CWM Hospital Fiji School of Medicine Depts. of Medicine

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CWM Hospital / Fiji School of Medicine Depts. of
Medicine Pathology
CPC Meeting 16/8/2001

• Leprosy

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Case 1. Clinical details

• An 8 year old boy presented to clinic with a
  right Bells palsy.
  
• In addition it was noted that he had multiple
  hypo-aesthetic pale patches on the skin.

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Mouth
Reduced nasolabial fold
Drooping corner of mouth
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Right arm
Slightly nodular hypopigmented rash
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Left leg
Slightly nodular hypopigmented rash
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Diagnosis

• ?Leprosy for skin biopsy

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Case 2

• A 29 year old man was screened for skin lesions
  after his father was found to have leprosy

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Left cheek
Raised erythematous plaque over left cheek
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• He was also noted to have swollen hands and feet.

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Diagnosis

• ?Leprosy

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Case 3. Clinical Details

• 57 year Fijian Female
• Scaly skin lesions
• Punch biopsy from lesion on arm
• Referred from skin OPD

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Skin Biopsy

• Epidermis
• Collections of Foamy macrophages in the upper
  dermis.
  
• Around adnexa

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Skin Biopsy

• Epidermis
• Collections of Foamy macrophages in the upper
  dermis.

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Skin Biopsy

• Epidermis
• Collections of Foamy macrophages in the upper
  dermis.

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Skin Biopsy

• Collections of Foamy macrophages in the upper
  dermis.

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Skin Biopsy

• Faintly positive ZN Stain

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Skin Biopsy

• Faintly positive ZN Stain

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Diagnosis Paucibacillary leprosy

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Leprosy (Hansens Disease)

• Leprosy (Hansens disease), is a chronic
  infectious disease due to the organism
  Mycobacterium leprae.
  
• Leprosy affects the skin, nervous system, eyes
  and respiratory tract mucosa. It affects all ages
  and both sexes.
  
• It has a long incubation period of 3-5 years,
  although many people have lived among lepers for
  much longer periods of time than this without
  contracting the disease. Transmission is via the
  respiratory tract or from direct inoculation.

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• Leprosy can be diagnosed by the presence of skin
  lesions and sensory loss. Skin lesions are
  biopsied and smear preparations may also be taken
  of unaffected skin in cool areas of the body. On
  the basis of these investigations, it can be
  classified into three main patterns of disease.
• Smear positive disease is known as multibacillary
  disease, smear negative disease with more than
  one lesion is known as paucibacillary disease,
  whereas smear negative disease with only one
  lesion is known as single lesion paucibacillary
  disease.

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Epidemiology

• In 1997, there were about 1.2 million people
  world-wide who were disabled by leprosy. Over 80
  of cases in the world occur in India, Brazil,
  Indonesia, Bangladesh and Myanmar.
• In the Western Pacific region, there are 6
  countries in which leprosy is endemic Cambodia,
  Micronesia, Kiribati the Marshall Islands, Laos
  and Papua New Guinea. There are other countries
  like Fiji were it has been mostly eradicated.

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History

• The earliest documented case of leprosy comes
  from a human specimen dated to 200BC. (Diamond,
  1997). Leprosy appears to have spread from the
  Middle East to the rest of Europe and Russia by
  about the 12th Century AD. It is uncertain when
  it first occurred in the Pacific.

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History (cont)

• Throughout history, leprosy has been a disease
  which has been greatly stigmatized. Among
  European Christians in the Middle Ages, lepers
  were particularly feared. In nearly all European
  countries, the church conducted a ceremony known
  as separatio leprosarum in which the leper was
  administered rites similar to the rites of the
  dead.

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History (cont).

• Lepers were forbidden to enter the church, the
  market-place, or any assembly of people could
  not wash in the brook or anywhere else, and could
  only take water to drink with jugs or cups they
  carried. A leper could go only barefoot in a
  habit that marked him as a leper, was forbidden
  to touch anything he wished to, but could only
  point to it with his staff. He could not travel
  by main roads and on paths he could not touch
  hedges or bushes on either side unless first he
  put on gloves. He could not touch little children
  or any young people, and was forbidden to eat or
  drink with anybody but other lepers. He wore a
  lepers robe of black with a veil over his mouth
  and carried clappers to warn of his approach.
• (Rosebury, 1971)

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History (cont)

• Mycobacterium leprae, the organism responsible
  for leprosy, was first isolated by Dr Hansen in
  1873.
  
