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Ophthalmology > LID
Hansen Disease
Article Last Updated: Jun 22, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Eva C Kim, MD, Fellow in Ocular Inflammation/Uveitis, Francis I Proctor Foundation for Research in Ophthalmology, Department of Ophthalmology, University of California at San Francisco
Editors: Jack L Wilson, PhD, Distinguished Professor, Department of Anatomy and Neurobiology, University of Tennessee at Memphis; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal, and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Author and Editor Disclosure
Synonyms and related keywords:
leprosy, Hansen's disease, Mycobacterium leprae, M leprae
Background
Leprosy, also known as Hansen disease, is a chronic infectious disease with a wide range of clinical manifestations. It is a chronic granulomatous disease of the skin, mucous membranes, nerves, lymph nodes, eyes, and internal organs such as the liver, spleen, and testicles.
Pathophysiology
Hansen disease is caused by a bacillus, Mycobacterium leprae, first described by G. Amauer Hansen, MD, of Norway in 1874, that multiplies very slowly and mainly affects the skin, nerves, and mucous membranes. They have a predilection for the cooler areas of the body. This acid-fast rod has never been grown on artificial media or tissue culture, but, in 1960, Shepard successfully grew M leprae bacilli in mouse foot pads. Studies have shown that solid staining bacilli were infectious to mice, while beaded were not. Incubation in humans ranges from 6 months to 8 years, and it is believed to enter the body through the skin or mucous membranes of the upper respiratory tract. The extent of involvement and the type of manifestation after infection depend on the immune response of the host (see Media file 1) and may consist of complete resistance, no disease, incomplete resistance, and the indeterminate, which can progress to the other forms. The tuberculoid type affects skin and nerves but not internal organs and eye except occasional lagophthalmos. Few if any bacilli are present in the affected tissues. The tuberculoid type derives its name from the characteristic granuloma of tuberculosis consisting of giant cells, epithelioid cells, and lymphocytes. The lepromatous type can affect practically any organ, including skin and nerves. Usually, numerous bacilli are present and are characterized by a foam cell granuloma, often separated from the epidermis by a zone of normal connective tissue. The Lepromin test developed by Mitsuda 4 consists of an intradermal injection of 0.1 cc of a suspension of killed lepra bacilli. It is positive when a papule 6 mm or larger appears after 3 weeks at the injection site. This shows a strong immune response, suggesting the tuberculoid type. A negative test result means a poor or absent immune response, suggesting the lepromatous type. Diagnosis must be established on the basis of clinical and histologic criteria.
Frequency
United States
An estimated 500,000 new Hansen disease cases are identified each year, with 96 new cases reported to the Centers for Disease Control and Prevention (CDC) in the United States in 2002.
International
It is estimated that approximately 15 million persons in the world, mainly in the equatorial regions/tropical countries of Asia, Africa, and South America, have the greatest number of cases. Most cases come from 55 countries where the disease continues to be endemic. In 2002, the World Health Organization (WHO) listed those countries reporting the most cases (90%) as Brazil, Madagascar, Mozambique, Tanzania, and Nepal. In 2002, the number of new cases worldwide was 763,917.
Mortality/Morbidity
Worldwide, 1-2 million persons are estimated to be permanently disabled as a result of Hansen disease. Serous complications of leprosy are seen because of involvement of the peripheral nerves of the hands and feet leading to crippling contractures, trauma, and infections due to anesthetic areas of the body. The eyes frequently are affected with lagophthalmos, loss of eyebrows, corneal exposure, keratitis, uveitis, scleritis, leproma of the conjunctiva and sclera, retinal pearls, and loss of sensation with corneal ulceration with scaring. Amyloidosis of the kidney with death is a result of renal failure. Ocular manifestations occur primarily in the lepromatous form, and their age incidence is related to the duration of the disease, immune response of the host, and treatment.
Race
There are no statistics regarding racial variations.
Sex
The disease affects males more than females.
Age
Any age can be affected.
History
A complete careful eye examination is necessary to establish some of the subtle changes as described below.
- A typical tuberculoid skin lesion shows asymmetrical either hypopigmented sharply demarcated macules or reddish plaques, which spread in the periphery and heal in the center. The lesions may be circinate or gyrate and can spread to the entire extremity or trunk. Nerve involvement in tuberculoid leprosy occurs early, is unilateral, and can result in muscle atrophy with claw hand or foot drop. Enlarged great auricular nerves frequently are found.
