嗜酸性粒细胞增多性皮炎1例

报告1例嗜酸性粒细胞增多性皮炎.患者男,77岁.全身红斑、斑块伴反复发热2年余,加重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:嗜酸性粒细胞增多性皮炎.给予糖皮质激素、抗感染及支持对症等治疗17天后出院.现随访中.     

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。     

嗜酸性蜂窝织炎

嗜酸性蜂窝织炎(Eosinophilic Cellulitis)是一种少见的、有独特的临床(开始似蜂窝织炎样、随后变为硬斑样的单发或多发性皮损)和组织病理改变(真皮有明显的嗜酸性粒细胞浸润和有火焰样图形的炎症性肉芽肿)特征,并伴外周血嗜酸性粒细胞增多的复发性皮肤病。本病首先由Wells在1971年以“伴有嗜酸性粒细胞增多的复发性肉芽肿性皮炎”(Recurrent Gnanulomatous Derma—     

嗜酸性粒细胞增多综合征1例

患者男,78岁。全身出现丘疹,伴瘙痒2年余,出现红斑,伴脱屑半年余,既往体健。外周血和骨髓嗜酸性粒细胞增多,组织病理改变符合嗜酸性粒细胞增多综合征。予糖皮质激素、抗感染和支持对症治疗20d,痊愈。     

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     

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.     

以皮肤损害为突出表现的嗜酸性粒细胞增多综合征1例

患者女,35岁,全身皮肤红斑、丘疹、肥厚伴瘙痒7年,加重6个月。外周血、骨髓涂片及活检、流式细胞术检测均提示嗜酸性粒细胞明显增多;皮损组织病理改变符合嗜酸性粒细胞增多综合征;淋巴结活检:淋巴结不典型增生,伴较多浆细胞及少量嗜酸性粒细胞浸润。IgE 7 100 IU/mL; HBsAg(+)、抗HBsAg(-)、HBeAg(+)、抗HBeAg(-)、抗HBcAg(+);乙肝病毒DNA定量:2. 04E+002。诊断:嗜酸性粒细胞增多综合征、慢性乙型肝炎。采用糖皮质激素联合抗乙肝病毒药物治疗,疗效显著。     

嗜酸性粒细胞增多综合征的诊断与治疗

嗜酸性粒细胞增多综合征（HES）是一类与嗜酸粒细胞相关并具有共同临床特点的谱性疾病.疾病的一端为病程良性的嗜酸性粒细胞增多性皮炎,仅累及皮肤,无器官或系统受累,预后较好;疾病的另一端为慢性嗜酸性粒细胞白血病或淋巴瘤,可以累及全身多个器官、系统,出现较严重的临床症状,甚至导致死亡,预后差.该病1968年由Hardy和Anderson首先提出,认为HES是病因不明、血液及骨髓中嗜酸性粒细胞（EOS）持续增多,组织中嗜酸性粒细胞（EOS）浸润为特征的一类疾病.     

嗜酸性粒细胞增多性血管淋巴样增生1例

报告1例嗜酸性粒细胞增多性血管淋巴样增生。30岁男性患者头顶多发暗红色圆形丘疹、结节3年，无瘙痒、疼痛等自觉症状；实验室检查示外周血嗜酸性粒细胞明显增多外，余常规检查均无明显异常；头皮结节组织病理检查示真皮血管显著增生，伴有以淋巴细胞和嗜酸性粒细胞为主的混合浸润。结合临床及组织病理学，诊断为嗜酸性粒细胞增多性血管淋巴样增生。     

Eosinophilic fasciitis associated with Borrelia burgdorferi infection

BACKGROUND: Eosinophilic fasciitis (Shulman syndrome) is defined by the association of sclerodermatous skin changes involving underlying fascia and hypereosinophilia. While the aetiology is unknown, some observations suggest an infectious origin. We report the association of eosinophilic fasciitis with an infection involving Borrelia burgdorferi. PATIENTS AND METHODS: A 54 year-old man consulted for a hardened oedema and stiffness of the calves associated with an oedema of the left hand evolving for 4 months. Routine blood tests showed hypereosinophilia at 1.01 G/l and moderate inflammatory syndrome. Diagnosis of eosinophilic fasciitis was confirmed by MRI and muscle biopsy. Since the patient had reported previous tick bites some months before onset, he was tested for Lyme disease. An ELISA test revealed IgG directed against Borrelia burgdorferi and this was confirmed by Western blot analysis. DISCUSSION: The association of eosinophilic fasciitis with Lyme disease raises the question of a real link or a fortuitous association between the two conditions. Similar cases have been described in the literature with or without isolation of the spirochete from skin or fascia lesions. The incidence of eosinophilic fasciitis remains low compared to the prevalence of the infection in endemic areas. We suggest that in some patients, perhaps genetically predisposed, infection with B. burgdorferi may be at the origin of fasciitis.     

