发热性溃疡性Mucha-Habermann病1例

患儿男,9岁,四肢反复皮疹伴疼痛,间断发热2年余,加重1年。患儿四肢可触及暗红色结节、斑块,部分皮损伴溃疡及萎缩性瘢痕,无关节痛、淋巴结及肝脾肿大。组织病理:真皮全层小血管增多,血管周围淋巴细胞浸润伴界面皮炎,亦可见水肿及灶性表皮坏死,未见淋巴细胞非典型性。诊断为发热性溃疡性Mucha-Habermann病。患儿男,9岁,四肢反复皮疹伴疼痛,间断发热2年余,加重1年。患儿四肢可触及暗红色结节、斑块,部分皮损伴溃疡及萎缩性瘢痕,无关节痛、淋巴结及肝脾肿大。组织病理:真皮全层小血管增多,血管周围淋巴细胞浸润伴界面皮炎,亦可见水肿及灶性表皮坏死,未见淋巴细胞非典型性。诊断为发热性溃疡性Mucha-Habermann病。     

急性发热性嗜中性皮病1例

患者女,52岁。因面部、颈部及四肢出现红色丘疹及斑块,不痛不痒1个月,于2007年8月8日至我院皮肤科就诊。患者于1个月前双上臂出现散在红色丘疹及斑块,无自觉症状,表面无鳞屑,未予治疗。嗣后皮损逐渐增多,面部、颈部及双下肢出现同样皮损,无疼痛、瘙痒及烧灼感,不伴发热,无关节疼痛。曾在外院就诊,疑诊为肉芽肿予以药物（具体用药不洋）治疗,皮损未见好转。     

发热溃疡坏死性Mucha-Habermann病一例

发热溃疡坏死性Mucha-Haberman病(Febrile ulceronecrotic Mucha-Habermann's disease)是急性痘疮样苔藓状糠疹(pityriasis lichenoides et varioIiformis acuta,PLEVA)一种极为罕见的严重类型.     

中毒性表皮坏死松解症2例并文献复习

回顾2例中毒性表皮坏死松解症（TEN）患者临床资料,检索相关文献,复习TEN并总结经验。     

急性发热性嗜中性皮病1例

患者女,34岁,反复颜面、上肢红色斑块、水疱4月,再发4天.皮肤科检查:前额、面部、左前臂、左腕部、右肘部、手背、指背关节处可见多发散在鲜红色、水肿性斑块,表面可见多发小脓疱、水疱.皮损以右面部为重,边缘稍隆起,表面结黄色浆液痂或脓痂.触之有浸润感,压痛.皮损组织病理检查:真皮乳头水肿,真皮浅、深层弥漫性中性粒细胞浸润...     

甲氨喋呤治疗红皮病14例分析

1995年 8月至 1998年 12月 ,我科共收治红皮病 14例 ,均使用甲氨喋呤治疗 ,取得了较满意的效果 ,现报告如下 :1　资料与方法1 1　临床资料　 14例中男性 12例 ,女性 2例 ,男女之比 6∶1,年龄 8～ 71岁 ,6 0岁以上占 5例 ,2 0岁以下 1例 ,平均年龄 49 5岁 ;病程半天至 5月 ,平均 46 5天。以红皮病为首发症状者 2例 (均大于 6 0岁 ) ,继发于银屑病 5例 (中年居多 ) ,泛发性湿疹 3例 ,接触性皮炎 2例 ,药疹 2例。因治疗不当引起者 7例 (不知名草药烫洗 2例 ,敌百虫稀释液外搽1例 ,长期使用“地塞米松”等药物 4例 ) ,原因不明者 7例。所有患者…     

20例急性发热性嗜中性皮病临床病理分析

对２０例ＡＦＮＤ进行临床病理分析，该病临床皮损主要表现为疼痛性水肿性红色斑块，皮损好发于面、颈部及四肢，多数伴有发热和中性多核粒细胞增多，病理特点主要在真皮内可见以中性多核粒细胞为主的弥漫性浸润。     