- The skin lesions in lepromatous leprosy are nodules and hypopigmented macules. The papules and nodules are frequently copper colored, sometimes flat topped, and can reach considerable size and imitate skin tumors. Ear nodules and loss of the lateral part of the eyebrows are common.
- A diffuse form of lepromatosis, described by Lucio and frequently seen in the state of Sinaloa, Mexico, with numbness of the extremities is usually the only subjective complaint. The normal-appearing skin on biopsy results shows a mild inflammatory infiltrate in the perivascular location consisting of round cells with acid-fast stain showing numerous acid-fast bacilli cuffing vessels.
- Leprosy usually is a slow chronic process interrupted by reactive phases.
- In tuberculoid, one finds exacerbation of skin lesions and neuritis, which may be extremely painful.
- Erythema nodosum leprosum (ENL) is a frequent finding in lepromatous leprosy with lesions that may appear anywhere on the body including the face and are accompanied by fever, arthralgia, lymphadenopathy, and often neuritis. These patients are extremely toxic and can die; to control this reaction, first and second episodes may be treated with aspirin if mild or with short courses (1 wk) of prednisone 40-60 mg/day if significant, in addition to antimicrobials. For recurrent cases, thalidomide 100-400 mg/day is the drug of choice. However, because of its teratogenicity, thalidomide should not be given to women who may become pregnant. Adverse effects are mild constipation, mild leukopenia, and sedation.
- In diffuse lepromatous reactions, sharply demarcated erythematous lesions ulcerate, and after healing, leave scars. This is called erythema necroticans or Lucio phenomenon.
- A review of 237 patients at the USPHS Hospital San Francisco showed the following findings related to Hansen disease:
- Ocular lesions (237 patients)
- Corneal reduced sensation 22%
- Avascular keratitis 39%
- Pannus and interstitial keratitis 20%
- Corneal facets 7%
- Beading or opaque corneal nerves 47%
- Conjunctival or episcleral hyperemia 20%
- Leproma 1%
- Uveal lesions
- Iridocyclitis and synechia 6%
- Iris pearls 4%
- Glaucoma 3%
- Chorioretinal lesions 6%
- Number of patients - Angioid streaks 1, retinal pearls 3, retinal pigment epithelium mottling 2, uveal effusion 1, old chorioretinitis scars undetermined etiology 3, macular degeneration (pigmentary, age related, solar) 5, retinal detachment 1
- Adnexal lesions (237 patients)
- Loss of eyebrows or cilia 61%
- Eyelid and facial lesions
- Nodules 11%
- Erythema nodosum 11%
- Erysipeloid reaction 1%
- Entropion or ectropion 6%
- Lagophthalmos 6%
- Tearing 9%
- See Mortality/Morbidity.
Physical
Leprotic uveal infections
- Incidence of leprotic uveal infections is 6-7% of patients with Hansen disease at the PHS Hospital, San Francisco.
- Histopathology is completed by corneal, conjunctival, and subcutaneous skin scrapings; anterior chamber or vitreous taps; or skin and tissue biopsy. Findings show multiple acid-fast bacilli with acid-fast stains or Fite-Faraco stain (see Lab Studies).
- Manifestations
- Insidious chronic anterior uveitis (chronic plasmoid iridocyclitis) - Posterior synechia, keratic precipitates, iris pearls, complicated cataract, vitreous opacities, retinal detachment, secondary glaucoma, and phthisis bulbi
- Diffuse plastic reactions (acute diffuse plasmoid iridocyclitis)
- Isolated leproma
- Posterior uveal involvement (rare) - Retinal pearls, uveal effusion
- Diagnosis
- Typical systemic disease is confirmed by histopathology of corneal conjunctival, subcutaneous skin scrapings, anterior chamber or vitreous taps, or by skin tissue biopsy. Findings show multiple acid-fast bacilli with acid-fast stains or Fite-Faraco stain (see Lab Studies) and iris pearls.
- Leprotic punctate keratitis usually
- Iris pearls with uveitis (lesions are diagnostic): These represent miliary lepromas. Iris pearls are creamy round spheres usually less than .5 mm in diameter located near the papillary border, the chamber angle, or they may migrate and fuse with other pearls. They contain acid-fast material, remains of lepromatous cellular debris, and calcium salts. They form deep in the iris stroma and are extruded to the surface and may persist for months or years.
