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chronic granulomatous communicable disease occurring in tropical and subtropical regions; characterized by inflamed nodules beneath the skin and wasting of body parts; caused by the bacillus Mycobacterium leprae (同)Hansen''s disease

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ハンセン病,らい病

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/12/30 11:41:37」(JST)

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Lepromatous leprosy is a skin condition consisting of pale macules.^[1]^:346

It results from the failure of Th1 cell activation which is necessary to eradicate the mycobacteria (Th1 response is required to activate macrophages that engulf and contain the disease). In lepromatous leprosy, TH2 response is turned on, and because of reciprocal inhibition (IL-4; IL-10), the cell-mediated response (TH1) is depressed.

This debilitating form of leprosy begins to spread causing the eyebrows to disappear and spongy tumor like swellings appear on the face and body. The disease attacks the internal organs, bones, joints and marrow of the body resulting in physical degeneration. The result is deformity with loss of feeling in the fingers and toes which eventually fall off. Contrary to popular belief, both forms of leprosy are curable, with the lepromatous form classically treated with antibiotics Dapsone, Rifampin and Clofazimine for as long as 2–5 years, but if left untreated the person may die up to 20 or 30 years from its inception.^[2]

Early detection of the disease is of utmost importance, since severe physical and neurological damage are irreversible even if cured (e.g. blindness, loss of digits/limbs/sensation). Early infection is characterized by a well demarcated, usually pale, skin lesion which has lost its hair, and there may be many of these lesions if the infection is more severe (most commonly found on the cooler parts of the body such as the elbows, knees, fingers, or scrotum, as the bacteria thrive in cooler environments). This early presentation is the same for both tuberculous and lepromatous forms of leprosy as they are a spectrum of the same disease (lepromatous being the more contagious and severe form in patients with impaired Th1 response). Disease progression is extremely slow, and signs of infection may not appear for years.^[3]

Family members, and especially children, who have family members with the disease are most at risk. The disease is believed to be spread through respiratory droplets in close quarters like its relative Mycobacterium tuberculosis, and similarly requires extended exposure to an individual in most situations, so outsiders and healthcare workers are normally not infected (except with the most infective individuals such as those in the most progressed lepromatous forms, as those patients have the highest bacterial loads).^[4]

References

^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
^ (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)
^ (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)
^ (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)

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1. ハンセン病の疫学、微生物学、臨床症状、および診断 epidemiology microbiology clinical manifestations and diagnosis of leprosy
2. ハンセン病の治療および予防 treatment and prevention of leprosy
3. Epidermodysplasia verruciformis
4. ロボミコーシス lobomycosis
5. 網状（角ばった）紫斑が認められる患者に対するアプローチ approach to the patient with retiform angulated purpura

English Journal

Langerhans cells (CD1a and CD207), dermal dendrocytes (FXIIIa) and plasmacytoid dendritic cells (CD123) in skin lesions of leprosy patients.

Hirai KE1, Aarão TL1, Silva LM1, de Sousa JR2, de Sousa J1, Dias LB Jr1, Oliveira Carneiro FR2, Fuzii HT2, Quaresma JA3.
Microbial pathogenesis.Microb Pathog.2015 Nov 27. pii: S0882-4010(15)30136-4. doi: 10.1016/j.micpath.2015.11.013. [Epub ahead of print]
The clinical course of infection with Mycobacterium leprae varies widely and depends on the pattern of the host immune response. Dendritic cells play an important role in the activation of the innate and adaptive immune system and seem to be essential for the development of the disease. To analyze t
PMID 26639680

Correlation between nerve growth factor and tissue expression of IL-17 in leprosy.

Aarão TL1, de Sousa JR2, Botelho BS1, Fuzii HT2, Quaresma JA3.
Microbial pathogenesis.Microb Pathog.2015 Nov 23;90:64-68. doi: 10.1016/j.micpath.2015.11.019. [Epub ahead of print]
Leprosy is a serious public health problem in peripheral and developing countries. Leprosy is a chronic infectious-contagious disease caused by the intracellular, bacillus Mycobacterium leprae, which causes tissue damage and demyelination of peripheral nerves. Recent studies have demonstrated the pa
PMID 26616164

Lucio's phenomenon, an uncommon occurrence among leprosy patients in Sri Lanka.

