II. Epidemiology

  1. Children more vulnerable to infection than adults
  2. Immunity in 95% of patients
  3. Endemic areas
    1. Asia
    2. Africa
    3. Latin America
    4. United States (rare except in a few states)
      1. Texas
      2. Louisiana
      3. Hawaii
      4. California

III. Etiology

  1. Mycobacterium leprae (acid fast bacilli)

IV. Pathophysiology

  1. Chronic granulomatous infection
  2. Incubation: 3 to 20 years
  3. Growth best in cooler parts of body at 35.6C (96.0F)
  4. Transmission
    1. Nasal secretions from untreated patients
    2. Low risk from casual and household contact

V. Types

  1. Tuberculoid Leprosy (intact cellular immunity)
    1. Nerve changes predominate
  2. Lepromatous Leprosy (defective cellular immunity)
    1. Skin changes predominate
    2. Extensive bilateral symmetric Macules and Papules
    3. High Bacterial load

VI. Signs

  1. Skin lesions
    1. Types
      1. Macules or Papules, Nodules or Plaques
      2. Hypopigmented in dark skinned individuals
      3. May be hyperpigmented in other patients
      4. Sensation may be dulled in these areas
      5. Loss of lateral eyebrows
    2. Sites
      1. Face and ears
      2. Wrists
      3. Buttocks
      4. Knees
    3. Spared areas
      1. Groin
      2. Axilla
      3. Hair covered scalp
  2. Nose and Throat changes
    1. Nasal symptoms to obstruction
    2. Laryngitis
    3. Hoarseness
  3. Neurologic changes
    1. Nerve tuberculoid changes (may be palpable)
      1. Ulnar Nerve
      2. Peroneal nerve
      3. Greater auricular nerve
    2. Muscle atrophy
    3. Contractures
  4. Eye changes
    1. Corneal Ulcerations
    2. Blindness

VII. Complications

  1. Crippling of hand (Worldwide most frequent cause)

VIII. Differential Diagnosis

IX. Labs

  1. VDRL false positive (10-20%)

X. Diagnosis

  1. Acid-fast stain (Fite method) for acid-fast bacilli
    1. Skin smears, or skin or nerve biopsy
    2. Bacteria abundant in Lepromatous Leprosy
  2. Skin lesion biopsy
    1. Tuberculoid Leprosy
      1. Epitheliod granulomas
      2. Numerous peripheral Lymphocytes
    2. Lepromatous Leprosy
      1. Macrophages with foamy cytoplasm
  3. Serology
    1. Lepromatous (95% sensitive)
    2. Tuberculoid (30% sensitive)

XI. Management

  1. Tuberculoid, Borderline Tuberculoid
    1. Dapsone 50-100 mg qd for 24-30 months AND
    2. Rifampin 600 mg qd for 6-12 month
  2. Borderline Lepromatous
    1. Dapsone 50-100 mg qd for 24 months AND
    2. Rifampin 600 mg qd for 24 months
  3. Lepromatous (may require indefinate treatment)
    1. Dapsone 50-100 mg qd for 24 months AND
    2. Rifampin 600 mg qd for 24 months AND
    3. Clofrazamine 50-200 mg qd for 24 months

XII. References

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Ontology: Leprosy (C0023343)

