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Untitled Prezi

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Nourhan M

on 12 May 2016

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non infectious diseases
Pemphigus vulgaris
Mucous Membrane Pemphigoid
& clinical significance
Vesiculo-Bullous
Erythema Multiforme
Objectives....
What are Visculo Bullous diseaes
Its types are " infectious & non-infectious "
Erythema Multiforme
Pemphigus Vulgaris
Mucous Membrane Pemphigoid
Conclusion
Conclusion
Types

Vesiculo- Bulous diseases mean ....
???!
An uncommon auto immune vesiculo bullous disease
common in 40-60 years
Also in Askino's and jews
affect the skin & mucous membrane
FATAL if untreated
Oral manifestations:
1
Etiology :
3
ORAL:
Thank you ^_^ !!
Intro:
Minor EM
Major EM
Toxic Epidermal Necrolysis "TEN"




skin manifestations

Oral manifestations


a type of mucocutaneous disease that is characterized by vesicles and bullae (i.e. Blisters).
Both vesicles and bullae are fluid-filled lesions , and they are distinguished by size
1. Vesiculobullos - Maculopapular lesion

central zone of erythema
zone of edema
outer zone of erythema
3. +ve Kopner's phenomenon
2.
Lips :
hemorrhagic crusting
Extensive Bulla ->Erosion ->
covered by greyish-white membrane.

{ steven Johnson’s }
it represents the extreme end of the Steven Johnson syndrome.
>30% of the body surface area is involved
Death usually occurs as a result of sepsis & subsequent multiorgan system failure.

Oral :
Skin :







Extensive erythema followed by vesiculobullos lesions
Atypical target lesions.
The +ve Nikolsky sign:
*a separation of the papillary dermis from the basal layer upon gentle lateral pressure
<10% of the body surface area is involved
Several organs are affected :
* Mouth *Eyes * Skin








* Male genitalia

*Anal MM

* Bronchitis & pneumonitis
<5-10mm
>5-10mm
Extensive Bullous Lesions
lip & tongue erosions
Purulent
conjunctivitis
Vesicles spread ->Rupture -> Erosions
Desquamative Gingivitis
Skin:
Large tense bullae
+ nickolysky's sign

EYE:
conjunctival erosion
corneal ulcers
Diagnosis
Treatment
Topical corticosteroids
Systemic corticosteroids
Direct immunofluorescence
Diagnosis
In EM
In PV & MMP
rapture of vesicles
Electrolyte imbalance
2ry infections
"FATAL"
2.Target lesions "Iris"
Etiology
residual erosions superfacial ,
ragged edges painful lesion

Desquamative Gingivitis
skin manifestations
The primary lesion is flaccid or blister bulla that easily raptured ,, leaving erosions and crusting and eventual marked post inflammatory changes
Flaccid Bullae

+ve Nicklosky's sign
Treatment
Biopsy
Treatment

If a
Drug

is suspected,--> withdrawn as soon as possible.

Infections
should be --> treated after cultures and/or serologic tests have been performed.

Suppression of herpes simplex virus (HSV)
can prevent
HSV-associated erythema multiforme.

analgesics, local skin care, and mouthwashes
(e.g., oral rinsing with warm saline)
Topical steroids

supportive care for eye
( topical lubricants for dry eyes, sweeping of conjunctival erosion )
IgG reacts with desmosomal junctions between basal cell layer and overlying epithelium( antigen)


intra epithelial bullae
sub epithelial bullae
Smear:
round cells with large nuclei & little cytoplasm
Direct immunofluorescence
labeled IgG reaction over prickle cell layers
-> Auto antibodies against desmologin 3
"an attachment molecule in desmosomes "
prednisolone 60-100mg/day alone
prednisolone + Azathioprine 1-1.5mg/Kg daily
1.
2.
intralesional steroid application
3.
to lower the dose of corticosteroids and reduce their side effect
due to reaction of IgG with hemidesmosomes"at Basement membrane level"
C.T infiltrated
with inflammatory cells
Bulla floor :
intact epithelium
Bulla roof
sub epithelial bulla
PM on dorsal surface of the tongue
widely spread vesicles ..
yellowish, white floor with erythematous hollow
**rarely
Other Mucous Membrane manifestations...
An endoscopic view of an 84-year-old man with MMP
- with extensive reddish erosion of the entire esophagus and whitish sheets and fragments of sloughed mucosa
Esophagitis
full thickness of epithelium
at BM Level
Indirect Immunofluorescence
of Pemphigoid Serum on Human Split Skin Substrate

