Dermatologic Manifestations of Leprosy Follow-up

  • Author: Felisa S Lewis, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 5, 2011
 

Further Inpatient Care

  • Patients with leprosy may need hospitalization for acute complications. Sanatoria, which were widely used in the past, are no longer necessary. Most patients can be treated in an outpatient setting.
  • Community-based rehabilitation programs are important to integrate and support people with leprosy and their families. A current list of Hansen disease clinics in the United States can be obtained from the National Hansen's Disease (Leprosy) Program.
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Further Outpatient Care

  • Follow-up to monitor post–multidrug therapy (MDT) reactions is no longer necessary because these reactions are rare. Nonetheless, prevention of disability and rehabilitation is important; therefore, suggested follow-up is 5-10 years after treatment is completed. Patients should be educated about the clinical signs of reoccurrence and should be instructed to return for an evaluation if they have any skin, eye, or nerve changes. Periodic assessments for neural impairment are recommended, and prompt treatment of reactions substantially reduces and prevents further damage and disability. Previous nerve involvement is predictive of further nerve function impairment.
  • Sensation and muscle strength in the hands, feet, and eyes should be checked on a regular basis. The eyes, nerves, and nose should be examined at follow-up to ensure timely recognition of reactivated disease.
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Deterrence/Prevention

  • No skin or serologic tests are available to identify a carrier of leprosy.
  • In the southern United States, close contact with armadillos should be avoided.[2]
  • Household contacts of patients with lepromatous disease should be monitored annually for 5 years after diagnosis.
    • Children especially should be observed for the development of disease.
    • In endemic countries, chemoprophylaxis may be useful in controlling leprosy. The Prospective (sero-)Epidemiological Study on Contact Transmission and Chemoprophylaxis in Leprosy (COLEP) found that a single dose of rifampin was 57% effective in preventing leprosy in contacts for the first 2 years after diagnosis of a new index case. However, the effect was not consistent in all subgroups. No statistical difference was noted between the rifampin group and the placebo group beyond 2 years. Therefore, no universal recommendation exists at this time, but it may be considered.[45]
    • In the United Kingdom, close contacts of lepromatous leprosy patients younger than 12 years are given rifampin at 15 mg/kg once a month for 6 months as prophylaxis.[46]
  • Attempts have been made to develop a vaccine against leprosy. Although not widely used, antileprosy vaccination can be immunoprophylactic and therapeutic. Current vaccines with various degrees of use are the BCG vaccine; the Mycobacterium w vaccine; the Mycobacterium avium-intracellulare complex (Mycobacterium ICRC) vaccine; and the BCG plus heat-killed Mycobacterium leprae, Mycobacterium tufu, and Mycobacterium habana vaccine.
    • The BCG vaccine has variable results in protecting certain populations; therefore, it is not widely prescribed. However, repeat immunization with the BCG vaccine may result in further protection. In the United Kingdom, the BCG vaccine is given to household contacts younger than 12 years.
    • In India, the Mycobacterium w and Mycobacterium ICRC vaccines are given. Mycobacterium w has a synergistic effect with chemotherapy, with accelerated clearing of the infection and shortening of treatment.
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Complications

