发热半年入院。既往有慢性结肠炎病史20年。入院检查外周血和骨髓嗜酸性粒细胞明显增多，淋巴结组织病理改变符合嗜酸性粒细胞白血病淋巴结改变给予糖皮质激素、抗感染和支持对症等治疗18d后出院。出院后1个月死于心力衰竭。     

嗜酸性粒细胞增多性皮炎的治疗进展

嗜酸性粒细胞增多综合征（hypereosinophilic syndrome,HES）是一组病因不明,以血液和/或骨髓嗜酸性粒细胞（eosinophil cell,EC）持续增多,组织中大量EC浸润为特征的疾病.而嗜酸性粒细胞增多性皮炎（HED）是嗜酸性粒细胞增多综合征（HES）的轻型或此疾病谱的良性端.它是嗜酸性粒细胞增多引起的皮肤病.近年来,临床上报道的HED病例越来越多.对其病因和发病机制的研究也已经取得很大进展,治疗上也取得一些的进展.     

新生儿Omenn综合征

报告1例新生儿Omenn综合征。患儿女,16 d。全身潮红伴脱屑15 d。体格检查:全身皮肤浸润、潮红。腹部膨隆,肝脾淋巴结肿大。实验室检查:外周血白细胞升高,嗜酸性粒细胞明显增多;B淋巴细胞缺如;血清IgG、IgA、IgM极低,IgE显著升高。骨髓检查:嗜酸性粒细胞比例明显升高。诊断:Omenn综合征。     

Bloom Syndrome in Two Siblings

Abstract: Bloom syndrome (congenital telangiectatic erythema) is a rare autosomal recessive disorder characterized by telangiectasias and photosensitivity, growth deficiency of prenatal onset, variable degrees of immunodeficiency, and increased susceptibility to neoplasms of many sites and types. We are reporting Bloom syndrome in two brothers from Kashmir (India), 8 and 6 years of age, who presented with erythematous rashes on the face, photosensitivity, and growth retardation.     

伴甲状腺损害的药物超敏综合征l例

患者女,19岁,使用可疑药物后出现发热,全身散在米粒大红色丘疹伴瘙痒．逐渐进展为红皮病。外周血嗜酸粒细胞增多,并发现异型淋巴细胞、甲状腺损害及肝功能受损。     

Collagenolytic (necrobiotic) granulomas: part 1--the "blue" granulomas

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. "blue" granulomas vs. "red" granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In this first part, the clinical presentation, pathogenesis, and histologic features of the "blue" collagenolytic granulomas are discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In the subsequent half of this two-part series, the "red" collagenolytic granulomas will be discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's syndrome).     

Toxic Epidermal Necrolysis Due to Concomitant Use of Lamotrigine and Valproic Acid

Anti-epileptic drugs can be associated with a wide spectrum of cutaneous adverse reactions ranging from simple maculopapular rashes to more severe and life threatening reactions like Stevens-Johnson syndrome and toxic epidermal necrolysis. These rashes are well documented with older antiepileptic drugs like phenytoin, phenobarbitone and carbamazapine. Lamotrigine is a newer, unrelated antiepileptic drug that causes skin rashes in 3-10% of new users. Higher starting dose or rapid escalation, concurrent treatment with valproic acid, and a previous history of a rash with other antiepileptic drugs are well recognized risk factors for lamotrigine related serious rashes. We report two patients with toxic epidermal necrolysis, resulting from concomitant use of lamotrigine and valproic acid. It is emphasized that clinicians adhere to the recommended dosage guidelines and adopt a slow dose titration when initiating treatment with lamotrigine.     

界限类偏结核样型麻风病1例

患者男,38岁。右手腕皮疹伴麻木1年,加重1周。皮肤科检查见右手腕屈侧黄红色环形斑块。组织病理示真皮全层可见沿血管、神经和汗腺分布的类椭圆形结核样肉芽肿,以真皮下部为著。予氨苯砜和利福平联合化疗,现随访中。     

Collagenolytic (necrobiotic) granulomas: part II--the 'red' granulomas

A collagenolytic or necrobiotic non-infectious granuloma is one in which a granulomatous infiltrate develops around a central area of altered collagen and elastic fibers. The altered fibers lose their distinct boundaries and exhibit new staining patterns, becoming either more basophilic or eosinophilic. Within the area of altered collagen, there may be deposition of acellular substances such as mucin (blue) or fibrin (red), or there may be neutrophils with nuclear dust (blue), eosinophils (red), or flame figures (red). These color distinctions can be used as a simple algorithm for the diagnosis of collagenolytic granulomas, i.e. 'blue' granulomas vs. 'red' granulomas. Eight diagnoses are included within these two groupings, which are discussed in this two-part article. In the previously published first part, the clinical presentation, pathogenesis and histologic features of the 'blue' collagenolytic granulomas were discussed. These are the lesions of granuloma annulare, Wegener's granulomatosis, and rheumatoid vasculitis. In this second half of the series, the 'red' collagenolytic granulomas are discussed; these are the lesions of necrobiosis lipoidica, necrobiotic xanthogranuloma, rheumatoid nodules, Churg-Strauss syndrome, and eosinophilic cellulitis (Well's Syndrome).     