- Beading of corneal nerves
- Beading of corneal nerves (abnormally wide edematous nerves) is probably the earliest detectable ocular findings. These are sometimes difficult to detect and are seen best using slit lamp broad beam, retroillumination, or sclerotic scatter. This opacification and beading is due to the localization and multiplication of bacilli in or adjacent to nerves with or without infiltration of plasma cells, lymphocytes, and epithelioid cells.
- The corneal stroma reacts, and the cornea becomes covered with milky chalky deposits causing a keratitis pathognomonic of Hansen disease. These avascular punctate subepithelial opacities usually require a slit lamp examination for their detection. The lesions are seen between the epithelium and the Bowman membrane, and they later spread and become confluent. They are lepromas containing acid-fast organisms, lymphocytes, and macrophages, and some become calcified and can destroy the Bowman membrane.
- With progression of the disease, superficial and deep vascularization develops with reduced vision. In Carville PHS Hospital, 16% of the first ocular lesions noticed were episcleritis, scleritis, or uveitis. This type of episcleritis usually has early pain, tenderness and circumcorneal injection, and lack of exudates or bacteria. Late lepromas may develop in the episcleral area because this is a low temperature area. Bacilli are present in the ciliary body and pass through the sclera, episclera, conjunctiva, cornea, and also into the anterior chamber and iris.
- Leprous pannus usually develops in the superior limbus and later extends around the entire corneal circumference.
- The uveitis in Hansen disease may be a severe and prolonged course and with extensive anterior or posterior synechia. After repeated attacks, extensive iris atrophy and intractable glaucoma develop.
- Uveal effusion in association with conjunctival and episcleral hyperemia as well as retinal pearls rarely may occur.
- Lagophthalmos or unexplained seventh nerve weakness, associated with skin or neurologic disorders should be considered in the differential diagnosis of Hansen disease. Exposure keratitis and corneal ulceration also may occur and especially with decreased corneal sensation, thickened eyelids and incomplete blinking, decreased mucin and meibomian secretions, and denervation of the lacrimal gland.
Causes
M leprae causes Hansen disease.
Onchocerciasis
Trachoma
Uveitis, Anterior, Granulomatous
Other Problems to be Considered
Differential diagnosis also includes the following:
Reticulum cell lymphoma
Syphilis (juxta-articular nodes)
Lupus vulgaris
Lupus vulgaris (ulcerans)
Papulonecrotic tuberculid
Idiopathic hemorrhagic sarcoma of Kaposi
Filarial elephantiasis
Yaws
Paraffinoma (due to cosmetic injections of paraffin)
Cerebrotendinous xanthomatosis
Leishmaniasis
Discoid lupus erythematosus
Basal cell carcinoma
Tuberculosis colliquativa
Actinomyces mexicanus (Mycetoma)
Elastosis perforans serpiginosa
Folliculitis mucinosa
Tinea corporis
Tinea versicolor
Eczematoid dermatitis
Neurofibromatosis
Scleroma
Keratoderma sulcatum
Trachoma (scaring of tarsal conjunctiva)
Pinta
Vitiligo
Enlargement of great auricular nerves (sometimes seen in athletic young men, a physiologic variant)
Nummular eczema
Sarcoid
Histiocytosis X
Syringomyelia
Cervical rib syndrome
Lead poisoning
Unexplained facial nerve palsies
To miss the diagnosis of Hansen disease can cause a great hardship for the affected individual because it may allow progression of the disease and leave a potential contagious person in the midst of a family. The diagnosis of the disease when the person does not have it can cause great mental suffering and can cause hardship by depriving them of treatment for their true malady. This is why a tissue diagnosis or acid-fast stained smear always should be used to establish the diagnosis in those suspected of having Hansen disease.
Many of the patients with typical Hansen disease come from tropical areas of the world and may have various tropical diseases such as microfilaria along with other parasitic diseases as well as tuberculosis and syphilis.
Lab Studies
- Routine lab studies have little use in the diagnosis of Hansen disease but can help rule out other causes of some of the findings.
- Routine white blood counts and differential
- Serologic test for syphilis (STS) or Treponema pallidum agglutination (TPA) for syphilis
- Tuberculin/purified derivative protein (PPD) skin test
- Lepromin skin test is of little value diagnostically.