Treatment may require the combined efforts of a team of specialists.
Dental specialists
(oral pathologists)
(
dermatologists
) (
ophthalmologists)
(otolaryngologists)
and other healthcare professionals
prednisone
& /or
immunosuppressive drugs
- is an antibiotic that destroys the cells
that produce antibodies
Treatment
Rituximab ->
mycophenolate mofetil
azathioprine
cyclophosphamide
" if the pemphigoid seems to be getting worse"
- early detection to oral manifestations
- gentle handling of the patient is required to avoid ....
**refers to skin lesions appearing on lines of trauma due to scratching or infective cause
-->early detection
severe homorrhagic crusting
Severe oral ulceration
Treatment of TEN
The primary treatment of TEN is discontinuation of the causative factor(s)

management in burn units or intensive care units, supportive management, & nutritional support.

Initial interest in
Intravenous immunoglobulin (IVIG)
.

**could inhibit keratinocyte apoptosis ,
so prevent further progression of the disease ..
"cicatricial pemphigoid"
Reference list
http://www.derm101.com/therapeutic/erythema-multiforme-2/clinical-cases/
http://www.wjgnet.com/1948-5190/full/v2/i7/252.htm
http://rarediseases.org/rare-diseases/mucous-membrane-pemphigoid/
https://spartanpharmacy.wordpress.com/tag/antihistamines/
https://dailymed.nlm.nih.gov/dailymed/archives/fdaDrugInfo.cfm?archiveid=44068
http://www.homewithoutcancer.com/images/homewithoutcancer_news/bbb33.jpg
https://www.webmedcentral.com/articlefiles/5e413cd6de10b2c3e4d9abb0bbb5d8ae.jpg
http://www.aafp.org/afp/2010/1201/afp20101201p1381-f3.jpg
http://www.waent.org/archives/2011/Vol4-2/20110828-stevens-johnson/3rd_pt_post_tretment-820.jpg
http://www.petmartpharmacy.com/azathioprine-pid-10365/
http://www.canadianpharmacy365.net/product/prednisolone/
https://www.sanovadermatology.com/eczema/what-are-intralesional-steroid-injections-used-for/
http://www.westernsafety.com/products/prostatfirstaid/prostatfirstaid2013pg9.html
http://medical-dictionary.thefreedictionary.com/toxic+epidermal+necrolysis
http://www.dermnetnz.org/reactions/sjs-ten.html
http://www.slideshare.net/tosifahmad395/stevens-johnson-syndrome-26583422
skin erosion
Fistooning is very characteristic
"Must be alcohol free"
"Livamisole hydrochloride "
Treponema Pallidum
as squamous cell carcinoma"


Salma alaa el-din 130339
Ramy Mohamed tarek Shousha 133317
Reem Usama Mahmoud Elmasry 136247
Vivian Abdel-Fady labeb 121351
Rawan omar M Holebah 102263
Sara mahmoud elmahgary 131135
Reem mohamed Daher 112931
Yousef ahmed jamali 061247
norhan mohamed sayed 134943
Tarek mahmoud abbas 135355
Nada gamal elsawy 131099
Taha mohammed hassan 134555
Tarek Ahmed Mushtaha 090429
reham awad Hafez mostafa 132885
Nourhan Medhat Abdelmoneim Khattab 134779
Done by:
History :
Albort M. Johnson
History
Under Supervision of :
Prof.Naglaa El Wakeel
Dr. yasmin Gamil
Full transcript