Leprosy (overview)A30.9

Authors:Prof. Dr. med. Peter Altmeyer, Ramona Tasar

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Last updated on: 03.07.2021

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Synonym(s)

Hanseniasis; Hansen M.; Hansenosis; Hansen`s disease; Hansen's disease; leprosy; Zaraath (bibl.)

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HistoryThis section has been translated automatically.

Hansen, 1874

DefinitionThis section has been translated automatically.

Important, chronic granulomatous infectious disease caused by Mycobacterium leprae and Mycobacteriumlepromatosis(see belowMycobacterium leprae), with preferential infestation of skin and peripheral nerves and of low contagiousness. While leprosy no longer plays a role in industrialized countries, it is still of great medical importance in developing countries. Infection and manifestation of the disease occurs mainly in populations with malnutrition, poor general health and immunodeficiencies. Leprosy is still a stigmatising disease; sufferers still have to fear exclusion from the community and family.

Reminder. Obligation to report!

PathogenThis section has been translated automatically.

Mycobacterium leprae (immobile, gram-positive, acid-fast rods). The pathogens are often arranged intracellularly in bundles. Very slow growth.

ClassificationThis section has been translated automatically.

Classification based on immunological, clinical, bacteriological and histological findings (see also Table 1). A distinction is made between:

Leprosy indeterminata

Leprosy lepromatosa - LL- (pathogen-rich, anergic, spots, papules, plaques and nodules)

Borderline leprosy

  • Borderline leprosy lepromatosa -BL- (pathogen-rich, plaques and spots, tendency to symmetry of lesions)
  • Mid Borderline Leprosy lepromatosa -BB- ( pathogen-rich, asymmetry of foci)
  • Boderline leprosy tuberculoides - BT- (pathogen-poor, asymmetrical, few plaques, satellites)

Leprosy tuberculoides -TT- (no pathogens detectable, 1-5 sharply demarcated plaques, hypopigmented, asymmetric).

Intermediate le prosy -I- (Intermediate leprosy; 1 or few, hypopigmented, patches no plaques).

Therapeutic relevance is the determination of the microscopic bacterial quantity or density, e.g. by nasal swab. The bacterial index (bacterial density per field of view) is classified in the following way:

  • Index 6: more than 1000 bacteria per field of view (Gf.),
  • Index 5: between 100 and 1000 bacteria per gf,
  • Index 4: between 10 and 100 bacteria per gf,
  • Index 3: between 1 and 10 bacteria per gf,
  • Index 2: between 1 and 10 bacteria per 10 gf,
  • Index 1: between 1 and 10 bacteria per 100 gf,
  • Index 0: 0 bacteria in 100 gf.

Occurrence/EpidemiologyThis section has been translated automatically.

In 2015, 211,973 new cases of leprosy were diagnosed worldwide. Currently, most new cases (>96%) were registered in the 22 high-risk countries (lepra priority countries) (WHO Fact Sheets 2017). Leprosy remains a disease of poverty rather than a disease of tropical or subtropical countries (endemic areas: India, Brazil, Bolivia, Indonesia, South Sudan, Sierra Leone). Malnutrition, poor nutrition, poor AZ, immunodeficiency, favour its outbreak.

Distribution: In a larger Indian collective (n=220), the following distribution was found:

  • Multibacillary leprosy: w>m. It was more prevalent in villagers (80.9%) than in urban dwellers (64.8%).
  • Borderline lepromatous leprosy (38.2%)
  • Lepromatous leprosy (28.2%)
  • Borderline tuberculoid leprosy (21.4%) (Arif T et al 2019).

EtiopathogenesisThis section has been translated automatically.

Infection from person to person by droplet infection (high bacterial density in the area of the nasal mucosa), also transmission from skin to skin, via objects of daily life and breast milk. Transmission of the pathogen depends on the immune status and the leprosy form. In leprosy lepromatosa and dimorphic leprosy there is a risk of infection. Transmission through blood-sucking arthropods is considered possible.

ManifestationThis section has been translated automatically.

The average age in a larger Indian study (Arif T et al. 2019) was between 20 and 40 years. Childhood or adolescence may also be affected.

Clinical featuresThis section has been translated automatically.

Incubation period: 3 to about 20 years; uncharacteristic early symptoms.

Skin symptoms: leprosy lepromatosa, leprosy tuberculoides, leprosy indeterminata, dimorphic leprosy. Depigmentation or hypopigmentation of the skin.

Sensitivity disorders: hyperaesthesia, paraesthesia, hypaesthesia, anaesthesia. Thickening of nerves, often of the ulnar nerve.

DiagnosisThis section has been translated automatically.

Sensory disturbances: Disturbance of the sensation of temperature, pain and touch.

Pathogen detection:

  • Direct: smear of skin (forehead, auricle, trunk, extensor sides of extremities) and nasal mucosa, skin or nerve biopsies, Faraco-Fite stain; Ziehl-Neelsen stain.
  • Cultural: breeding only by transfer to animals, esp. mice, armadillos, chipmunks, hedgehogs. Altogether difficult and not very promising.

Lepromin reaction: For individual leprosy classification and prognosis.

Histamine test: Intracutaneous application of histamine - lack of reflex erythema.

Sweat test: Absence of sweat secretion in lepromatous foci; see also Minor sweat test.

PGL I antibody: Detection of specific antibodies against PGL I antigen.

General therapyThis section has been translated automatically.