急性发热性嗜中性皮病1例

急性发热性嗜中性皮病亦称斯威特(Sweeet)综合征。是一种原因不明的慢性易复发性红斑与结节性皮肤病，临床常见但泛发伴有水疱，表皮坏死者少见。我们发现1例报道如下。临床资料患者男，43岁，农民。因感冒发热，伴发扁桃体炎，在当地医院静脉滴注青霉素5天好转。3周后在颈部、面部及四肢相继出现淡红色至暗红色斑块和结节，自觉灼热疼痛，触痛明显以致病人有时不能忍受，下肢膝关节痛，上肢肘、腕关节痛。在当地医院按病毒感染性疱疹治疗(药物不详)，效果不佳，于2003年12月来我站就诊，     

汗孔角化病2例及文献复习

报告2例汗孔角化病。例1.患者女,57岁,面部、颈部及四肢斑块渐增多30年。皮肤科检查：面部、颈部及四肢等暴光部位可见圆形、花环状或不规则的角化过度斑,边缘隆起,中央轻度凹陷、萎缩。诊断：播散性浅表性光线性汗孔角化病。例2.患者男,45岁,全身散在斑块20年余。皮肤科检查：全身散在棕褐色角化性斑块,部分边缘隆起,纤细如线,离心性扩大,中央色素沉着。诊断：播散性浅表性汗孔角化病。2例皮损组织病理检查均可见角化不全柱斜插入凹陷的表皮内。全外显子测序发现相关突变,例1为MVK c.1126G>A,例2为ATP2C1 c.899+4A>T。     

播散性皮肤组织胞浆菌病1例及文献复习

患者女,31岁。多发性骨关节疼痛伴发热8月,多发性皮肤脓肿4月。脓液及组织真菌培养可见双相真菌荚膜组织胞浆菌,经rDNA的ITS区鉴定该菌与荚膜组织胞浆菌荚膜变种高度同源。诊断:播散性皮肤组织胞浆菌病。予口服伊曲康唑0.2g(2次/d)和5-氟胞嘧啶1g(3次/d)治疗,5个月后患者病情明显好转。并对相关文献进行复习。     

Febrile Ulceronecrotic Mucha-Habermann's Disease

Febrile ulceronecrotic Mucha-Habermann's disease (FUMH) was first described by Degos in 1966. In the literature, nine cases of FUMH have been reported in both children and adults. We report a 16-year-old boy with the febrile ulceronecrotic type. A review of the nine cases in the literature showed acute necrotic lesions, as well as rare complications such as fever, superinfected lesions and viral infection which are not as common in pityriasis lichenoides et varioliformis acuta. There is no definitive treatment, but systemic corticosteroid, methotrexate, antibiotics (tetracycline, erythromycin), aciclovir, and 4,4-diaminodiphenyl sulfone (DDS) have been frequently used. The most common histologic feature is mononuclear perivascular infiltrates consisting of T lymphocytes. The etiology is not known, but a hypersensitivity reaction, possibly to an infectious agent, is suggested.     

Febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta with fatal outcome

Ulceronecrotic Mucha-Habermann disease is a severe, febrile form of pityriasis lichenoides et varioliformis acuta. The condition may sometimes have a fatal outcome, especially in elderly patients. In this paper, we are reporting on a 60-year-old male patient who suffered from the eruption of erythematous, haemorrhagic, ulceronecrotic papules accompanied by high temperature. Erosions and ulceronecrotic papules covered over 80% of his body. Three weeks after his admittance to our hospital, clinical signs resembling ileus together with the leucocytosis occurred. The patient was transferred to the department of internal medicine and later to the surgery department. The fulminant course of the disease could not be stopped, and the patient died of severe intestinal and colon gangrene caused by the massive thrombosis of superior mesenteric artery. Up to date, only 23 cases of this severe form of the disease have been reported, and even with the early recognition, fulminant course may lead to death.     

