Hansen's disease

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an inflammatory disease caused by Mycobacterium leprae, manifested in various ways, depending on the host's ability to develop cell-mediated immunity. It is a chronic communicable disease characterized by the production of granulomatous lesions of the skin, mucous membranes, and peripheral nervous system. Not readily contagious, it often results in severe disability but is rarely fatal. Called also Hansen's disease. adj., adj lep´rous.
Frequency and Transmission. Leprosy is essentially a tropical disease, although it has occurred in every country in the world. According to the World Health Organization, the number of leprosy patients in the world was less than 600,000 at the beginning of 2001. Its control remains a problem in six countries: Brazil, India, Madagascar, Mozambique, Myanmar, and Nepal.

Leprosy is not inherited, but the actual means of transmission have not yet been established. It is known that the source of infection is the discharge from lesions of persons with active cases. It is believed that the bacillus enters the body through the skin or through the mucous membranes of the nose and throat. Leprosy is considered one of the least contagious of infectious diseases; only 3 to 5 per cent of those exposed to it ever contract it.
Symptoms. The average incubation period of leprosy is 3 years. Initially, the infection is confined to the sheaths of nerves in the dermis. The disease progresses by spreading up the nerve sheath, resulting in loss of sensation, or by forming subcutaneous nodules and skin lesions.

In the lepromatous type, open sores later appear on the face, earlobes, and forehead, with tests showing large numbers of bacilli in the discharge from these lesions. If progress of the disease is not checked by treatment, the fingers and toes disintegrate and there may be other disfiguring due to trauma to the insensitive extremities. Death may occur in extreme cases of this type, but more often it is due to a secondary infection, such as tuberculosis or pneumonia.

In the tuberculoid type, there is loss of sensation on sections of the skin and atrophy of muscles. This often results in contraction of the hand into a claw.

Leprosy is further classified as either paucibacillary or multibacillary according to whether there are fewer or more than five lesions or patches present.
Treatment. Leprosy is most effectively and inexpensively treated with sulfone medications, such as dapsone, developed around 1950. In cases of sulfone resistance, the drug clofazimine (Lamprone) may be prescribed. A semisynthetic antibacterial, rifampin, is very effective in killing leprosy bacilli rapidly, so that patients receiving it may be considered minimal public health risks within a few days after treatment is begun. However, these drugs are expensive, have serious side effects, and are not readily available in many countries.

Treatment continues for several years at least, and sometimes indefinitely. In addition to specific medical therapy, adequate rest, diet, and exercise are provided. Physical therapy is employed to retrain affected muscles. Psychiatric help, not only for leprosy patients but for their close contacts and those who only imagine they have been exposed, is invaluable in relieving the anxieties arising from the age-old misconceptions about the disease.
Prevention. Preventive measures include establishment of clinics and hospitals for diagnosis and treatment. Early diagnosis and prompt treatment with multidrug therapy are key to prevention. Many patients return to their homes completely free of symptoms and are able to resume normal lives. Cure has been most successful in cases that were diagnosed and treated at an early stage, especially among the young.

Among the public health measures used to prevent leprosy are the laws in most countries requiring that all cases be reported to the local authorities and that all discharged leprous patients be examined at six-month intervals. Most countries also refuse entry to immigrants known to be infected. In the United States, information about leprosy, as well as treatment, can be obtained from the Gillis Long Hansen's Disease Center, Carville, LA 70721, telephone 800-642-2477.

Hansen's disease


Hansen's disease

See leprosy.