Isolation and shielding of the sick from the outside world in their own hospitals is obsolete!

Internal therapyThis section has been translated automatically.

Recommended (WHO therapy recommendations) are combination therapies with dapsone (DADPS) (e.g. Dapsone Fatol), rifampicin (e.g. Eremfat) and, if necessary, clofazimine (e.g. Lampren), see Table 2. Therapy with dapsone is started in a gradual dosage: initial 25 mg/week, slowly increasing over several months to 100 mg/day. In cases of neuronal involvement, glucocorticoids in medium dosages such as prednisolone 60-80 mg/day (e.g. Decortin H) are helpful.

Remember! It is important to monitor and treat secondary infections, as these are often the cause of the death of leprosy patients!

  • Pregnancy: Leprosy bacteria reach the unborn child via the placenta. Therefore, treatment must be carried out in any case during pregnancy. Teratogenic effects of the leprosy medication are not yet known. Dapsone, rifampicin as well as clofazimine are placental.
  • Rehabilitation: Subsequent rehabilitation measures according to the clinical symptoms of the patient, such as plastic reconstructive surgery and physiotherapy.

Progression/forecastThis section has been translated automatically.

More favourable in tuberculoid leprosy,The immune status of the patient is decisive for the course of leprosy! In poor immune status, there is a strong multiplication of pathogens; development of leprosy lepromatosa with unfavourable prognosis. Favourable prognosis in tuberculoid leprosy. Further impairment by secondary infections.

ProphylaxisThis section has been translated automatically.

Clarification. Since the contagiousness is low and the antibiotic therapy is successful, strict isolation of the sick is no longer required today. It is important to improve the hygienic conditions and to check contact persons regularly (every 6 months). If necessary, a single administration of rifampicin after exposure can suppress the manifestation of a disease.

TablesThis section has been translated automatically.

Characteristics of the leprosy forms

TT

BT

BB

BL

LL

skin lesions

Quantity

individual

few

moderate

many

a great many

Symmetry

-

-

-

±

+

Nerve lesions

Anaesthesia

stresses

stresses

moderate

weak

hardly

Thickening

++

+

+

±

±

Immune status

well

medium

bad

Bacteria Index

0

1-2 +

2-4 +

3-5 +

5-6 +

Lepromin test

+++

±

-

-

-

Leprosy Reaction

Type I

0

+

++

+

0

type II

0

0

0

+

++

Leprosy treatment

Dapson

Rifampicin

Clofazimine

Therapy duration

Low bacterial density (index 1; leprosy tuberculoides, dimorphic leprosy)

100 mg/day (1-2 mg/kg bw/day)

600 mg once a month

Over at least 6 months

High bacterial density (leprosy lepromatosa, leprosy indeterminata)

100 mg/day (1-2 mg/kg bw/day)

600 mg once a month

50-100 mg/day (1-2 mg/kg bw/day)

Over 2 years or until negative bacterial smear

For dapson resistance or intolerance

600 mg once a month for 3 years

50-100 mg/kg bw/day

Over at least 10 years

As an alternative to clofazimine, ethionamide may be given at 250 mg/day (for an indefinite period)

LiteratureThis section has been translated automatically.

  1. Abulafia J et al (2001) Leprosy: accessory immune system as effector of infectious, metabolic, and immunologic reactions. Int J Dermatol 40: 673-687
  2. Arif T et al (2019) Leprosy in the post-elimination era: a clinico-epidemiological study from
    anorthern Indian tertiary care hospital. Acta Dermatovenerol Alp Pannonica Adriat 28:7-10.
  3. de Almeida Junior HL et al. (2000) Postinfectious Lucio phenomenon in diffuse leprosy. Report of 2 cases. Dermatologist 51: 945-949
  4. Fucha J et al (1992) Diagnostic and therapeutic problems in leprosy patients from a dermatological point of view. Act Dermatol 18: 231-235
  5. Hansen GA (1874) Advising the Norske medicinskes Selskab i Christiania on the support of a journey for the same day, for the purpose of inactive and insuring the informed party, in part carried out together with Hartwig Forest. Norsk Magazin for Lægevidenskaben 4: 1-88
  6. Hatta M et al (2003) Epidemiology of leprosy. Molecular, biological, and immunological approach. Adv Exp Med Biol 531: 269-278
  7. Ishii N (2003) Recent advances in the treatment of leprosy. Dermatol Online J 9: 5
  8. Niemeier V et al (1996) Lepromatous leprosy - Therapy options for glucose-6-phosphate dehydrogenase deficiency. Z Hautkr 71: 625-629
  9. Peters F et al (2016) Germ or no germ: Challenges in the diagnosis of mycobacterial skin infections. J Dtsch Dermatol Ges 14:1227-1236
  10. Preimsberger J(2014) Hansen disease at sea. Close Dermatology 30:44-45
  11. Ramos-e-Silva M et al (2001) Leprosy. Recognition and treatment. At J Clin Dermatol 2: 203-211
  12. Stingl P (1990) Leprosy. Pathogenesis-classification-diagnostic treatment. dermatologist 41: 126-130
  13. Teo SK et al (2002) Thalidomide in the treatment of leprosy. Microbes Infect 4: 1193-1202
  14. WHO (1988) A guide to leprosy control 2nd ed. Geneva: 27-28
  15. WHO (2017) WHO Fact Sheets Updated February 2017. ww.who.int/fact sheets/lepra.

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Last updated on: 03.07.2021