川崎病患儿皮疹表现和分析

目的　探讨川崎病 (KD)患儿皮疹表现及其特点 ,提高川崎病的早期诊断率。方法　详细描述记录KD患儿皮疹的类型及表现 ,入院次日做血细胞计数、C 反应蛋白、血沉等检查 ,入院时至病程第 6周进行心脏冠状动脉检查 2～ 3次。结果　KD患儿皮疹多在病程 3～ 4天出现 ,种类以猩红热样、麻疹样、荨麻疹样皮疹较多见 ,1例表现为无菌性脓疱疹。有皮疹的患儿误诊率较高 ,冠状动脉病变发生率较高。结论　临床医生对小儿发热疹性疾病应高度警惕KD ,以免因误诊而失去预防冠状血管损伤的最佳时机。     

Anticonvulsiva-Hypersensitivitäts-Syndrom auf Carbamazepin

Summary. In a 39-year-old woman an anticonvulsant therapy was initiated because of focal attacks in the left arm and face. The patient experienced generalized maculopapular skin rashes in response to each of four chemically similar anticonvulsant drugs: phenytoin, carbamazepine, primidone and clonazepam. During administration of carbamazepine the clinical features included fever, hepatitis and haemotological eosinophilia in addition to the skin rash (anticonvulsant hypersensitivity syndrome). The anticonvulsant hypersensitivity syndrome is defined as an idiosyncratic reaction caused by disturbed drug metabolism. Positive lymphocytetransformation tests with carbamazepine and phenytoin indicate an immunological mechanism underlying the rashes in our patient. Patch testing with the four anticonvulsant drugs gave positive results only with carbamazepine. Skin biopsy showed the histological features of a delayed-type allergy. The anticonvulsant therapy was continued with a chemically unrelated preparation, valproic acid; this drug is well tolerated and has proved appropriate for prevention of seizures. Eingegangen am 2. August 1993 / Angenommen am 27. Oktober 1993     

与成人Still病相关的皮肤表现

成人Still病是一种病因未明的系统性炎症性疾病,临床上以规律性发热、关节炎或关节痛、淋巴结肿大、肝脾肿大及皮疹为主要特征.现有诊断标准中,Yamaguchi标准具有较高的敏感性和特异性.皮肤受累为成人Still病的主要症状之一,典型的一过性皮疹为Yamaguchi标准的一项重要表现.非典型皮疹具有多形性,包括持久性丘疹,斑块、荨麻疹样、皮肌炎样皮疹等.持久性丘疹,斑块及荨麻疹样皮疹特征性的临床及病理表现有利于疾病早期诊断,而持久性丘疹,斑块与皮肌炎样皮疹可能与疾病严重程度、预后相关.     

Histiocytoid neutrophilic dermatoses and panniculitides: variations on a theme

Requena et al, in their article titled "Histiocytoid Sweet syndrome," in 2005, established that the dermal infiltrate in some patients with Sweet's syndrome is composed of histiocyte-like immature myeloid cells, not polymorphonuclear leukocytes as is the norm. With this premise in mind, we report on 6 cases of inflammatory skin disease in which the common denominator was a dermal and/or subcutaneous infiltrate of histiocytoid myeloid cells in patients with new-onset cutaneous eruptions and systemic symptoms. The cases were diverse clinically and microscopically, fell short of the criteria necessary for a diagnosis of classical Sweet's syndrome, and were difficult to categorize at the outset. The systemic manifestations ranged from malaise alone to a combination of fever, chills, night sweats, and polyarthralgia. The clinical morphology of the cutaneous eruptions varied from being papulovesicular in 1 patient to mainly consisting of erythematous plaques and nodules in the remainder. The dermatologists' differential diagnoses included Sweet's syndrome in 3 cases, a drug eruption in 2, and other entities such as erythema nodosum and Well's syndrome. Biopsies in all cases revealed a dermal and/or subcutaneous infiltrate composed predominantly of mononuclear histiocytoid cells of myeloid origin. With the benefit of detailed clinicopathologic correlation, the cases were classified for the purpose of this report as follows: Sweet's-like neutrophilic dermatosis, histiocytoid (3 cases); subcutaneous Sweet's syndrome, histiocytoid (2 cases); histiocytoid neutrophilic dermatosis, unspecified (1 case). In addition, we describe a further instructive case that exhibited overlap with those in the series but proved ultimately to represent leukemia cutis. The spectrum of observations in this report supports and expands the original concept of histiocytoid Sweet's syndrome.