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Hansen Disease: Treatment & Medication
Updated: Jun 22, 2006
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
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Treatment
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.
Medication
Advice on diagnosis and treatment is available from the Gillis W. Long Hansen Disease Center in Carville, La (504-642-4755).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.
Dapsone (Avlosulfon)
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.
Adult
50-100 mg/d PO
Pediatric
<2 years: Not established
2-6 years: 25 mg 3 times/wk
6-12 years: 25 mg/d
12-18 years: 50 mg/d
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
Documented hypersensitivity; G-6-PD deficiency
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
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)
Rifampin (Rimactane, Rifadin)
Primarily bactericidal for M leprae. In many developing countries, it is too expensive if given at the recommended dose.
Adult
600 mg/d PO
Pediatric
Not established
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)
Documented hypersensitivity
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
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
Clofazimine (Lamprene)
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.
Adult
50-100 mg/d PO tid (reduce if GI toxicity develops); 300 mg/d is moderately active against ENL-type reactions
Pediatric
Not established
Dapsone may inhibit anti-inflammatory activity of clofazimine
Documented hypersensitivity
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
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
Minocycline (Minocin)
Treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species.
Adult
100 mg/d PO
Pediatric
<8 years: Not recommended
>8 years: Not established
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
Documented hypersensitivity; severe hepatic dysfunction
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
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
Ofloxacin (Floxin)
Pyridine carboxylic acid derivative with broad-spectrum bactericidal effect.
Adult
400 mg PO qd
Pediatric
<18 years: Not recommended
>18 years: Administer as in adults
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)
Documented hypersensitivity
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
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
Ethionamide (Trecator)
Bacteriostatic against Mycobacterium tuberculosis.
Adult
250-500 mg/d PO
Pediatric
Not established
None reported
Documented hypersensitivity; severe hepatic damage
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
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
Immunomodulatory agents
Has effect in key factors of the immune system.
Thalidomide (Thalomid)
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.
Adult
100-400 mg/d PO qd
Pediatric
Not established
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
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
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
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
Dexamethasone (AK-Dex, Decadron)
Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Adult
Solution: 4 mg/mL subconjunctival injection
Pediatric
Not established
None reported
Documented hypersensitivity; active bacterial or fungal infection
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
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)
Prednisone (Deltasone, Pred Forte)
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.
Adult
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
Pediatric
Not established
None reported
Documented hypersensitivity; viral, fungal, or tubercular infections
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
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)
Anticholinergic agents
These agents are believed to work centrally by suppressing conduction in the vestibular cerebellar pathways. Control pupil size.
Atropine 1% (Isopto)
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.
Adult
1-2 gtt into conjunctival sac tid/qid
Pediatric
Not established
None reported
Documented hypersensitivity; thyrotoxicosis; narrow-angle glaucoma; tachycardia
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
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
Adrenergic agonists
May decrease intraocular pressure.
Phenylephrine (AK-Dilate, Neo-Synephrine)
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.
Adult
Ophthalmic drops 10%: 1-2 gtt into conjunctival sac tid/qid
Pediatric
Not established
None reported
Documented hypersensitivity; severe hypertension or ventricular tachycardia
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
Precautions
Caution in myocardial disease, bradycardia, partial heart block
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| Differential Diagnoses & Workup: Hansen Disease |
 Treatment & Medication: Hansen Disease |
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References
Allen JH, Beyers JL. The pathology of ocular leprosy I. Cornea Arch Ophth. 1960;64:216.
American Medical Association (AMA). AMA Drug Evaluations. 6th ed. Littleton, Mass: Publishing Sciences Group Inc; 1986:1548.
Centers for Disease Control and Prevention. Hansen disease. Centers for Disease Control and Prevention. Available at http://www.cdc.gov/ncidod/dbmd/diseaseinfo/hansens_t.htm. Accessed 2000.
Elliott DC. An interpretation of the ocular manifestations of leprosy. Ann N Y Acad Sci. 1951;54:84-100.
Fasal P. A primer of leprosy. Cutis. 1971;7:525-534.
Fasal P. But it was not leprosy. Cutis. 1975;15:499-509.
Fasal P. Histopathology of leprosy: a tribute to Kensuke Mitsuda (1876-1964). Cutis. Jul 1976;18(1):66-72. [Medline].
Gelber RH. Leprosy. In: The Merck Manual. 17th ed. Merck & Company; 1999:1206-1209.
Hendrick SS, Wilkin JK. Leprosy. Am Fam Physician. Nov 1982;26(5):161-6. [Medline].
Holmes WJ. Leprosy of the eye. Tr Am Ophth Soc. 1957;55:145-187.
Johnson HA. A modification of the gilles, temporalis transfer for the surgical treatment of the lagophthalmos of leprosy. Plast Reconst Surg. 1962;30:378.
Masters FW, Robinson DW, Simons JN. Temporalis transfer for lagophthalmos due to seventh nerve palsy. Am J Surg. Oct 1965;110(4):607-11. [Medline].
Michelson JB, Roth AM, Waring GO 3rd. Lepromatous iridocyclitis diagnosed by anterior chamber paracentesis. Am J Ophthalmol. Oct 1979;88(4):674-9. [Medline].
Richards WW, Arrington JM. Unsuspected ocular leprosy. Am J Ophthalmol. Sep 1969;68(3):492-9. [Medline].
Shepard CC. The experimental disease that follows the injection of human leprosy bacilli into footpads of mice. Jour Exp Med. 1960;112:445.
Veterans Administration. Medical Bulletin MB-10: Hansen Disease. Veterans Administration; 1965.
World Health Organization (WHO). WHO Expert Committee on Leprosy. 7th Report. World Health Organization (WHO). Available at http://www.who.int/en/. Accessed 1998.
Further Reading
Keywords
leprosy, Hansen's disease, Mycobacterium leprae, M leprae