• In 1897 the first International Leprosy Congress
  in Berlin recommended compulsory isolation of
  known leprosy cases, which started soon
  afterwards in Fiji in a settlement in Walu Bay.
• In 1900, this settlement was moved to Soliyaga
  Peninsula in Beqa, and in 1908 work commenced on
  a new leprosy hospital at Makogai. This hospital
  opened in 1911, and by 1922 its reputation had
  spread to the extent that patients from Samoa
  began being admitted from that year and patients
  from New Zealand began to be treated from 1925.

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History (cont)

• In 1941 the sulphone drugs were discovered, and
  they were first used at Makogai in 1948. In the
  1950s there was a decline in the number of
  patients at Makogai as many were cured and some
  were then repatriated back to was opened other
  parts of the Pacific. Makogai was closed in 1969
  and leprosy patients were the Twomey Hospital in
  Suva, where patients would henceforth be treated,
  was opened in the same year

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History (Cont)

• During the 58 years of the hospital at Makogai
  there were approximately 4,500 admissions, of
  whom 2,500 were successfully treated and
  discharged, 500 were repatriated to other
  countries in the Pacific, and 1500 died at the
  hospital.
• Reverend Mother Mary Agnes worked at Makogai from
  1916 until her death in 1955 and is buried at the
  hospital cemetery and Dr Austin was medical
  superintendent of the hospital for 23 years.

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History

• In the 1970s and 80s it became apparent that drug
  resistance to dapsone was beginning to result in
  treatment failures. This was found to be due to a
  number of causes including irregular drug-taking
  by patients, taking drugs at lower doses than
  prescribed, and the prescription of single rather
  than multiple drugs.
• In 1981 a WHO expert committee suggested
  multi-drug therapy for leprosy, And in June 1982
  a drug policy seminar for the South Pacific held
  in Suva recommended the implementation of
  multi-drug treatment in Fiji and this was
  commenced in November 1983.

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Multi drug therapy

• Multi-drug therapy treats each of the three types
  of the disease slightly differently.
  
• In paucibacillary disease, patients are given a
  total of 6 monthly cycles in which Rifampicin
  600mg and Dapsone 100mg are given on day 1 and
  Dapsone 100mg is given from days 2-28.
• In multibacillary leprosy. Patients are given a
  total of 12 monthly cycles in which Rifampicin
  600mg, Clofazamine 300mg and Dapsone 100mg are
  given on day 1 and Clofazamine 50mg and Dapsone
  100mg are given on days 2-28.
• In single lesion paucibacillary disease,
  Rifampicin 600mg, Oflaxacin 400mg and Minocyclin
  100mg are given as a single dose.
  

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Epidemiology

• In November 1983, at the time that multi-drug
  therapy commenced, there were known to be 441
  people with active leprosy in Fiji of whom 339
  had multibacillary and 102 had paucibacillary
  disease. This suggested a prevalence of
  6.5/10,000 population. After 5 years of
  multi-drug therapy, there were 203 known cases,
  183 with multibacillary and 21 with
  paucibacillary disease, giving a prevalence rate
  of 2.8/100,000 population.

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Epidemiology

• By October 1995, there were 30 known cases, with
  a prevalence of 0.38/10,000 population. Since
  1995 there have been between 6 and 9 new cases of
  leprosy identified each year, the majority
  occurring within Fijian males. 1-3 children per
  year have been identified with the disease.

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• The low prevalence of leprosy in Fiji since the
  early 1990s has resulted in the integration of
  the leprosy services into the general dermatology
  services.
• The dermatology service continues to conduct
  screening of school children and household
  contacts of leprosy patients and has created a
  computerized database to help in the surveillance
  of patients and to identify relapsed cases.

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References

• Much of the information for this topic is from a
  talk by Dr Tuicakau, dermatologist CWM.
  
• Diamond J. (1997). Guns, germs and steel. Vintage
  Press, Australia.
  
• Rosebury T. (1971) Microbes and morals.
  Ballantine Books, New York.