  • Reactional states occur in approximately one third of patients and are acute inflammations of the disease. They may be induced by MDT, physical or mental stress, trauma, pregnancy, or surgical procedures. A leprous reaction should be considered a medical emergency and mandates immediate care. These states can result in permanent neurologic sequelae, resulting in disability and deformity. Patients at the highest risk are those with multibacillary leprosy and/or preexisting nerve impairment.
    • Lepra type I (reversal) reactions usually affect patients with borderline disease. Reversal reactions are a shift toward the tuberculoid pole after the start of therapy, and they are type IV cell-mediated allergic hypersensitivities, indicating an improvement in cell-mediated immunity. Puberty, pregnancy, and childbirth can also precipitate type I reactions.
      • These reactions usually result in skin erythema, with edema and tenderness of peripheral nerves. New skin lesions are common, and the patient may have an acute febrile illness. The peak time for type I reactions is during the first 2 months of therapy and for up to 12 months.
      • Corticosteroid treatment is aimed at controlling acute inflammation, relieving pain, and reversing nerve and eye damage. With treatment, approximately 60-70% of the patient's nerve function is recovered.[47] If neuritis is absent, NSAIDs may be helpful. MDT should be continued during type I reactions. Skin lesions have been successfully treated with the addition of topical tacrolimus.[48]
    • Lepra type II reactions, or erythema nodosum leprosum (ENL), occur in approximately 10% of patients with borderline lepromatous leprosy and in 20% of patients with lepromatous leprosy. These reactions are type III humoral (antibody-antigen) hypersensitivities, with a systemic inflammatory response to immune complex deposition. The most common presenting symptoms are crops of painful erythematous nodules of the skin and subcutaneous tissue. Sweet syndrome –like lesions have also occurred.[49] Bullae, ulcers, and necrosis also may occur.[50] Nerve damage is slower than in reversal reactions. The reaction usually manifests after a few years of therapy, and, although a single acute episode is possible, relapses occur intermittently over several years. Associated fever, malaise, arthralgias, neuralgia, iridocyclitis, dactylitis, orchitis, and proteinuria may be present.
      • The use of clofazimine in MDT substantially reduces the incidence of ENL to 5%. Clofazimine has also been used to treat ENL.
      • Thalidomide is effective except in the case of neuritis or iritis, in which case corticosteroids should be used.
      • Other treatment therapies reported to be effective include colchicine, pentoxifylline, cyclosporine A, intravenous immunoglobulin, infliximab,[51] and etanercept.[52]
      • Lowering the dose of dapsone may decrease the severity of bullae and ulcers.
    • Lucio phenomenon[53] is a cutaneous necrotizing vasculitis that is sometimes designated a type II reaction. It is common in Mexico and Central America and is characterized by erythematous, geometric, irregular-shaped macules that rapidly progress to ulceration and necrosis on acral areas or extremities of patients with diffuse lepromatous leprosy. Systemic symptoms such as hepatosplenomegaly, fever, arthritis, and nephritis are usually present. Thalidomide is ineffective in treating this type of reaction; however, no consensus on treatment had been determined.[54] Most patients with Lucio phenomenon have not received MDT or were treated irregularly; therefore, MDT is recommended. Azathioprine or cyclophosphamide with corticosteroids with or without plasmapheresis has also been used.
  • The real challenge in managing leprosy is the treatment of reactional states.
    • If the course of MDT is not complete, continue taking those medications as directed.
    • Systemic steroids are effective in reducing inflammation and edema in reversal reactions; therefore, they are the most helpful medications in preventing nerve damage.
    • Prednisone at 40-80 mg/d should be given for 5-7 days then tapered slowly over 3-6 months. This long course is necessary to decrease the severity of disabilities and deformities. One study recommended a low-dose (30 mg/d) regimen for 20 weeks for controlling type I reactions.
    • Clofazimine can also be used as a steroid-sparing agent for reversal reactions, alone or with corticosteroids.
    • Although the World Health Organization (WHO) does not support its use for ENL, thalidomide is highly effective with ENL. It is ineffective for the treatment of reversal reactions.
  • Neuropathy induced by leprosy can result in trauma, pressure necrosis, or secondary infection that goes unnoticed, leading to amputation of digits or limbs. Wrist and foot drop are also common. Silent neuropathy can occur in the absence of overt signs of nerve or skin inflammation. Even with corticosteroid treatment, only approximately 60% of nerve function is recovered. Cyclosporine A may be useful in controlling nerve impairment and pain.[55] Intraneural corticosteroid injection in one case resulted in nerve regeneration and improved sensory and motor function.[56] Tibialis posterior transfer by the interosseus route with early postoperative mobilization can correct foot drop in leprosy.[57, 58]
  • Injuries can result in ulcerations, cellulitis, scarring, and bony destruction. Foot ulcers discovered early should be treated with rest because they heal if they are not subject to weight bearing.
  • Osteoporosis and fractures can result from bony changes due to leprosy. Risedronate and other bisphosphates may help improve lumbar bone mineral density.[59]
  • Contractures can develop and may result in fixation. Common sequelae include clawing of hands and feet.
  • Arthritis/arthralgias may occur in approximately 10% of patients with leprosy and joint symptoms, and in conjunction with reactional states. Most have polyarticular symmetric arthritis. The arthritis is generally not responsive to conventional therapy, but 50% become asymptomatic within 24 months of diagnosis.[60]
  • Eye damage, especially in the anterior portion of the eye, can result in loss of the corneal reflex, lagophthalmos, ectropion, entropion, and blindness. One study found the risk of ocular complications in patients with multibacillary disease, after completion of MDT, to be 5.6%, with eye-threatening complications at 3.9%.
  • Skin drying and fissures can be caused by autonomic disruption.
  • Hypogonadism and testicular atrophy can lead to sterility and gynecomastia. Testosterone replacement is the treatment of choice.
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Prognosis