Definition (MSHCZE) Chronická granulomatózní infekce vyvolaná MYCOBACTERIUM LEPRAE. Granulomatózní léze se projevují na kůži, sliznici a periferních nervech. Lepra má dvě základní formy - lepromatózní (nervová) a tuberkuloidní (kožní neboli uzlová).
Definition (NCI) A bacterial granulomatous infection caused by Mycobacterium leprae. It is a progressive disease affecting the skin, peripheral nerves, and limbs. If untreated, it causes permanent tissue damage leading to autoamputations.
Definition (MSH) A chronic granulomatous infection caused by MYCOBACTERIUM LEPRAE. The granulomatous lesions are manifested in the skin, the mucous membranes, and the peripheral nerves. Two polar or principal types are lepromatous and tuberculoid.
Definition (CSP) chronic granulomatous infection caused by Mycobacterium leprae; granulomatous lesions are manifested in the skin, the mucous membranes, and the peripheral nerves; two polar or principal types are lepromatous and tuberculoid.
Concepts Disease or Syndrome (T047)
MSH D007918
ICD9 030.9, 030
ICD10 A30.9 , A30
SnomedCT 187316006, 186339006, 154298007, 266184007, 81004002
English Disease, Hansen, Hansen Disease, Hansen's Disease, Hansens Disease, Leprosies, Disease, Hansen's, Leprosy NOS, Leprosy, unspecified, [X]Leprosy, unspecified, HANSEN DIS, HANSENS DIS, leprosy, leprosy (diagnosis), Mycobacterium leprae infection NOS, infection due to Mycobacterium leprae, Leprosy [Hansen's disease], Leprosy [Disease/Finding], hansen disease, hansen's disease, Disease;Hansens (Leprosy), hansens disease, leprosy nos, Leprous infections, Leprosy NOS (disorder), [X]Leprosy, unspecified (disorder), Hansen's disease, Infection due to Mycobacterium leprae, Mycobacterium leprae infection, Leprosy (disorder), Hansen, Leprosy, NOS, Leprosy, Hansens (leprosy) disease
Italian Infezioni leprose, Malattia di Hansen, Lebbra, non specificata, Lebbra NAS, Infezione da Mycobacterium leprae NAS, Morbo di Hansen, Lebbra
Dutch lepra, niet-gespecificeerd, Mycobacterium leprae-infectie NAO, ziekte van Hansen, lepra NAO, Lepra, niet gespecificeerd, lepra-infecties, lepra, Lepra [ziekte van Hansen], Hansen-ziekte, Lepra, Ziekte van Hansen
French Lèpre, non précisée, Lèpre SAI, Infection à Mycobacterium leprae SAI, Infections lépreuses, Lèpre, Maladie de Hansen
German Mycobacterium leprae Infektion NNB, Lepra NNB, Lepra, unspezifisch, Lepra [Aussatz], Lepra, nicht naeher bezeichnet, Leprainfektionen, Aussatz, Hansen-Krankheit, Lepra
Portuguese Lepra NE, Infecção a Mycobacterium leprae NE, Lepra, Infecções leprosas, Doença de Hansen, Hanseníase
Spanish Lepra NEOM, Infección por Mycobacterium leprae NEOM, Lepra no especificada, lepra, SAI, lepra, SAI (trastorno), [X]lepra, no especificada (trastorno), [X]lepra, no especificada, Mal de Hansen, enfermedad de Hansen, infección por Mycobacterium leprae, lepra (trastorno), lepra, Infecciones por Mycobacterium leprae, Lepra, Enfermedad de Hansen
Japanese ハンセン病、詳細不明, ハンセン病NOS, らい菌感染NOS, らい菌感染, ライキンカンセン, ライキンカンセンNOS, ハンセンビョウショウサイフメイ, ハンセンビョウ, ハンセンビョウNOS, Hansen病, らい, らい病, ハンセン病, レプラ, , 癩病, ハンセン氏病
Swedish Spetälska
Czech lepra, Lepra NOS, Lepra, Hansenova nemoc, Infekce Mycobacterium leprae NOS, Lepra, blíže neurčená, Infekce způsobené Mycobacterium leprae
Finnish Lepra
Russian PROKAZA, GANSENA BOLEZN', LEPRA, ГАНСЕНА БОЛЕЗНЬ, ЛЕПРА, ПРОКАЗА
Korean 나병[한센병], 상세불명의 나병
Croatian LEPRA
Polish Choroba Hansena, Trąd
Hungarian lepra k.m.n., Hansen-betegség, lepra, nem meghatározott, lepra, Mycobacterium leprae fertőzés k.m.n., Leprás fertőzések
Norwegian Hansens sykdom, Spedalskhet, Lepra