发热溃疡坏死性急性痘疮样苔藓样糠疹的护理

总结1例发热溃疡坏死性急性痘疮样苔藓样糠疹的护理要点,包括:严密监测病情,针对皮损的程度实施皮肤创面综合护理,做好留置导尿护理,营养支持,心理护理。     

Febrile Ulceronecrotic Mucha-Habermann''s Disease with Interstitial Pneumonitis

A case of febrile ulceronecrotic Mucha-Habermann's is presented. This disorder is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by the sudden eruption of diffuse coalescent ulcerations associated with high fever. In the present case the disease was preceded by the milder typical form of PLEVA. Histologically a leukocytoclastic vasculitis was seen in addition to the usual lymphocytic perivascular and lichenoid infiltrate. During the course of the disease the patient developed an interstitial pneumonitis which resolved concomitantly with the cutaneous lesions. Adenovirus type II recovered at the height of the illness from the patient's urine may have etiologic implications in the pathogenesis of the disease.     

Febrile ulceronecrotic Mucha-Habermann's disease and its successful therapy with DDS

A case of febrile ulceronecrotic Mucha-Habermann's disease and its therapy with 4,4-diaminodiphenyl sulfone (DDS) are reported. This disorder is a severe form of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by the sudden eruption of diffuse coalescent ulcerations associated with systemic symptoms. In our case, the disease was preceded by the mild form of PLEVA and the patient was admitted with diffuse ulceronecrotic eruptions and high fever. After admission, the patient, who had not responded to antibiotics, recovered dramatically with DDS therapy.     

Febrile ulceronecrotic Mucha‐Habermann disease: A case with systemic symptoms managed with subcutaneous methotrexate

Febrile ulceronecrotic Mucha‐Habermann disease (FUMHD) is a rare, idiopathic, acquired dermatosis that can affect all ages and ethnic groups. We present a 10‐year‐old patient with FUMHD associated with arthritis and chronic fatigue, managed with methotrexate. Through our literature review, we also explore treatment protocols for a disease for which internationally standardized management is yet to be formulated.     

Varicella Precipitating Febrile Ulceronecrotic Mucha‐Habermann Disease

We report a 9‐year‐old boy with skin lesions clinically and histologically compatible with pityriasis lichenoides et varioliformis acuta that evolved to the severe variant febrile ulceronecrotic Mucha‐Habermann disease and finally to pityriasis lichenoides chronica. Varicella‐zoster virus (VZV) was isolated in culture medium from the skin lesions and serum serology was positive for VZV. This is the first time that a virus has been isolated in culture in this condition.     

以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状的间变性大细胞淋巴瘤1例

患儿女,7岁,以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状,半年后左下肢出现皮下肿物确诊为间变性大细胞淋巴瘤。患儿首发临床表现为全身皮肤水疱、溃疡、结痂伴发热、腹股沟淋巴结肿大。皮损组织病理:表皮可见角化不良细胞,界面改变,表皮少量淋巴细胞浸润,未见异型细胞;淋巴结病理:淋巴结大片坏死,其间血管壁坏死,周围绕以多数淋巴样细胞;肿物病理:大量淋巴样细胞弥漫分布。免疫组织化学:CD30(+),ALK胞浆(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+)。诊断:间变性大细胞淋巴瘤。     

Febrile Ulceronecrotic Mucha‐Habermann Disease with Central Nervous System Vasculitis

Abstract: Febrile ulceronecrotic Mucha‐Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). We report a case of FUMHD in an 11‐year‐old boy who subsequently developed neurologic symptoms and was found to have necrotizing vasculitis on brain biopsy. Over 5 years of follow‐up, he had one biopsy‐proven recurrence of his skin lesions and continued rehabilitation and treatment for residual neurologic complications. This case provides histological evidence of central nervous system vasculitis associated with FUMHD. Our patient’s disease was eventually controlled with cyclophosphamide.