Symptoms o Painless skin patch accompanied by loss of sensation but not itchiness (Loss of sensation is a feature of tuberculoid leprosy,

unlike lepromatous leprosy, in which sensation is preserved.) Chronic insensate patch is seen in the mage below.

o o o

Chronic insensate patch due to leprosy infection. Ho Chi Minh City, Vietnam. (Courtesy of D. Scott Smith, MD) Loss of sensation or paresthesias where the affected peripheral nerves are distributed Wasting and muscle weakness Foot drop or clawed hands (may result from neuritic pain and rapid peripheral

nerve damage; as seen in the image below) Characteristic clawed hand deformity caused by ulnar involvement in leprosy. Daloa, Ivory Coast. (Courtesy of D. Scott Smith, MD) Ulcerations on hands or feet (ulcer at the metatarsal head is seen in the image

below) Chronic nonhealing ulcer at the metatarsal head resulting from loss of sensation in the feet. Karigiri, Tamil Nadu, India. (Courtesy of Tara Ramachandra) o Lagophthalmos, iridocyclitis, corneal ulceration, and/or secondary cataract due to nerve damage and direct bacillary skin or eye invasion[7] Symptoms in reactions o Type 1 (reversal) - Sudden onset of skin redness and new lesions o Type 2 (erythema nodosum leprosum [ENL]; as seen in the image below) - Many skin nodules, fever, redness of eyes, muscle pain, and joint pain

Patient with erythema nodosum leprosum type 2 reaction several weeks after initiation of drug therapy. This photograph was taken after tendon release. Redwood City, California. (Courtesy of D. Scott Smith, MD) Travel: Leprosy should be considered in anyone who has lived in the tropics or who has traveled for prolonged periods to endemic areas. Exposure: The incubation period of leprosy is long, ranging from a few months to 20-50 years. The mean incubation time is estimated to be 10 years for lepromatous leprosy and 4 years for tuberculoid leprosy. The organism's slow dividing time (once every 2 wk) contributes to the challenge of epidemiologically linking exposures to the development of disease. Because of immunologic reasons, only around 5-10% of the population is estimated to be susceptible to infection.

Physical
The cardinal signs of leprosy include hypoesthesia, skin lesions, and peripheral neuropathy. The first physical signs of leprosy are usually cutaneous. The subtype of leprosy often determines the degree of skin involvement.

Physical examination should include the following: o Evaluation of skin lesions o Careful sensory and motor examination o Palpation of peripheral nerves for pain or enlargement, with particular attention paid to the following locations: Elbows - Ulnar nerve Wrist - Superficial radial cutaneous and median nerves Popliteal fossa - Common peroneal nerve Neck - Great auricular nerve Physical findings in specific leprosy subtypes include the following: o Tuberculoid leprosy The initial lesion is often a sharply demarcated hypopigmented macule that is ovoid, circular, or serpiginous. The lesions may be somewhat elevated with a dry scaly center and erythematous borders. Common lesion sites include the buttocks, face, and extensor surfaces of limbs. The perineum, scalp, and axilla are not normally involved because of the temperature differential in these zones, as predilection is toward cooler zones. As the disease progresses, lesions tend to destroy the normal skin organs such as sweat glands and hair follicles.

Superficial nerves that lead from the lesions tend to enlarge and are sometimes palpable. The patient may experience severe neuropathic pain. Nerve involvement can also lead to trauma and muscle atrophy. Lepromatous leprosy This form is characterized by extensive bilaterally symmetric cutaneous involvement, which can include macules, nodules, plaques, or papules. Multiple flat hypopigmented lesions are seen in the image below.

Multiple flat hypopigmented lesions on shoulder and neck, suggestive of multibacillary leprosy. Note ulceration of hypothenar area of hand, indicative of ulnar neuropathy. Redwood City, California, United States. (Courtesy of D. Scott Smith, MD) Unlike lesions in tuberculoid leprosy, those in lepromatous leprosy have poorly defined borders and raised and indurated centers. As in all forms of leprosy, lepromatous lesions are worst on cooler parts of the body. Common areas of involvement include the face, ears, wrists, elbows, buttocks, and knees. Hoarseness, loss of eyebrows and eyelashes, and nasal collapse secondary to septa perforation may occur in advanced cases of disease. Involvement of the eye may include keratitis, glaucoma, or iridocyclitis as seen in the image below.

Man with advanced deformities caused by unmanaged leprosy. Keratitis, loss of eyebrow, thickened skin, and typical hand impairments. Ho Chi Minh City, Vietnam. (Courtesy of D. Scott Smith, MD) The leonine facies associated with leprosy develop as the disease progresses, and the facial skin becomes thickened and corrugated. Axillary and inguinal adenopathy may develop, in addition to scarring of the testes and subsequent gynecomastia and sterility. Nerve involvement in lepromatous leprosy is not as severe as in tuberculoid leprosy, since nerves, although visibly thickened and highly infected, still function reasonably well in early stages of the disease. Borderline tuberculoid leprosy: The lesions are few or moderate and asymmetric with almost complete anesthesia. Peripheral nerves are often involved and thickened asymmetrically, and cutaneous nerves are sometimes enlarged.