- Liver function test depending on drugs used to treat
Imaging Studies
- Chest x-ray or other lung screening imaging to check for tuberculosis (if later, it is necessary to use corticosteroids, eg, prednisone, systematically to control acute inflammatory reactions)
Procedures
- Biopsy specimens should be taken from the advancing edge of tuberculoid lesions because most normal-appearing skin will have pathologic changes. See Histologic Findings.
Histologic Findings
Tuberculoid biopsy specimens contain granulomas of lymphocytes, epithelioid cells, and foreign body giant cells. Occasionally, patients with tuberculoid form have only nonspecific chronic inflammation consisting of a scattered lymphocytic infiltration. Peripheral nerve damage may occur in sarcoidosis involving the skin, but actual nerve invasion resulting in axonal degeneration and occasionally caseous necrosis is pathonomic for Hansen disease. See Media file 2. Specimens of patients with leprosy should be taken from nodules or plaques, although pathologic changes will be found in normal-appearing skin. Skin biopsy specimens from patients with leprosy contain vacuolated macrophages (foam cells), few lymphocytes, and numerous acid-fast bacilli often in clumps or globi. These cells persist for years even after therapy.
Medical Care
- Prophylaxis with bacille Calmette-Guérin (BCG) vaccine or dapsone is only marginally effective and is not recommended.
- With the present treatment available, the sequelae can be minimized, but the deformities can be stigmatizing for the patient and their families. The disability aspect of this disease untreated is tremendous as stated earlier. It is beyond the scope of this article to discuss all of the facets of this treatment. It is highly recommended, if available, that a trained leprologist be included in the treatment of these patients. Special prosthetic devices are a valuable adjunct to help prevent ulcerations and deformities with resultant secondary infections.
- Ocular therapy depends on the manifestations. Aggressive treatment of iridocyclitis is probably one of the more important considerations as well as protecting anesthetic corneas from exposure, erosion, and ulceration in face of lagophthalmos. If corneal staining is demonstrated by fluorescein solution, frequent use of artificial tears (1% methylcellulose drops) or lubricating ointment. Moisture shields commercially available, Saran Wrap, or the use of swimming goggles to occlude the eyes. Taping the eyelids closed at night is also useful. Secondary glaucoma may be treated with Diamox (acetazolamide) to reduce intraocular fluid production and reduce intraocular tension. Sometimes, surgery is required as listed in Surgical Care.
- Plasmoid (plastic) iridocyclitis with anterior uveitis should be treated aggressively to avoid synechia, iris bombé, and other complications (see Medication).
Surgical Care
- Cataracts with reduction of vision, surgical extraction (The authors have elected to be conservative and not insert intraocular lenses because of the problems of repeat uveitis and synechia.)
- Iris bombé due to posterior synechia and glaucoma can be treated with sector iridectomies to help reduce intraocular pressure and improve vision in some patients with complicated cataracts or corneal opacities.
- Lagophthalmos due to seventh nerve palsy can be treated by a partial tarsorrhaphy or lateral canthoplasty or other lid-shortening procedures. Temporalis transfers are also useful in some patients.
- Eyebrow loss may be treated by makeup, tattooing, or free grafts from the scalp.
- Entropion or ectropion should be treated by appropriate surgical procedures.
- Trichiasis may be treated by repeated epilation or by electrolysis.
- With a painful blind eye due to glaucoma, enucleation may be the treatment of choice.
Consultations
It is strongly recommended that a leprologist be consulted if one is available.
Advice on diagnosis and treatment is available from the Gillis W. Long Hansen Disease Center in Carville, La (504-642-4755).
Drug Category: Antileprosy agents
In both forms of Hansen disease, dapsone is the mainstay of therapy. However, dapsone-resistant leprosy has been reported. Most patients with primary dapsone resistance demonstrate only partial resistance and respond to usual doses. The WHO recommends multiple drug therapy (MDT) for all forms of leprosy. MDT 14 consists of rifampin, ofloxacin, and minocycline. For MB (multibacillary, >5 skin lesions) in adults who cannot take rifampin, the committee recommends the daily administration of 50 mg of clofazimine with 400 mg of ofloxacin and 100 mg of minocycline for 6 months, followed by daily dose of 50 mg of clofazimine, together with 1000 mg of minocycline or 400 mg of ofloxacin for at least an additional 18 months. In the United States, where dapsone-resistance is rare, drug sensitivity testing in mice is recommended for all newly diagnosed and relapsed MB (lepromatous and borderline). For MB leprosy in adults, the WHO advocates dapsone 100 mg/d, clofazimine 50 mg/d plus 300 mg once monthly, and rifampin 600 mg once monthly. This regimen should be maintained for at least 2 years or until results of skin biopsies are negative (usually about 5 y). For paucibacillary leprosy (patients with tuberculoid leprosy without demonstrable AFB), the WHO recommends dapsone 100 mg/d and rifampin 600 mg once monthly for 6 months. Some recommend the treatment be extended to 1 year. In the United States, lepromatous leprosy is treated with dapsone 100 mg/d for life, with an initial 2-3 years regimen of rifampin 600 mg/d. World-known leprologist, Dr Paul Fasal of San Francisco, told the author (oral communication, June 1980) that if he was diagnosed with Hansen disease he would take rifampin for 1 year as the treatment of choice. Tuberculoid leprosy is treated with dapsone 100 mg/d for 5 years.
