Hansens Flashcards
countries that account for 80% of cases world wide
Brazil; India, Indonesia
How many percent of persons infected w m leprae are able to resist infection
90%
genetic susceptibility to acquire hansens disease
Monozygotic twins 60-85%
dizygotic 15-25%
latency period for paucibacillary and multi
5 yrs- paucibacillary
10 yrs- Multibacillary
M leprae grows in what temp
Below 30 degrees. the core body temp of humans.
spares what area?
Sparing midline and scalp
organism may be cultivated where?
Mouse footpads
surface ycolipid unique to leprosy
PGL1 (phenolic glycolipid) 1
M lepromatosus is isolated from where?
Mexico
Diffuse type of lepromatous leprosy
Lucio leprosy.
smears are speific how many percent of hansens disease have negative smears?
70%
smears are taken from lesions and cooler areas of the skin such as earlobes, elbows, knees
skin smears of paucibacilliary and multibacillary
Paucibacillary- negative
Multibacillary/ organisms found on skin smears
enlarged nerves are found in how kany percent multi and pauci
Multi- More than 90%
pauci- 75-85
Classification name
Ridley and Jopling scale
1st clinical manif in 90% of pxs.
Numbness
earliest sensory changes
loss if senses of temperature and light touch most often in the feet or hands.
1st lesion noted
Solitary poorly defined hypopigmented macule
histo
Lymphocytic infiltrate without granulomas sometimes w involvement of cutaneous nerves
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.
Tuberculoid leprosy
tuberculoid lesion
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.
diff tuberculoid leprosy from borderline tuberculoid.
Borderline tuberculoid are more smaller and more numerous. Satellite lesions around large macules or plaques are characteristic.
Desc Borderline borderline leprosy BB
Skin lesions are numerous but countable and consist or red, irreg shaped plaques. generalized but asymmetric.
Nerves be thickened and tender.
Borderline lepromatous leprosy (BL)
symmetric and numerous too many to count. include macules, papules, plaques, and nodules.
nerves are large tender or both. sensation or sweating normal.
Lepromatous leprosy
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.
uncommon form if multibacillary hansens wc skin lesions appear as large yellow red shiny papules and nodules on dermis or subcutaneous tissue.
resemble molluscum.
Histoid leprosy
histopathologic hllmark of hansens disease
neurotropism
nerve involvement in Pauci and multi
Pauci- anesthesia within skin lesions.
multi/ lepromatous- progressive stocking glove peripheral neuropathy.
neuropathy is termed : what in who grade 1 and 2
Who grade 1: primary impairments
2: Secondary or visible impairments.
neuropathy is present in how many percent of pauci and multi
- 3-3.5% of pauci
7. 5-24% of multi.
secondary impairments occur in how many perfent of multi
33-56%
first symptom
inability to distinguish hot and cold.
result from involvement if 7th cranial nerve
Corneal erosions
exposure kerstitis
ulcerations
of multi pxs, how many percent are blind at dx and will have potentially blinding orocess
2.8% -4.6 Blind at diagnosis.
11%a Potentially blinding process
most freq involved kucous membrane
nasal mucosa
organs not contain leprsoy bacilli
GI lungs brain
visceral infection is restricted mostly to what system
reticuloendothelial system
pregnancy affect on leprosy
exacerbation or reactivation after apparent cure
hansens dx reported in the fr organ transplant
renal liver heart bm
immunopathogenesis in leprosy
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
what stain used for demonst m leprae
frite fraco
histo of lepromatous leprosy comp of
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)
type of reaction w represent an enhanced cell mediated immune response to m leprae
Type 1 reacuton
if rxns occur w antibiotic chemo theybare called what
reversal reactions
Type 1 rxns clinically represent as:
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.
histo of type 1 rxn
Perivascular and perineural edema and large num of lymphocytes.
severe rxns may demonstrate tissue necrosis.
bacilli reduced.
PBL- 1
other name for type 2 rxn
Erythema nodosum leprosum
type 2 rxn char
Multisystem involvement
systemic sx (fever myalgia arthralgia anorexia)
skin lesions are char erythematous subcutaenous dermal nodules
severe skin lesions ulcerate.
Type 2 histo
ENL demosntrate a leukoclastic vasculitis:
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.
Lucio phenomenon
lucio histo
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.
tx. dapsone monotherapy promotes
resistance
std tx
MDT
WHO defines paucibacillary disease as presence of
No bacilli on smears or biopsy and <=5 lesions
tx for rifampin resistant patients
MCCM
moxifloxacin 400mg
Clofazimine 50mg
Clarithromycin 500mg
minocycline 100mg
daily for6 months
continuous phase for rifampin resistant
MCM
moxifloxacin 400mg
Clarithromycin 1000mg
minocycline 200mg
once monthly supervised for another 18 mos
Type 1 rxns tx
Systemic steroids.
prednisone orally starting dise 40-60mg/day.
neuritis and eye lesions aee urgent indications for
systemic steroid therapy
used if steroids fail or as a steroid sparingagent
cyclosporine
tx of choice for ENL
Thalidomide . initial recomm dose is up to 400 mg/day in pxs more than 50kg.
thalidomide SE
Teratogen . long term use assoc w risk w sensory neuropathy. and short term use assoc w risk of thromboembolic phenomena also constipation
given to pxs w enl when thalidomide cannot be used or to avoid the use of systemic steroids to manage severe enl.
Comb of pentoxifylline 400-800mg twice a day and clofazimine 300mg/day
poorly resposive to both steroid and thalidomide
Lucio phenomenon
BCG prov how many percent protectioj against m leprae
34-80%