Midborderline leprosy: The number of skin lesions is moderate, and they are asymmetrical and somewhat anesthetic. Peripheral nerves may be somewhat symmetrically enlarged, but cutaneous nerves are not. Borderline lepromatous leprosy: Moderate to numerous slightly asymmetrical skin lesions appear with minor or no anesthesia. Peripheral nerves are often enlarged symmetrically, but cutaneous nerves are not. Indeterminate leprosy: Skin lesions are typically either hypopigmented or hyperpigmented macules or plaques. Patients may note that these lesions are anesthetic or paresthetic.

Causes

M leprae is the causative agent associated with leprosy, which has been recognized as an infectious disease for the last 2 millennia. M leprae was discovered as the causative agent in 1873. The acid fast, gram-positive bacillus is an obligate intracellular organism with a predilection for Schwann cells and macrophages. M leprae has not been successfully grown using artificial media. The route of transmission has not been definitively established, although human-to-human aerosol spread of nasal secretions is thought to be the most likely mode of transmission in most cases. Leprosy is not spread by touch, since the mycobacteria are incapable of crossing intact skin. Living near people with leprosy is associated with increased transmission. Among household contacts, the relative risk for leprosy is increased 8- to 10-fold in multibacillary and 2to 4-fold in paucibacillary forms. Animal reservoirs do exist (armadillos, certain nonhuman primates), and cases of suspected zoonotic transmission have been reported.

How is leprosy diagnosed?

The majority of cases of leprosy are diagnosed by clinical findings, especially since most current cases are diagnosed in areas that have limited or no laboratory equipment available. Hypopigmented patches of skin or reddish skin patches with loss of sensation, thickened peripheral nerves, or both clinical findings together often comprise the clinical diagnosis. Skin smears or biopsy material that show acid-fast bacilli with the Ziehl-Neelsen stain or the Fite stain (biopsy) can diagnose multibacillary leprosy, or if bacteria are absent, diagnose paucibacillary leprosy. Other tests can be done, but most of these are done by specialized labs and may help a clinician to place the patient in the more detailed Ridley-Jopling classification and are not routinely done (lepromin test, phenolic glycolipid-1 test, PCR, lymphocyte migration inhibition test or LMIT). Other tests such as CBC test, liver function tests, creatinine test, or a nerve biopsy may be done to help determine if other organ systems have been affected.

Leprosy (Hansen's Disease) At A Glance

Leprosy is a slowly developing, progressive disease that damages the skin and nervous system. Leprosy is caused by an infection with Mycobacterium leprae or M. lepromatosis bacteria. Early symptoms begin in cooler areas of the body and include loss of sensation. Signs of leprosy are painless ulcers, skin lesions of hypopigmented macules (flat, pale areas of skin), and eye damage (dryness, reduced blinking). Later, large ulcerations, loss of digits, skin nodules, and facial disfigurement may develop.

The infection is thought to be spread person to person by nasal secretions or droplets. Leprosy is rarely transmitted from chimpanzees, mangabey monkeys, and nine-banded armadillos to humans by droplets or direct contact. Susceptibility to getting leprosy may be due to certain human genes. Antibiotics are used in the treatment of leprosy.

Pathophysiology: The areas most commonly affected by leprosy are the superficial peripheral nerves, skin, mucous membranes of the upper respiratory tract, anterior chamber of the eyes, and testes. These areas tend to be cooler parts of the body. Tissue damage is caused by the degree to which cell-mediated immunity is expressed, the extent of bacillary spread and multiplication, the appearance of tissue-damaging immunologic complications (ie, lepra reactions), and the development of nerve damage and its sequelae. M leprae is an obligate intracellular acid-fast bacillus with a unique ability to enter nerves.

Leprosy (Hansen's disease)

Last Reviewed: October 2011

What is leprosy?

Leprosy is a chronic bacterial disease of the skin and nerves in the hands and feet and, in some cases, the lining of the nose. Leprosy is a rare disease in the United States.

Who gets leprosy?

susceptible than adults.

Anyone can get leprosy, but children seem to be more

How is leprosy spread?

It is not clear how the leprosy germ is spread, but household and prolonged close contact is important. The germs probably enter the body through the nose and possibly through broken skin. The germs get in the air through nasal discharge of untreated lepromatous patients.

What are the symptoms of leprosy?

Tuberculoid leprosy symptoms are a few well-defined skin lesions that are numb. Lepromatous leprosy symptoms are a chronically stuffy nose and many skin lesions and nodules on both sides of the body.

How soon after exposure do symptoms appear?

It usually takes about four years for tuberculoid leprosy symptoms to appear and about eight years for lepromatous leprosy symptoms to appear.

When and for how long is a person able to spread leprosy?

In most cases, a person will not infect others within a day of beginning treatment with multidrug therapy.

What is the treatment for leprosy?

Patients with leprosy should be treated by a doctor who has experience with the disease. Treatment is with multiple drugs for six months to two years.

How can leprosy be prevented?

The best way to prevent the spread of leprosy is the early diagnosis and treatment of people who are infected. For household contacts, immediate and annual examinations are recommended for at least five years after last contact with a person who is infectious.