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A-17 Hansens Disease > Hansens > Flashcards

Hansens Flashcards

1
Q

countries that account for 80% of cases world wide

A

Brazil; India, Indonesia

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2
Q

How many percent of persons infected w m leprae are able to resist infection

A

90%

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3
Q

genetic susceptibility to acquire hansens disease

A

Monozygotic twins 60-85%

dizygotic 15-25%

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4
Q

latency period for paucibacillary and multi

A

5 yrs- paucibacillary

10 yrs- Multibacillary

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5
Q

M leprae grows in what temp

A

Below 30 degrees. the core body temp of humans.

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6
Q

spares what area?

A

Sparing midline and scalp

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7
Q

organism may be cultivated where?

A

Mouse footpads

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8
Q

surface ycolipid unique to leprosy

A

PGL1 (phenolic glycolipid) 1

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9
Q

M lepromatosus is isolated from where?

A

Mexico

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10
Q

Diffuse type of lepromatous leprosy

A

Lucio leprosy.

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11
Q

smears are speific how many percent of hansens disease have negative smears?

A

70%

smears are taken from lesions and cooler areas of the skin such as earlobes, elbows, knees

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12
Q

skin smears of paucibacilliary and multibacillary

A

Paucibacillary- negative

Multibacillary/ organisms found on skin smears

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13
Q

enlarged nerves are found in how kany percent multi and pauci

A

Multi- More than 90%

pauci- 75-85

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14
Q

Classification name

A

Ridley and Jopling scale

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15
Q

1st clinical manif in 90% of pxs.

A

Numbness

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16
Q

earliest sensory changes

A

loss if senses of temperature and light touch most often in the feet or hands.

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17
Q

1st lesion noted

A

Solitary poorly defined hypopigmented macule

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18
Q

histo

A

Lymphocytic infiltrate without granulomas sometimes w involvement of cutaneous nerves

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19
Q

jsolitary few in number asymmetrical
hypopogmented or erythematous usually dry scaly and hairless.
“saucer right side up”
mosy common loc: face limb or trunk,

scalp axillae groin; perineum not involved.

A

Tuberculoid leprosy

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20
Q

tuberculoid lesion

A

Anesthetic or hypesthetic and anhidrotic. and superficial peripheral nerves serving or proximal to the lesion are enlarged tender or both. greater auricular nerve and sup peroneal nerve may be visibly enlarged.
atrophy.
spont remission in 3 years.

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21
Q

diff tuberculoid leprosy from borderline tuberculoid.

A

Borderline tuberculoid are more smaller and more numerous. Satellite lesions around large macules or plaques are characteristic.

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22
Q

Desc Borderline borderline leprosy BB

A

Skin lesions are numerous but countable and consist or red, irreg shaped plaques. generalized but asymmetric.
Nerves be thickened and tender.

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23
Q

Borderline lepromatous leprosy (BL)

A

symmetric and numerous too many to count. include macules, papules, plaques, and nodules.
nerves are large tender or both. sensation or sweating normal.

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24
Q

Lepromatous leprosy

A

Pale macules. diffuse infiltration of skin.
divided into polar and subpolar.
macular lesions dist diffusely and symmetrically over the body.
small and numerous. poorly defined, show no change in skin texture.
minimal or no loss of sensatioj over lesions
no nerve thickening
no change in sweating.
loss of hair on outer third of eyebrows.
nodules called lepromas. most often in actal parts: ears brows nose chin elbows hands buttocks or knees.
nerve involvement occurs very slowly.
nerve disease us bilaterally symmetric- stocking glove pattern.

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25
Q

uncommon form if multibacillary hansens wc skin lesions appear as large yellow red shiny papules and nodules on dermis or subcutaneous tissue.
resemble molluscum.

A

Histoid leprosy

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26
Q

histopathologic hllmark of hansens disease

A

neurotropism

27
Q

nerve involvement in Pauci and multi

A

Pauci- anesthesia within skin lesions.

multi/ lepromatous- progressive stocking glove peripheral neuropathy.

28
Q

neuropathy is termed : what in who grade 1 and 2

A

Who grade 1: primary impairments

2: Secondary or visible impairments.

29
Q

neuropathy is present in how many percent of pauci and multi

A
  1. 3-3.5% of pauci

7. 5-24% of multi.

30
Q

secondary impairments occur in how many perfent of multi

A

33-56%

31
Q

first symptom

A

inability to distinguish hot and cold.