嗜酸性粒细胞增多综合征1例

患者男,65岁。全身反复出现红斑和丘疹,伴瘙痒5年,加重2周。伴心、肝和肺多器官功能损害。骨髓及外周血嗜酸性粒细胞明显增多。诊断:嗜酸性粒细胞增多综合征。予糖皮质激素、雷公藤多甙及对症支持治疗半月后,症状明显好转。     

Gleich's syndrome

BACKGROUND: Episodic angioedema with eosinophilia is a new syndrome associating hypereosinophilia, episodic angioedema and elevation of immunoglobulin M. This syndrome, first described by Gerald Gleich, has a good prognosis and no organ involvement. CASE REPORT: A 39-year old woman developed for 10 years, recurrent episodes of swelling, weight gain of 4 to 6 kg, hypereosinophilia and hyperimmunoglobulinemia M and G. All symptoms disappeared spontaneously within a few days. Numerous investigations (cutaneous, cardiac, pulmonary, immunologic, parasitologic) were negative. The patient was recently hospitalized because of increased recurrence of episodes and major discomfort. We noted hypereosinophilia up to 30. 731 eosinophils/mm(3), elevated lactate dehydrogenase to 902 U/l (N: 204-412), elevated eosinophile cationic protein to 371 microg/l (N<12) and elevated immunoglobulin E to 140 U/l (N<100). Renal, pulmonary and cardiac functions were normal. Bone marrow biopsy showed no abnormality. DISCUSSION: Gleich's syndrome is a benign but often incapacitating disease of unknown etiology. Systemic glucocorticoids may control flare-ups.     

Rare manifestation of hypereosinophilic syndrome: Diffuse-type hair loss with massive perifollicular eosinophils

A 46-year-old woman consulted our hospital with diffuse alopecia and blood eosinophilia. Histological examination of the scalp revealed dense eosinophilic infiltration around the hair follicles and in the surrounding subcutis. Oral corticosteroid was effective to reduce hair loss and blood eosinophilia, but these conditions immediately relapsed after ending treatment. In addition to alopecia, she had diarrhea and colitis showing histological findings of dense eosinophilic infiltrations in the submucosa. We diagnosed hypereosinophilic syndrome based on hypereosinophilia of blood and tissue with clinical symptoms of alopecia and diarrhea. We suppose diffuse alopecia showing massive eosinophilic infiltration around the hair follicle is a rare symptom of hypereosinophilic syndrome.     

Lymphocytic variant of hypereosinophilic syndrome

Hypereosinophilia may be associated with any of several underlying diseases. Atopy or allergic drug reactions are the most common causes, but infections with bacteria and parasites should also be considered in the differential diagnosis. When thorough evaluation of a patient with chronic hypereosinophilia fails to reveal an underlying disease, the diagnosis of idiopathic hypereosinophilic syndrome (HES) should be considered. We report a patient with unexplained persistent hypereosinophilia associated with a chronic pruritic rash and an underlying diagnosis of HES (lymphocytic variant).     

A case of hypereosinophilic syndrome

A 22-year-old man developed papules on his legs in November of 2001, which then spread to cover almost his entire body. He was treated with a topical steroid and PUVA therapy at another hospital. The symptoms showed no improvement, and, in February of 2002, he came to our hospital. Examination revealed hypereosinophilia, and, because he had symptoms of organ involvement by the heart, lung, and inguinal lymph nodes as well as the skin, we diagnosed him with idiopathic hypereosinophilic syndrome (HES). The patient was treated with oral prednisolone at a dose of 60 mg/day, and his cutaneous lesions and other organ symptoms improved.     

Churg-Strauss syndrome (CSS) manifested as necrosis of fingers and toes and liver infarction

We report a case of Churg-Strauss syndrome (CSS) with necrosis of the fingers and toes and liver infarction. A 59-year-old man with asthma suddenly noticed that his fingers and toes felt unusually cold. This condition worsened progressively, and some digits became necrotic within several weeks. Laboratory studies revealed hypereosinophilia and an extremely elevated serum level of IgE. Digital subtraction angiography of the extremities revealed extensive irregular narrowing of small and medium-sized arteries in the extremities. Abdominal computed tomography (CT) revealed an area of low density at the periphery of the right lobe of the liver. Angiography revealed irregular narrowing of small arteries that corresponded to the ischemic area. A nerve conduction study suggested sensory nerve neuropathy. The preceding asthma, acute onset of digital necrosis, liver infarction, neuropathy, and hypereosinophilia strongly suggested a diagnosis of CSS. The patient was treated with 40 mg of prednisolone and 120 micro g of intravenous prostaglandin E1 daily, and all the digits that had turned black and necrotic were amputated. After the amputation, the dose of prednisolone was gradually reduced, and no new lesions appeared on the skin or in the liver. The rare possible complications of CSS, including necrosis of digits and liver infarction, should not be ignored.     

Hypereosinophilic syndrome as prodrome of bullous pemphigoid:report of a case

Persistent hypereosinophilia, cardiac involvement and a recurrent erythematous-papular pruritic eruption histologically characterized by eosinophilic spongiosis are described in a 77-year-old man. This condition, suggestive of "idiopathic" hypereosinophilic syndrome, represented the prodromic phase of atypical bullous pemphigoid, which manifested 7 months later.     

Dress syndrome with sepsis, acute respiratory distress syndrome and pneumomediastinum

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome reflects a serious hypersensitivity reaction to drugs, and is characterized by skin rash, fever, lymph node enlargement, and internal organ involvement. So far, numerous drugs such as sulfonamides, phenobarbital, sulfasalazine, carbamazepine, and phenytoin have been reported to cause DRESS syndrome. We report a case of a 10-year-old girl who developed clinical manifestations of fever, rash, lymphadenopathy, hypereosinophilia, and visceral involvement (hepatitis and pneumonitis) after taking phenobarbital for seizures, with subsequent development of sepsis, acute respiratory distress syndrome (ARDS) and spontaneous air leak syndrome (pnemothorax and pneumomediastinum). She was put on steroids and various antibiotics and was ventilated, but ultimately succumbed to sepsis and pulmonary complications.