Hansen's disease

Leprosy. An infection of the skin and the nerves, caused by the slowly replicating organism Mycobacterium leprae . Infectivity is low and the incubation period of the disease is from 2–5 years. Untreated Hansen's disease may cause loss of fingers and toes due to trauma to anaesthetized parts, and severe facial disfigurement and blindness. In the lepromatous form the body's immune reaction is poor and tissue destruction is great. In the tuberculoid form there is a good immune response and the disease is milder and non-infectious. Treatment with the drug dapsone is now less effective because of bacterial resistance and this has been replaced by drugs such as rifampicin, clofazimine and ethionamide, used in combination. Thalidomide is also valuable in the treatment of Hansen's disease.
Hansen's disease; leprosy chronic disease prevalent in tropical and warm/temperate regions, transmitted by inhalation of Mycobacterium lepra -contaminated aerosol droplets; characterized by Schwann-cell granuloma formation and significant sensory, autonomic and motor neuropathy; the patient presents initially with macular skin patches which later become atrophic, hypopigmented and insensate; superficial nerves eventually hypertrophy and become palpable, with significant neuropathy, trophic ulceration, and phalangeal and metatarsal resorption (see Table 1and Table 2); patient susceptibility to infection depends on gender (male:female ratio 2:1), genetic susceptibility and cell-mediated immunity (CMI); patients with high CMI tend to develop localized disease (tuberculoid leprosy), but patients with less CMI develop more serious and invasive forms of leprosy, including borderline and lepromatous forms; treatment includes antileprotic agents (dapsone, rifampicin and clofazimine); some cases respond to thalidomide
Table 1: Presentations of Hansen's disease (leprosy)
Leprosy typeCharacteristics
Tuberculoid leprosy (TT) or paucibacillary leprosy (PB)Vigorous host resistance and low infection
A localized disease in patients with high cell-mediated immunity characterized by a single, clearly demarcated hypopigmented anaesthetic skin area of the face, hands or feet, with thickening of the subserving nerve
Borderline tuberculoid leprosy (BT)As TT, but more numerous, smaller skin lesions with thickening of peripheral nerves and deformity of the hands and feet
Borderline leprosy (BB)Numerous skin lesions of varying size and form (macules, papules, plaques) with punched-out, hypopigmented anaesthetic centres; widespread nerve involvement and limb deformity
Borderline lepromatous leprosy (BL)Large number of florid asymmetrical skin lesions of variable form, strongly positive to acid-fast bacilli
Lepromatous leprosy (LL) or multibacillary leprosy (MB)Severely compromised host resistance and massive infection Skin changes of the face, earlobes, buttocks, upper and lower limbs, peripheral oedema, rhinitis and loss of the outer one-third of the eyebrows are characteristic of the early stages, with later mucous membrane involvement causing nasal stuffiness, laryngitis and hoarseness, thence nasal septum perforation and collapse of the nasal cartilages (saddle-nose deformity), glove-and-stocking anaesthesia, gynaecomastia, testicular atrophy, ichthyosis, nerve palsies and neurotrophic resorption of the phalanges. Lucio's phenomenon (endarteritis and ulceration) is noted in Mexico and Central America
Table 2: Lower-limb involvement in Hansen's disease
Disease effectClinical symptoms
Sensory anaesthesiaPainless trophic plantar ulceration along the lateral plantar border of the foot, of apical tissues, second metatarsophalangeal joint, pulp and interphalangeal joint area of the hallux
Motor paralysisIntrinsic muscle paralysis causing claw-toe formation and anterior drift of the plantar fat pad, with relative exposure of the plantar aspects of the metatarsophalangeal joints to abnormally high plantar pressures
Extrinsic muscle paralysis, i.e. paralysis of the anterior and peroneal muscles of the lower leg with footdrop and hindfoot inversion and the development of trophic ulcers along the lateral border of the foot
Autonomic neuropathyLoss of sweat gland function with dry skin and fissure
Compromised superficial and deep arterial circulation due to loss of control of arteriolar sphincters, so that, although the skin and superficial tissues are warm, skin perfusion is reduced and there is a greater than normal flow of blood through bone (bone hyperaemia) leading to osteoporosis, bone resorption and pathological fracture
Tarsal disintegrationInfiltration of the foot bones with bacilli, causing rarefaction of cancellous bone, fracture of tarsal bones followed by profuse recalcification with loss of normal bony architecture
Hyperaemia-related osteoclastic resorption of bone
Osteoporosis and osteomyelitisAbsorption of phalanges and pathological fractures of affected bones in neuropathic and immobile feet
References in periodicals archive ?
Sensing that a study with high quality and high-level of evidence would be welcome, we convened an expert group of peripheral nerve surgeons, reconstructive surgeons, and immunologists who have extensive experience with Hansen's Disease.
Through the current study, we sought to find the correlation between the behavior of Hansen's disease in its different clinical states and its evolution with respect to the ELISA method.
Hansen's disease is still, however, a significant problem in some parts of the world where treatments are not available (Britton, 1993; White, 2003; Wolf, 2000; WHO, 2008; Zewe, 1998).
Scientifically, leprosy is known as Hansen's disease, after Armauer Hansen who in 1873 discovered the bacillus Mycobacterium leprae, which causes the disease.
The museum has attracted more than 140,000 people, including elementary and junior high school students, so far, and it was reopened in April following expansion work as the National Hansen's Disease Museum.
Leprosy--a highly stigmatized disease that patients preferred to refer to by its more technical nomenclature, Hansen's disease, or, more cryptically, as "this package" or, in Cajun, as la maladie que tu nom pas (the disease you do not name)-- (4) affected only an estimated 1,500 to 5,000 people in the US.
Such unsubstantiated and erroneous ideas about the condition have led to the isolation and stigmatization of Hansen's disease patients and have made the term "leper" representative of a reviled outsider.
in addition to establishing a home for women with leprosy, or Hansen's disease, she started what is now the Maui Memorial Hospital, the first on the island.
Dietrich Varez, a Honolulu-based artist, has designed an aloha shirt for Hawaiian shirt maker Reyn Spooner that bears the image of Blessed Damien de Veuster, the Belgian-born missionary who served Hansen's disease patients on Molokai island more than a century ago.