| Drug Name | Dapsone (Avlosulfon) |
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| Description | Sulfone that continues to be DOC for all patients infected with sulfone sensitive M leprae. Inexpensive and nontoxic in the usual dosage range. Peak serum levels that exceed the MIC by a factor of approximately 500 and has some bactericidal activity against leprosy bacillus is produced by 100 mg/d. Maximum dose should be used from the start of therapy. Can be used alone but usually used with antileprosy drugs (eg, rifampin, clofazimine). However, there seems to be an increase of sulfone resistant organisms and consideration should be given to combine with other antileprosy agents. |
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| Adult Dose | 50-100 mg/d PO |
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| Pediatric Dose | <2 years: Not established
2-6 years: 25 mg 3 times/wk
6-12 years: 25 mg/d
12-18 years: 50 mg/d |
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| Contraindications | Documented hypersensitivity; G-6-PD deficiency |
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| Interactions | May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during the second and third months of therapy); probenecid increases dapsone toxicity; trimethoprim with dapsone may increase toxicity of both drugs; due to increased in renal clearance, dapsone levels may decrease significantly when administered concurrently with rifampin |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Perform weekly blood counts (1st mo), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis is seen; caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M due to high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light; adverse effects include allergic dermatoses that can be severe and rarely, a syndrome including an exfoliative dermatitis, high fever, and mononucleosislike WBC differential (called the sulfone syndrome) |
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| Drug Name | Rifampin (Rimactane, Rifadin) |
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| Description | Primarily bactericidal for M leprae. In many developing countries, it is too expensive if given at the recommended dose. |
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| Adult Dose | 600 mg/d PO |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Induces microsomal enzymes, which may decrease effects of acetaminophen, oral anticoagulants, barbiturates, benzodiazepines, beta-blockers, chloramphenicol, oral contraceptives, corticosteroids, mexiletine, cyclosporine, digitoxin, disopyramide, estrogens, hydantoins, methadone, clofibrate, quinidine, dapsone, tazobactam, sulfonylureas, theophyllines, tocainide, and digoxin; blood pressure may increase with coadministration of enalapril; coadministration with isoniazid may result in higher rate of hepatotoxicity than with either agent alone (discontinue one or both agents if alterations in LFTs occur) |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Obtain CBCs and baseline clinical chemistries prior to and throughout therapy; in liver disease, weigh benefits against risk of further liver damage; interruption of therapy and high-dose intermittent therapy are associated with thrombocytopenia that is reversible if therapy is discontinued as soon as purpura occurs; if treatment is continued or resumed after appearance of purpura, cerebral hemorrhage or death may occur |
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| Drug Name | Clofazimine (Lamprene) |
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| Description | Inhibits mycobacterial growth, binds preferentially to mycobacterial DNA. Has antimicrobial properties but mechanism of action is unknown. Phenazine dye that is similar to dapsone in activity. |
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| Adult Dose | 50-100 mg/d PO tid (reduce if GI toxicity develops); 300 mg/d is moderately active against ENL-type reactions |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Dapsone may inhibit anti-inflammatory activity of clofazimine |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Severe abdominal symptoms may require exploratory laparotomies; caution in patients with GI problems (eg, abdominal pain, diarrhea); skin discoloration due to drug may result in depression or suicide; apply oil to skin for dryness and ichthyosis |
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| Drug Name | Minocycline (Minocin) |
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| Description | Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species. |
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| Adult Dose | 100 mg/d PO |
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| Pediatric Dose | <8 years: Not recommended
>8 years: Not established |
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| Contraindications | Documented hypersensitivity; severe hepatic dysfunction |
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| Interactions | Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants |
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| Pregnancy | D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
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| Precautions | Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last one half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines |
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| Drug Name | Ofloxacin (Floxin) |
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| Description | Pyridine carboxylic acid derivative with broad-spectrum bactericidal effect. |
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| Adult Dose | 400 mg PO qd |