32
Q

result from involvement if 7th cranial nerve

A

Corneal erosions
exposure kerstitis
ulcerations

33
Q

of multi pxs, how many percent are blind at dx and will have potentially blinding orocess

A

2.8% -4.6 Blind at diagnosis.

11%a Potentially blinding process

34
Q

most freq involved kucous membrane

A

nasal mucosa

35
Q

organs not contain leprsoy bacilli

A

GI lungs brain

36
Q

visceral infection is restricted mostly to what system

A

reticuloendothelial system

37
Q

pregnancy affect on leprosy

A

exacerbation or reactivation after apparent cure

38
Q

hansens dx reported in the fr organ transplant

A

renal liver heart bm

39
Q

immunopathogenesis in leprosy

A

tuberculoid lesions : good cell mediated immunity with IFN gamma and IL2 present.
lepromatous: cytokines reduced. IL4/5 /10 . ytokines that downregulate cell med immunity and enhance suppressor function Nd antibody prod

40
Q

what stain used for demonst m leprae

A

frite fraco

41
Q

histo of lepromatous leprosy comp of

A

bacilli laden and lipid laden histiocytes so called lepra cells or foam cells of virchow.
infiltrate is kocalized in dermis and may be purely perivascular or sheet like and seprated from the epidermis by a well dfined grenz zone
AFB are abundant and appear as round clumps (globi)

42
Q

type of reaction w represent an enhanced cell mediated immune response to m leprae

A

Type 1 reacuton

43
Q

if rxns occur w antibiotic chemo theybare called what

A

reversal reactions

44
Q

Type 1 rxns clinically represent as:

A

Inflammatioj of excisting lesions
no systemic symptoms such as fever chills or arthralgias.
lesions swell become erythematous and sometimes tender simulating cellulitis. severe cases; ulceration.

45
Q

histo of type 1 rxn

A

Perivascular and perineural edema and large num of lymphocytes.
severe rxns may demonstrate tissue necrosis.
bacilli reduced.

PBL- 1

46
Q

other name for type 2 rxn

A

Erythema nodosum leprosum

47
Q

type 2 rxn char

A

Multisystem involvement
systemic sx (fever myalgia arthralgia anorexia)
skin lesions are char erythematous subcutaenous dermal nodules
severe skin lesions ulcerate.

48
Q

Type 2 histo

A

ENL demosntrate a leukoclastic vasculitis:

49
Q

is an uncommon and unusual rxn that occurs in pxs w diffuse lepromatous leprosy of the “la bonita” most often in western mexico.
lacks neutrophilia and systemic symptoms.
pruritic macules evolve to bullous lesions thy rapidly ulcerate spec below the knees.

A

Lucio phenomenon

50
Q

lucio histo

A

Bacilli are numerous and in add to being in the dermis are seen within blood vessel walls w thrombosis of middermal vessels resulting in cutaneous infarction.

51
Q

tx. dapsone monotherapy promotes

A

resistance

52
Q

std tx

A

MDT

53
Q

WHO defines paucibacillary disease as presence of

A

No bacilli on smears or biopsy and <=5 lesions

54
Q

tx for rifampin resistant patients

A

MCCM

moxifloxacin 400mg
Clofazimine 50mg
Clarithromycin 500mg
minocycline 100mg

daily for6 months

55
Q

continuous phase for rifampin resistant

A

MCM

moxifloxacin 400mg
Clarithromycin 1000mg
minocycline 200mg
once monthly supervised for another 18 mos

56
Q

Type 1 rxns tx

A

Systemic steroids.

prednisone orally starting dise 40-60mg/day.

57
Q

neuritis and eye lesions aee urgent indications for

A

systemic steroid therapy

58
Q

used if steroids fail or as a steroid sparingagent

A

cyclosporine

59
Q

tx of choice for ENL

A

Thalidomide . initial recomm dose is up to 400 mg/day in pxs more than 50kg.

60
Q

thalidomide SE

A

Teratogen . long term use assoc w risk w sensory neuropathy. and short term use assoc w risk of thromboembolic phenomena also constipation

61
Q

given to pxs w enl when thalidomide cannot be used or to avoid the use of systemic steroids to manage severe enl.

A

Comb of pentoxifylline 400-800mg twice a day and clofazimine 300mg/day

62
Q

poorly resposive to both steroid and thalidomide

A

Lucio phenomenon

63
Q

BCG prov how many percent protectioj against m leprae

A

34-80%

A-17 Hansens Disease (1 decks)

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