Leprosy

(Morbus Hansen [MH])

(Hansen’s disease)

Definition: A chronic mycobacterial disease primarily affecting the peripheral nervous system and
secondarily involving skin and other tissues.

History
 600 B.C – written records in India
 150 B.C – written records in China
 2nd century BC – Egyptian mummies
 From Egypt -> Europe (150 AD: Greece)
 Then -> Americas & other parts of the world
 1983: WHO -> Estimated total in world – 10.5 million
 1996 (Multiple drug treatment) -> 1.3 million
 Malaysia –Common Orang Asli & Foreign workers

Etiology
Mycobacterium Leprae [unculturable in lab] (Armauer Hansen 1874)

Grown in:

- mouse footpad
- 9 banded armadillos (discovered because of Armauer Hansen’s wife)

Man – only significant reservoir

Transmission:

- via nasal secretions by droplets

- Skin
Pathogenesis
Principal target cell = Schwann cells

Nerve damage

- Anaesthesia
- Muscle paralysis

Skin -> usually first sign/symptom

- Non-specific lesions with pigmentary changes (hypopigmented) – anaesthesia /

hypoaesthesia (Whether the patient still feels pain at the area. Use a needle to prick)
- Often heals spontaneuously

Clinical manifestations depend on immune status of patient

Range TUBERCULOID -> LEPROMATOUS

TUBERCULOID
Hyperactive immune response
Few localized skin lesions with few bacilli
(paucibacillary)
Intense granulomatous response -> Damage
major nerves (hardening)
LEPROMATOUS
Anergy
Numerous skin lesions – often confluent
Bacilli ++++ (multicacillary)
Immune response greatly weakened (anergy)
Ear lones – bacilli ++++

- Those with the better immune system will suffer from the tuberculoid form.
- Those with the lepromatous form will have a negative tuberculin test.
- The safest area to wear earings/studs is at the ear lobes. Anywhere else, then it will easily
affect the ear cartilage. It could lead to cauliflower ear. (Pak Nasa’s story)

More WHO criteria:

- Borderline tuberculoid (BT)

- Mid-Borderline (BB)
- Borderline Lepromatous (BL)

Other signs & Symptoms

Eye

- Uveitis
 - Corneal infection secondary to eyelid paralysis
- The 2 symptoms above might lead the person to suffer from blindness

Bone resorbtion

- Collapse of nasal bone

- Shortening of fingers & toes (autoamputation)

Neuritis

Orchitis

Immune-Complex Nephritis

Facial skin may become crumpled and look like a lion (Facies leonina)

DIAGNOSIS
History & physical examination

Slit-skin smear and punch biopsy (AFB)

- Ear lobe
- Nasal secretion
- Nasal mucosa
Histology – granuloma + caseation

Skin test

- Lepromin test (only guide)

- Fernandez (early) Rx 1-2/7
- Mutsida (late) Rx 7/7 (Usually negative in Lepromatous Leprosy)

Most cases of Lepromatous Leprosy cases in Malaysia are among the Chinese

Split-skin smear - Acid fast staining

Record # of bacteria/high power

1+ : 1-10 per 100 fields

2+ : 1-10 per 10 fields

3+ : 1-10 per field

4+ : 10-100 per field

5+ : 100-1000 per field

6+ : >1000 per field

= Bacillary Index (BI)

Acid Fast Bacilli:

- if viable -> stains strongly and evenly

- Dead when stains unevenly & weakly

Morphological Index (MI):

 Percentage (%) of regularly stained (viable)

 Increase MI – Active disease
 MI decreases with chemotherapy

Other Diagnosis methods:

- α-PGL
- Polymerase Chain Reaction (PCR)

Treatment:

Dapsone (diaminophenyl sulphone –DDS)

- Before 1982 – Standard monotherapy for all forms

DDS – Resistance -> MDT

Add

- Rifampin
- Clofazimine (side effect: Skin discolouration)

Other drugs

- Prothionamide
- Ofloxacin
- Minocycline

WHO

Paucibacillary

- Rifampin
- Dapsone
- (Give these for 6 months)

Multibacillary

- Rifampin
- Dapsone
- Clofazimine
- (Given for 2 years or more)

Reactions:

Lepra/Jopling’s Type I:

- Delayed Type Hypersensitivity

- Vasculitis (Type II HS)

Lepra/Jopling’s Type II

- Erythema Nodosum
- Leprosum

Infections by environmental (Opportunistic) mycobacteria (Pak Nasa said this part is not important)

Runyon’s classification

a) Photochromogens (Group I)
- M. Kansasii
- M. Marinum
- M. Simiae
b) Scotochromogens (Group II)
- M. Scrofulaceum
- M. gordonae
- M. szulgai
 c) Non-Chromogens (Group III)
- M. avium
- M. intercellulare
- M. malmoense
- M. xenopi
- M. ulcerans
- M. terrae
d) Rapid Growers (Group IV)
- M. chelonae

Principal types of opportunistic mycobacterial infections in man

Lymphadenopathy

- M. Avium complex
- M. Scrofulaceum

Skin lesions

a) Post-trauma abscesses
- M. Chelonae
- M. Fortuitum
- M. Terrae
b) Swimming Pool Granuloma
- M. Marinum
c) Buruli ulcer
- M. Ulcerans

Pulmonary Disease

- M. Avium complex
- M. Kansasii
- M. Xenopi
- M. Malmoeure

Disseminated Disease

a) AIDS related
- M. Avium complex
- M. Genevense
b) Non-AIDS related
- M. Avium complex
- M. Chelonae