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| Pediatric Dose | <18 years: Not recommended
>18 years: Administer as in adults |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Antacids, iron salts, and zinc salts may reduce serum levels; administer antacids 2-4 h before or after taking fluoroquinolones; cimetidine may interfere with metabolism of fluoroquinolones; ciprofloxacin reduces therapeutic effects of phenytoin; probenecid may increase ciprofloxacin serum concentrations; may increase toxicity of theophylline, caffeine, cyclosporine, and digoxin (monitor digoxin levels); may increase effects of anticoagulants (monitor PT) |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | In prolonged therapy, perform periodic evaluations of organ system functions (eg, renal, hepatic, hematopoietic); adjust dose in renal function impairment; superinfections may occur with prolonged or repeated antibiotic therapy |
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| Drug Name | Ethionamide (Trecator) |
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| Description | Bacteriostatic against Mycobacterium tuberculosis. |
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| Adult Dose | 250-500 mg/d PO |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; severe hepatic damage |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Make determinations of serum transaminase (AST, ALT) prior to therapy and q2-4wk thereafter; management of diabetes mellitus may be more difficult and hepatitis may occur more frequently |
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Drug Category: Immunomodulatory agents
Has effect in key factors of the immune system.
| Drug Name | Thalidomide (Thalomid) |
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| Description | Immunomodulatory agent that may suppress excessive production of tumor necrosis factor-a (TNF-a) and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration. DOC in ENL reactions, but it can cause teratogenicity and should not be given to pregnant women. |
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| Adult Dose | 100-400 mg/d PO qd |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | May increase sedation of alcohol, barbiturates, chlorpromazine, and reserpine; because of teratogenic effects, women must use 2 additional methods of contraception or abstain from intercourse |
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| Pregnancy | X - Contraindicated; benefit does not outweigh risk
|
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| Precautions | Perform pregnancy test within 24-h period prior to initiating therapy (weekly during first month, followed by monthly tests in women with regular menstrual cycles or q2wk with irregular menstrual cycles); bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); caution in liver impairment |
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Drug Category: Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Dexamethasone (AK-Dex, Decadron) |
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| Description | Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability. |
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| Adult Dose | Solution: 4 mg/mL subconjunctival injection |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; active bacterial or fungal infection |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Prolonged use may increase hazard of secondary ocular infection; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate) |
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| Drug Name | Prednisone (Deltasone, Pred Forte) |
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| Description | Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing increased capillary permeability.
Treats acute inflammations following eye surgery or other types of insults to eye. |
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| Adult Dose | Plasmoid iridocyclitis: 40-80 mg/d PO in conjunction with other antileprosy oral medications
Ophthalmic drops 1%: 1-2 gtt into conjunctival sac q30min initially; if cells and flare are reduced, then reduce to qid, later decreased as inflammation reduced
Episcleritis and scleritis: Ophthalmic drops qid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; viral, fungal, or tubercular infections |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Caution in hypertension; known to cause cataract formation with long-term use; suspect fungal invasion in any persistent corneal ulceration where a corticosteroid has been used or is in use (obtain fungal cultures when appropriate) |
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Drug Category: Anticholinergic agents
These agents are believed to work centrally by suppressing conduction in the vestibular cerebellar pathways. Control pupil size.
| Drug Name | Atropine 1% (Isopto) |
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| Description | Acts at parasympathetic sites in smooth muscle to block response of sphincter muscle of iris and muscle of ciliary body to acetylcholine, causing mydriasis and cycloplegia. |
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| Adult Dose | 1-2 gtt into conjunctival sac tid/qid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; thyrotoxicosis; narrow-angle glaucoma; tachycardia |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
|
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| Precautions | Avoid in patients with Down syndrome and/or children with brain damage to prevent hyperreactive response; avoid also in patients with coronary heart disease, tachycardia, congestive heart failure, cardiac arrhythmias, and hypertension |
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Drug Category: Adrenergic agonists
May decrease intraocular pressure.