Herath S1, Navinan MR2, Liyanage I3, Rathnayaka N4, Yudhishdran J5, Fernando J6, Sirimanne G7, Kulatunga A8.
BMC research notes.BMC Res Notes.2015 Nov 13;8(1):672. doi: 10.1186/s13104-015-1671-1.
BACKGROUND: Lucio's phenomenon is a rare manifestation of untreated leprosy which is seen almost exclusively in regions surrounding the Gulf of Mexico. Its occurrence elsewhere though documented is considered uncommon. We present a case of Lucio's phenomenon in a previously undiagnosed leprosy patie
PMID 26566619

Japanese Journal

らい菌貪食に伴うマクロファージの抗Mycobacterium intracellulare 殺菌能の低下

多田納豊,佐野千晶,江森方子,斎藤肇,佐藤勝昌,清水利朗,冨岡治明
日本ハンセン病学会雑誌 81(3), 175-183, 2012
　らい腫型（L 型）患者でみられるらい腫抗原に対する特異的な免疫不応性とこれに起因する感染抵抗性の欠如・低下は、T 細胞のらい菌抗原不応性に起因しているものと考えられている。本研究では、L 型のらい病変部において、らい菌に接触あるいは感染したマクロファージ（MΦ）そのものの殺菌能の減弱の可能性について明らかにするため、らい菌を貪食した MΦ のMycobacterium avium complex …
NAID 130002141101

チンパンジーとハンセン病

石井則久,鵜殿俊史,藤澤道子,伊谷原一,谷川和也,宮村達男,鈴木幸一
日本ハンセン病学会雑誌 = Japanese journal of leprosy
… Leprosy is suspected to develop after a long period of latency following infection with Mycobacterium leprae (M. … We found a rare case of leprosy in a chimpanzee (Pan troglodytes) born in West Africa (Sierra Leone) and brought to Japan around 2 years of age. … At 31, the ape started exhibiting pathognomic signs of leprosy. …
NAID 10027809196

日本人のハンセン病発症における細菌センサー分子の遺伝子多型の関与

金澤伸雄,三木田直哉,李洪錦,中谷友美,尾崎元昭,小坂眞紀,石井則久,西村泰行,古川福実
日本ハンセン病学会雑誌 = Japanese journal of leprosy
らい菌による慢性感染症であるハンセン病は、本邦においては新規発生をほぼ制圧できているものの、途上国ではまだ多くの患者がいる。ハンセン病発症に作用する宿主側要因の一つとして、遺伝的素因の関与が想定され、実際にいくつかの遺伝子変異が、発症の有無のみならず、らい腫型／類結核型／らい反応といった病型の違いにおいても関与することが報告されている。今回我々は、近年の自然免疫に関する研究の進展に基づき、代表的な …
NAID 10026084926

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★リンクテーブル★

リンク元	「らい腫型ハンセン病」「LL」「L型ハンセン病」「らい種性らい」「らい腫性ハンセン病」

「らい腫型ハンセン病」

Lepromatous leprosy
	Leonine facies (lepromatous leprosy)
Classification and external resources
Specialty	infectious disease
ICD-10	A30.5
ICD-9-CM	030.0
DiseasesDB	8478
	[edit on Wikidata]

　　[★]

英: lepromatous leprosy LL
同？: L型ハンセン病、らい腫性ハンセン病、らい種性らい

[show details]

L型ハンセン病 : 25 件
らい種性らい : 12 件
らい腫性ハンセン病 : 17 件
らい腫型ハンセン病 : 27 件

「LL」

　　[★]

「L型ハンセン病」

　　[★]

英: lepromatous leprosy
関: らい腫性ハンセン病、らい種性らい、らい腫型ハンセン病、皮膚らい

「らい種性らい」

　　[★]

英: lepromatous leprosy
関: L型ハンセン病、らい腫性ハンセン病、らい腫型ハンセン病

「らい腫性ハンセン病」

　　[★]

英: lepromatous leprosy
関: L型ハンセン病、らい種性らい、らい腫型ハンセン病

[Andrews-1] James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.

[2] (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)

[3] (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)

[4] (PDF) http://www.hrsa.gov/hansensdisease/pdfs/faq.pdf. Missing or empty |title= (help)

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案内

lepromatous leprosy