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1、麻风的诊断与治疗,概念,麻风（leprosy）又称Hansen病。由麻风分枝杆菌（Mycobacterium leprae）引起的一种慢性传染性疾病，主要侵犯皮肤、黏膜和周围神经，少数情况下可侵犯深部组织和内脏器官。,Dr. GH Armauer Hansen( Norway) and his Patient,The Louisiana Leper Home was established in 1894 to provide “a place of refuge, not reproach; a place of treatment and research, not detention.“
2、,流行病学,流行区域： 世界性：非洲、东南亚、拉丁美洲和西太平洋地区 中国：沿海与长江流域发病情况： 青壮年 男女之比3:1 家庭聚集性 社会因素影响：贫穷、居住环境、伴有HIV感染等,流行病学,传染源： 人是唯一传染源，特别是未经治疗的多菌型麻风病人是主要传染源传播途径： 传统观点：与麻风病人长期接触 新近观点：与肺结核类似的空气传播易感人群： 取决于人体的免疫反应与功能 正常成人一般感染后不发病,Leprosy is asymmetrically distributed globally, with the greatest incidence and prevalence in the
3、developing world. Globally, the incidence has fallen by 70% and the prevalence has fallen by 90% since 2000 thanks to aggressive eradication efforts with multidrug treatment regimens.,病因与发病机制,麻风杆菌（Mycobacterium leprae）革兰染色阳性，抗酸染色阳性形态多样，与病人治疗情况密切相关迄今体外培养未成功,病因与发病机制,发病机制主要分布于皮肤（主要位于真皮层的末梢神经、巨噬细胞、立毛肌、毛
4、囊与皮脂腺）、黏膜（主要是鼻黏膜）、周围神经（不侵入中枢神经），引起相应病理反应。破损皮肤和鼻黏膜排出大量麻风杆菌，为麻风主要传染源。,Mycobacterium leprae is an acid-fast, rod-shaped bacillus. It is an obligate, intracellular organism that preferentially proliferates in tissues with cooler temperatures. The organism exhibits the longest reproduction time among all
5、 bacteria, requiring 13 days to double in experimentally infected mice, and with an incubation time of up to 50 years in humans.,In the United States, armadillos(南美犰狳) are a major reservoir of leprosy infection. They are one of the few animals known to become infected and are used in research effort
6、s to study the disease. They have an unusually low body temperature of around 34C, which is hospitable to M leprae growth.,临床表现,平均潜伏期25年。发病率约15%。因患者免疫力不同，临床表现差异较大，几乎涉及临床各个学科。,临床表现,皮肤表现：皮损形态多样：有斑疹、丘疹、结节、斑块、浸润、水疱、溃疡等皮肤附属器侵犯：表现为头发、眉毛脱落，闭汗、皮肤干燥,临床表现,与其他皮肤病不同在于：局部感觉障碍出汗障碍可找到麻风杆菌,Leprosy: tuberculoid type
7、 Well-defined, hypopigmented, slightly scaling, anesthetic macules and plaques on the posterior trunk.,Leprosy: borderline-type A 31-year old Vietnamese female. A. Geographic shaped plaque on the buttock with raised red indurated margins and central clearing. There is extension of the infection indi
8、cated by the erythematous papules beyond the margins. B. Similar geographic plaque on the legs with raised red margins and central clearing.,A,B,Leprosy: borderline-type A 26-year-old Vietnamese male. A. Well-demarcated, infiltrated,erythematous plaques on the face. B. Identical red plaques on the l
9、ower back.,A solitary, anesthetic, and annular lesion of polar tuberculoid leprosy, which had been present for 3 months. Its sharp margins, erythema, and scale are more evident than its elevation.,One of several lesions of borderline tuberculoid leprosy, which had an incompletely annular configurati
10、on with satellite papules. Compared to the polar tuberculoid lesion, there is less erythema, no evident scales, but sharp margination.,Characteristic borderline or dimorphic lesion is an indurated and elevated annular plaque,It has a well-defined “tuberculoid” interior margin but a poorly defined “l
11、epromatous” exterior margin. Having both morphologies, it is termed “dimorphic.”,Multiple lesions in a patient with borderline lepromatous leprosy. The annular lesions vary in size and are asymmetrically distributed. In contrast, the poorly defined papular and nodular lesions are roughly symmetric.
12、Impairment of sensation was present in most lesions.,Multiple dermatofibroma-like (histoid) lesions, which are solitary and confluent, in a patient with subpolar lepromatous leprosy. Their uniformly sharp margins and erythema are in contrast to most of the lesions in borderline lepromatous leprosy.,
13、These multiple, barely palpable, erythematous, and asymptomatic lesions had been erupting over the previous 2 months in the same patient. With treatment, as lesions remitted, they became mildly hyperpigmented.,临床表现,周围神经症状：发生于几乎所有患者，表现为神经粗大侵犯神经：尺神经、耳大神经、正中神经、腓总神经、眶上神经、面神经、桡神经、胫神经,临床表现,周围神经症状： 功能障碍： 浅
14、感觉障碍：最早最常见，温觉最早，痛觉次之，触觉最后 运动障碍：肌肉萎缩或瘫痪所致，表现为爪形手、足下垂、面瘫等 营养性障碍：皮肤干燥、萎缩、脱毛，易形成水疱或溃疡 循环障碍：手足发绀、温度降低、肿胀 出汗障碍：,The facial nerve is another commonly damaged nerve in leprosy. The patient shown has both a facial nerve palsy and contractures of the hand from leprosy.,Long-standing neuropathy in the feet can
15、 lead to nonhealing ulcers, abscess formation, and osteomyelitis. If untreated early in the disease course, deformities may occur. The image shown is of an individual with advanced lepromatous leprosy with amputation of the toes and trophic ulceration.,Plain film x-rays can demonstrate significant b
16、ony changes in patients with long-standing disease. The x-ray of the foot shows absence of the second to fifth toes and distal tapering of the metatarsals secondary to neuropathy. The phalanges of the great toe show destruction secondary to osteomyelitis.,临床表现,眼部症状：由于麻风反应、麻风本身、三叉神经受累所致，表现为慢性结膜炎、角膜炎、巩膜炎、泪囊炎、虹膜睫状体炎等。鼻部症状：鼻黏膜充血、肿胀、糜烂、结痂、结节、浸润、肥厚等。其他：咽喉不适，淋巴结肿大、骨骼和内脏受累。,麻风反应,为机体免疫系统对感染的免疫学反应发生变化所致，常导致神经损伤，但并不意味着疾病进展或治疗失败。型麻风反应： 与细胞免疫有关，又称迟发型超敏反应。免疫力增强时为升级反应或逆向反应，多发生于正规药物治疗患者，表现为界限类麻风向结核型转化，原有皮损加重或出现新发皮损。因神经反应可诱发或加重畸形。免疫力降低时为降级反应，病情加重，向瘤型方向转化。,