| Drug Name | Phenylephrine (AK-Dilate, Neo-Synephrine) |
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| Description | Strong postsynaptic alpha-receptor stimulant with little beta-adrenergic activity that produces vasoconstriction of arterioles in the body. Phenylephrine (2.5% or 10% solution) concurrently with atropine, may prevent formation of synechiae by producing wide dilation of pupil. |
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| Adult Dose | Ophthalmic drops 10%: 1-2 gtt into conjunctival sac tid/qid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; severe hypertension or ventricular tachycardia |
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| Interactions | None reported |
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| Pregnancy | C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
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| Precautions | Caution in myocardial disease, bradycardia, partial heart block |
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Further Outpatient Care
- After patients are on rifampin for practical purposes they are no longer contagious to other people. However, they should not be lost to follow up to ensure they are complying with their medical regimen. Also, patients should receive follow-up care to see if they have developed sequelae and if they could benefit to changes in their therapy (eg, corneal exposure keratitis, secondary glaucoma, cataracts and recurrent uveitis, iridocyclitis).
- Household contacts should be checked for the disease; this is defined as anyone who has lived with the patient for at least 1 month since the onset of symptoms. It has been established that 20-50% of leprosy cases may be traced to household contacts. Family members are said to be 8 times more likely to develop lepromatous leprosy and 4 times more likely to develop tuberculoid leprosy. It is recommended that all family members be screened annually for at least 5 years.
In/Out Patient Meds
Complications
Prognosis
- Frequently tuberculoid forms are self-healing and may not have symptoms but may have resultant deformities. Lepromatous Hansen disease is progressive and if untreated, can produce blindness and be fatal. Because the response to therapy may vary with medications used, prognosis is variable. When chemotherapy is started early there will be fewer complications.
Medical/Legal Pitfalls
- The socioeconomic results of this diagnosis make it essential that histologic sections confirm the diagnosis.
- Be careful about the publication in the lay press of photos that could identify the patient or the family.
- Attempting to diagnosis this disease when the symptoms could be confused for others as in the differential diagnosis above.
- Concerns for other family members should not be neglected by failure to screen them for this disease.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors, Joseph B Michelson, MD, FACS, and James M Arrington, MD †, to the development and writing of this article.
| Media file 1:
Classification of Hansen disease after Paul Fasal, MD. |
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| Media file 3:
Hansen disease plasmoid (plastic) iridocyclitis. |
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Hansen disease corneal scraping acid-fast bacilli. |
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| Media file 7:
Hansen disease anterior chamber tap acid-fast bacilli globi. |
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| Media file 11:
Facial nodules of a patient with Hansen disease. |
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| Media file 12:
Lagophthalmos of a patient with Hansen disease. |
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| Media file 13:
Tuberculoid leg ulcer and biopsy site arm of a patient with Hansen disease. |
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| Media file 17:
Lagophthalmos seventh nerve of a patient with Hansen disease. |
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| Media file 18:
Hansen disease. Corneal scar seventh nerve exposure. |
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| Media file 19:
Hansen disease. Facial nodule and erythema nodosum leprosum. |
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| Media file 20:
Corneal neovascularization iris pearls of a patient with Hansen disease. |
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| Media file 21:
Avascular keratitis of a patient with Hansen disease. |
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| Media file 22:
Corneal neovascularization of a patient with Hansen disease. |
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| Media file 23:
Hansen disease. Corneal sensitivity determination with nylon monofilament. |
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| Media file 24:
Beaded corneal nerve of a patient with Hansen disease. |
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| Media file 25:
Hansen disease. Iris pearl avascular keratitis. |
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| Media file 26:
Temporal thin eyebrows of a patient with Hansen disease. |
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| Media file 27:
Cosmetic cover thin eyebrows of a patient with Hansen disease. |
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| Media file 28:
Thin eyebrows of a patient with Hansen disease. |
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| Media file 29:
Cataracts glaucoma synechia of a patient with Hansen disease. |
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| Media file 30:
Retinal scar uveal effusion of a patient with Hansen disease. |
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| Media file 31:
Retinal pearl of a patient with Hansen disease. |
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| Media file 32:
Nodular episcleritis of a patient with Hansen disease. |
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| Media file 33:
Episcleritis of a patient with Hansen disease. |
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| Media file 34:
Plasmoid (plastic) iridocyclitis of a patient with Hansen disease. |
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| Media file 35:
Purple skin from clofazimine of a patient with Hansen disease. |
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Hansen Disease excerpt Article Last Updated: Jun 22, 2006
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