  • The prognosis depends on the stage of disease. Borderline tuberculoid leprosy usually involves rapid and severe nerve damage. Reversal reactions are uncommon with lepromatous disease; therefore, lepromatous leprosy is a chronic state with long-term complications. Even with MDT, patients have long-term nerve damage and disability.
  • The prognosis also depends on the patient's access to therapy, the patient's compliance, and the early initiation of treatment.
  • Relapse (new disease after adequate MDT is completed) occurs in 0.01-0.14% of patients per year in the first 10 years. Dapsone and/or rifampin resistance should be considered.[61]
  • Approximately 5-10% of patients have a type I reversal reaction in the first year after completing MDT.
  • Because of reduced cell-mediated immunity, pregnancy can precipitate a relapse or reaction of the disease, especially type II reactions in pregnant women younger than 40 years. Dapsone is generally thought to be safe in pregnancy; the safety of clofazimine and rifampin are controversial, and thalidomide (used in type II reactions) is contraindicated during pregnancy.
  • Type I and type II reactions can precipitate a relapse of the disease.
  • Perineural granulomas have been reported to persist 18 months after MDT and clinical improvement, and they are not considered to be a relapse of the disease.[62]
  • Overall, children have a good prognosis because multibacillary disease and leprous reactions are uncommon.
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Patient Education

  • Patients first need an explanation of the diagnosis and prognosis.
    • Their fears should be addressed because of the cultural stigma associated with leprosy. Importantly, refute any myths that the patient may have about leprosy.
    • Patients may need psychological counseling because they may have difficulty coming to terms with the disease or may feel rejected by society.
    • The patient should be reassured that within a few days of starting MDT, they are not infectious and can lead a normal life.
  • Patients need education about how to deal with anesthesia of a hand or foot.
    • They must learn to carefully inspect their extremities for trauma each day. Patients should also be told to wear proper footwear and protective equipment as necessary. Inexpensive canvas shoes with protective insoles are as effective as special orthopedic shoes.
    • Inspecting limbs and eyes for the onset of anesthesia or weakness is also important.
    • Physical therapy and occupational therapy are important tools in rehabilitation.
  • Patients must learn how to recognize the onset of lepra reactions, and they should be told to seek immediate medical attention if these reactions develop.
  • Potential deformities can be prevented by educating patients about how to deal with existing nerve damage and by treating any sequelae of this damage.
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Contributor Information and Disclosures
Author

Felisa S Lewis, MD  Staff Physician, Department of Dermatology, Madigan Army Medical Center

Felisa S Lewis, MD, is a member of the following medical societies: American Academy of Dermatology, International Society of Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Theresa Dressler Conologue, DO, FAAD  Physician, Department of Dermatology, Geisinger Medical Center

Theresa Dressler Conologue, DO, FAAD is a member of the following medical societies: American Academy of Cosmetic Surgery, American Academy of Dermatology, and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Elyse Harrop, MD  Clinical Instructor, Department of Dermatology, Metrohealth Medical Center

Elyse Harrop, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Terry L Barrett, MD  Clinical Professor of Dermatology and Pathology, University of Texas Southwestern School of Medicine; Director, ProPath Dermatopathology, Dallas, Texas

Terry L Barrett, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, College of American Pathologists, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Lester F Libow, MD  Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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